Sarcoidosis Remission and Aden Protocol

Sarcoidosis Remission and Aden Protocol

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Sarcoidosis Remission and Aden Protocol Overview

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Clinical Manifestations Of Sarcoidosis

Sarcoidosis Submandibular Gland

Sarcoidosis occurs most commonly in American blacks and northern European Caucasians. It is eight times more common in American blacks than American Caucasians (Hellmann 1993). Women are affected slightly more frequently than men. Patients with sarcoidosis generally present with one of the following four problems respiratory symptoms such as dry cough, shortness of breath, and chest pain (40-50 ) constitutional symptoms such as fever, weight loss, and malaise (25 ) extrathoracic inflammation such as peripheral lymphadenopathy (25 ) and rheumatic symptoms such as arthritis (5-10 ) (Hellmann 1993). Respiratory symptoms are the most common presenting chief complaints including those previously mentioned. Regardless of symptoms, greater than 90 of patients with sarcoidosis have an Table 6.2. Clinical involvement by sarcoidosis. Table 6.2. Clinical involvement by sarcoidosis. Sarcoidosis abnormal chest radiograph. Four types of radiographic appearance have been described type 0 is normal...

Diagnosis Of Sarcoidosis With Salivary Gland Biopsy

Natural Medicine For Sarcoidosis

As with Sjogren's syndrome diagnoses with salivary gland biopsies, early stage disease is perhaps more readily diagnosed with a parotid biopsy rather than a minor salivary gland biopsy. It has been pointed out that cases of sarcoidosis that do not clinically produce parotid enlargement nonetheless show involvement at the microscopic level (Marx 1995). In this review, the labial biopsy was positive in 38 of cases while 88 of parotid biopsies were positive for sarcoidosis. The lesions of sarcoidosis in labial salivary gland biopsies tend to be sparse such that multiple labial glands require excision for microscopic analysis. Another report investigated the yield of minor salivary gland biopsy in the diagnosis of sarcoidosis (Nessan and Jacoway 1979). In this study of 75 patients, non-caseating granulomas were present in minor salivary gland biopsies in 44 patients (58 ). There was no correlation with minor salivary gland biopsy yield and stage of the disease. The highest yield for...

Sarcoidosis

Sarcoidosis is a chronic systemic disease characterized by the production of non-caseating granulomas whose etiology is unknown. It can affect any organ system, thereby mimicking rheumatic diseases causing fever, arthritis, uveitis, myositis, and rash (Table 6.2). The peripheral blood shows a dichotomy of depressed cellular immunity and enhanced humoral immunity. Depressed cellular immunity is manifested by lymphopenia and cutaneous energy. The enhanced humoral immunity is noted by polyclonal gammopathy and autoanti-body production.

Dacryoadenitis Clinical Summary

Dacryoadenitis is an uncommon inflammatory disorder of the lacrimal gland, located under the lateral portion of the upper lid. The most common causes are mumps and herpes virus. Bacterial causes include Staphylococcus, Streptococcus, gonorrhea, Chlamydia, and syphilis. Dacryoadenitis is associated with systemic inflammatory conditions such as sarcoidosis, and Sjgren syndrome. Clinical findings include painful swelling of the lateral third of the upper lid, conjunctival hyperemia, chemosis, and an S-shaped curve to the lid margin from ptosis of the upper lid. Diplopia may be present from involvement of the lateral rectus muscle.

Optic Neuritis Clinical Summary

Optic neuritis must be differentiated from papilledema (bilateral disk swelling, typically with no acute visual loss with the exception of transient visual changes), ischemic neuropathy (pale, swollen disk in an older individual with sudden monocular vision loss), tumors, metabolic or endocrine disorders. Most cases of optic neuritis are of unknown etiology. Some known causes of optic neuritis include demyelinating disease, infections (including viral, syphilis, tuberculosis, sarcoidosis), or inflammations from contiguous structures (sinuses, meninges, orbit).

Evaluation Guidelines

If infectious, neoplastic (especially meningeal carcinomatosis), or inflammatory (sarcoidosis) etiologies are suspected, cerebrospinal fluid (CSF) evaluation is warranted. CSF glucose level, protein level, differential white and red blood cell counts, and cytology tests are compulsory, whereas other studies to isolate mycobacterial, fungal, rickettsial, parasitic, and viral pathogens should be addressed on an individual basis. In the immunocompromised person (e.g., one with cancer, acquired immunodeficiency syndrome AIDS , and organ transplant), opportunistic pathogens causing infections such as cryptococcosis, candidiasis, mucormycosis, toxoplasmosis, and cytomegalovirus need to be seriously considered in the setting of any acute or subacute neurological presentation.

Preganglionic Syndromes

Trigeminal nerve compression can occur in the area between the brain stem nuclei and the gasserian ganglion, specifically within the brain stem itself or in the cerebellopontine angle. Patients can present with reduced facial sensation in association with poor hearing, nystagmus, limb ataxia, facial weakness, and a diminished corneal reflex. Common lesions in this area include tumorsy such as acoustic or trigeminal neuromas,y meningiomas, metastatic cancers, carcinomatous meningitis, and invasive nasopharyngeal carcinomas, inflammatory disorders such as sarcoidosis, or infectious processes such as mycobacterial (especially tuberculosis), fungal (candidal, histoplasmotic), parasitic, and bacterial organisms. Traumatic injury to this region may also result in sensory loss or motor deficits. Extensive brain stem and cerebellar signs may be evident from lesions in the cerebellopontine angle.

Associated Medical Findings

The skin should be checked for rashes, lesions, or evidence of insect bites. The head and neck should be examined for masses, signs of trauma, or postoperative scars. The auricle and ear canal should be examined thoroughly for vesicles, ulcers, or other lesions. The tympanic membrane should be checked for perforation, drainage, or cholesteatoma. Infections, neoplasms, and evidence of prior otological surgery should be sought while examining the middle ear. The oral cavity and pharynx should be checked for masses, ulcerations, fissuring of the tongue, and other lesions. The parotid should always be palpated for tumors and inspected for inflammation. If nonsuppurative parotitis, uveitis, and mild fever are found, Heerfordt's disease, a variant of sarcoidosis, may be present. Endocrinological function should be evaluated, because diabetes insipidus or other evidence of pituitary dysfunction can signify neurosarcoidosis. If there is incomplete eyelid closure, the cornea should always be...

Evaluation Guidelines Table132

Occasionally, biopsy of tissue outside the CNS may be productive in establishing a diagnosis involving the ninth and tenth cranial nerves. Amyloid neuropathy involving the cranial nerves may be demonstrated on peripheral nerve biopsy. Sarcoidosis with basilar meningitis and cranial neuropathies may be evident on lung or lymph node biopsy.

Diagnosis and Staging

Spirometry is the key to making a formal diagnosis, as well as for staging the severity of illness. COPD may be diagnosed when obstructive lung disease is not fully reversible, defined as a postbronchodilator FVC of less than 80 of predicted in a patient with evidence of airway obstruction (FEVj FVC ratio < 70 ). A pattern of restrictive lung disease (FVC < 80 of predicted in presence of normal FEVj FVC ratio) would suggest alternative diagnoses, such as pulmonary fibrosis, sarcoidosis, autoimmune conditions, or primary CHF.

Evaluation Guidelines Table142

Blood work may also be of diagnostic value (e.g., alkaline phosphatase levels in Paget's disease, angiotensin converting enzyme levels in sarcoidosis, Venereal Disease Research Laboratory test VDRL in syphilis, viral cultures in poliomyelitis, genetic testing in neurofibromatosis). Purified protein derivative (PPD) is helpful in diagnosing tuberculosis, as is lymph node biopsy in sarcoidosis. Other Tests. Plain films of the chest are of diagnostic value in evaluating pulmonary disorders that extend beyond the pleura to involve CN XI, including tuberculosis, lung carcinoma, and pulmonary sarcoidosis.

Epidemiology and Risk Factors

Secondary causes of pulmonary hypertension include chronic lung disease (COPD and chronic bronchitis), cardiac disease (congenital defects, mitral stenosis, left atrial myxoma), autoimmune or inflammatory conditions such as scleroderma and SLE (Paolini et al., 2004), and granulomatous disease such as sarcoidosis. Certain drugs (fenfluramine) can also cause the condition, as can chronic liver disease with portal hypertension. Some patients experience pulmonary hypertension as a complication of arterial clotting or chronic damage from single or multiple episodes of PE.

Clinical Presentation

Sarcoidosis typically presents in early adulthood, between ages 20 and 40. Although the disease is systemic, a spectrum of clinical manifestations may suggest the prognosis. The onset of fever, arthralgias, bilateral hilar adenopathy on chest radiograph, and a raised, reddish skin lesion along the anterior tibial surfaces (erythema nodosum) characterize Lofgren's syndrome, an acute, self-limiting form of sarcoidosis that often undergoes spontaneous remission and has a favorable prognosis. The insidious onset of dyspnea, dry cough, hilar adenopathy and infiltrates on chest film, new skin lesions of the trunk and extremities, and complaints of recent vision changes characterize a chronic progressive form of sarcoidosis marked by multiple flares of disease requiring repeated treatment throughout the patient's lifetime. Symptoms that bring patients to medical attention most often emanate from the lungs, skin, or the eyes (uveitis and lacrimal gland enlargement). Diagnosis of sarcoidosis...

Interstitial Lung Diseases

Interstitial lung diseases (ILDs) represent a broad range of acute and chronic lung disorders. The pathology may display varying degrees of pulmonary inflammation and fibrosis, leading ultimately to end-stage lung disease. ILDs are classified under the larger designation of diffuse parenchymal lung disease, which includes disorders from known causes (occupational and environmental exposures, as well as progressive infections), and unknown causes (sarcoidosis, lymphangi-oleiomyomatosis, pulmonary histiocytosis X, eosinophilic pneumonia, idiopathic interstitial lung diseases). The idiopathic ILDs have been subclassified by the American Thoracic Society and European Respiratory Society (2002) into the following clinicopathologic entities, in order of relative frequency idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia (NSIP), cryptogenic organizing pneumonia (COP), acute interstitial pneumonia (AIP), respiratory bronchiolitis-associated interstitial lung disease...

Diagnosis Of Sialosis With Salivary Gland Biopsy

Sialosis is a disease limited to the major salivary glands such that an incisional biopsy of parotid enlargement is indicated, rather than an incisional biopsy of the lip as might be considered in Sjogren's syndrome or sarcoidosis. As such, a minor salivary gland biopsy is of no value in making a diagnosis of sialosis. While histopathologic confirmation of this process is valuable, it is certainly possible to make a clinical diagnosis of sialosis based on historical findings (Mandel, Vakkas, and Saqi 2005). In addition, once a histopathologic diagnosis of sialosis has been established, the underlying cause of this disorder must be ascertained, if not already known preoperatively. Prompt treatment of the underlying disease process must then occur.

Inflammatory Neck Masses

Sarcoidosis is a granulomatous disease that causes cervical lymphadenopathy and may be the presenting sign in 10 to 15 of cases. This disorder typically affects the African American population. Other findings include fever, sinusitis, parotid swelling, and hilar adenopathy on chest x-ray films. Diagnosis is classically made by tissue biopsy showing non-caseating granulomas. High angiotensin-converting enzyme level is common but not diagnostic. Other studies include cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) to rule out other granulomatous diseases, purified protein derivative and acid-fast bacillus stains to rule out tuberculosis, and Venereal Disease Research Laboratory (VRDL) and RPR tests to exclude syphilis.

Indications and Contraindications for Surgical Reconstruction

Some patients may report a history of cortisone injections to treat the symptoms. This history needs to be taken into consideration when planning the reconstruction as it may have contributed to tendon degeneration. Other problematic conditions include systemic diseases (diabetes mellitus, seronegative inflammatory disease, spondyloarthropathies, or sarcoidosis) and previous infections in the area. The surgeon needs to assess all risk factors very carefully so as not to put the result of his intervention at risk. This is also the case for patients with tobacco use or chronic arterial or venous disease. Patients with severe vascular disease and with sensorimotor deficits, such as peripheral neuropathy or Parkinson's disease, should be excluded from surgical treatment.

Neurological Applications in Diagnosis and Treatment Extradural Spinal Lesions

Intramedullary lesions causing enlargement of the cord or conus with concomitant narrowing of the adjacent subarachnoid space include primary cord neoplasms such as ependymomas, astrocytomas, and hemangioblastomas metastases inflammatory conditions such as sarcoidosis and abscess formation cord hematomas and infarcts vascular malformations and congenital lipomas, dermoids, and epidermoids. Although these groups of lesions will most certainly cause widening of the silhouette of the cord or conus, the intrinsic pathology and extent of these intramedullary lesions are far better imaged with MRI.

Restrictive Lung Disease

This category includes a wide range of conditions which affect the lung and chest wall. Lung diseases include sarcoidosis and fibrosing alveolitis, while lesions of chest wall include kyphoscoliosis and ankylosing spondylitis. Pulmonary function tests reveal a decrease in both FEVi and FVC, with a normal FEVj q FVC ratio and a decreased FRC and total lung capacity (TLC). Small airways closure occurs during tidal ventilation, with resultant shunting and hypoxaemia. Lung or chest wall compliance is decreased thus, the

Catscratch Disease Clinical Summary

Catscratch disease is a benign, self-limited condition caused primarily by Bartonella henselae that manifests with regional lymphadenopathy (Fig. 14.54), which usually follows (1-3 weeks) red-brown, nontender papules at the presumed site of bacterial inoculation. A history of contact with or scratch from a cat is usually present. Lymphadenopathy may persist for months and in rare cases patients may develop complications such as encephalitis, osteolytic lesions, hepatosplenic lesions, weight loss, prolonged fever, and fatigue. The differential diagnosis includes lymphogranuloma venereum, bacterial adenitis, sarcoidosis, infectious mononucleosis, tumors (benign or malignant), tuberculosis, tularemia, brucellosis, and histoplasmosis.

Miscellaneous Extramedullary Pathology

Inflammatory pathologies such as sarcoidosis, tubercu-loma, or subdural empyema rarely occur as intradural mass lesions. Although spinal carcinomatous meningitis often complicates systemic cancer, secondary metastatic mass lesions of the intradural, extramedullary compartment are rare. Malignant intracranial neoplasms that are in apposition to the subarach-noid space or ventricles are the most likely intracranial tumors to demonstrate CSF drop metastasis into the spinal subarach-noid space.5 Systemic cancer accesses the subarachnoid space either through direct dural root sleeve penetration, or more commonly, hematogenously via the choroid plexus.

Clinical Manifestations and Pathology

Massive numbers of organisms in all reticuloendothelial organs with little tendency to granuloma formation. Clinical features include high fever, hepato-splenomegaly, lymphadenopathy, and pancytopenia due to bone marrow involvement. This type of disseminated histoplasmosis has been called the infantile form and may lead to death within days or weeks. Other patients, often older adults, have a more indolent illness, many months in duration, characterized by low or moderate fever, weight loss, and skin and mucous membrane lesions. Biopsies of involved tissues show well-formed granulomas similar to sarcoidosis. Organisms are scanty and often are demonstrated only with special stains.

Causes of Failed Initial Exploration for Hyperparathyroidism

It has been estimated that 2 to 10 of surgical failures may be attributed to an incorrect diagnosis.4 However, this is much less of a problem today with the advent of a reliable radioimmunoassay for intact parathyroid hormone (PTH). In patients with renal disease, PTH clearance is compromised by impaired renal clearance and may lead to spurious elevations of PTH levels if the hormone is measured by assays that fail to detect intact PTH molecular structures. In such circumstances, the measurement of PTH using double-antibody methods will help resolve this issue. Another, albeit rare, diagnostic pitfall are patients with benign familial hypocalciuric hypercalcemia (BFHH). This disorder is associated with moderate hypercalcemia and normal or slightly elevated blood PTH levels. BFHH can be diagnosed by a positive family history of hypercalcemia at times associated with unsuccessful parathyroid surgery, a 24-hour urinary calcium excretion of less than 100 mg, and the calculated value of...

Neurooncology Imaging

Enlargement may be viewed as neoplastic versus non-neoplas-tic. Neoplastic enlargement of the optic nerve may be caused by glioma, meningioma, neuroma, hemangioblastoma, metastasis, or lymphoma. Non-neoplastic conditions associated with optic nerve enlargement include elevated intracranial pressure, optic neuritis, Graves disease, sarcoidosis, toxoplasmosis, central vein occlusion, and tuberculosis. Posterior optic gliomas involving the chiasm and hypothalamus must be differentiated from other sellar and suprasellar lesions, including germ cell tumors, cran-iopharyngiomas, and pituitary adenomas.

Lymphocyte Disorders Lymphocytosis

Lymphocytosis can be classified as primary (malignant) or secondary (reactive). Primary lymphocytosis is defined in the context of an acute or chronic lymphoproliferative disorder, often caused by dysregulation of lymphocyte development and production. Leukemias (chronic lymphocytic, acute lymphocytic, hairy cell), lymphoma, and B-cell lymphocy-tosis are examples of primary or malignant lymphocytosis. Secondary lymphocytosis is defined as a lymphocytosis in a patient who does not have a known hematologic disorder and in whom the lymphocyte count is expected to return to normal in less than 2 months after cessation of the inciting condition. Reactive lymphocytosis can sometimes be mistaken for a primary or malignant lymphocytosis when examining the peripheral blood smear, particularly in infectious mononucleosis with a marked increase in larger, atypical, or transformed lymphocytes. Other causes of secondary lym-phocytosis may generate small lymphocytes, as in pertussis. Viral...

Surgery For Renal Tract Stones

Renal stones are frequently idiopathic but may be caused by hypercalciuria arising from sarcoidosis, malignancy, renal tubular acidosis, hyperparathyroidism, Cushing's syndrome or administration of adrenal corticosteroids. All these diseases may have anaesthetic implications and should be investigated at preoperative assessment. Patients with recurrent urinary tract infection related to bladder malfunction, e.g. neurological diseases or congenital abnormalities, form stones. This type of stone may be large and grow to become a staghorn calculus in the renal pelvis.

Laboratory Evaluation

There is no specific laboratory study or serum marker for the diagnosis of peripheral neuropathy. Information from the history and physical examination may direct specific laboratory tests (e.g., testing for specific toxins, infections, or inflammatory disorders). If the cause of neuropathy is not obvious, some screening laboratory studies should be considered ESR, CBC, LFTs, and determination of fasting blood glucose, glycosylated hemoglobin, BUN, creatinine, serum vitamin B12, and TSH levels. Additional studies may be warranted, depending on the initial workup, such as chest radiograph to rule out sarcoidosis, pulmonary function tests for GBS, or ECG for processes that affect cardiac conduction. An LP for CSF showing an elevated protein level with normal white blood cells (WBCs) may indicate an acquired inflammatory neuropathy (GBS or CIDP). Nerve biopsy is helpful only in specific cases and is usually a last step in the workup. This includes patients with suspected amyloidosis,...

Which organisms are associated with nontuberculous granulomatous spinal infections

Atypical mycobacteria (Actinomyces, Nocardia, and Brucella spp.), as well as fungal infections (coccidioidomycosis, blastomycosis, cryptomycosis, candidiasis, aspergillosis), are potential pathogens. Immunocompromised patients are at high risk for developing infections with atypical mycobacteria. Fungal infections can occur following use of broad-spectrum antibiotics in combination with central venous catheters for parenteral nutrition. Sarcoidosis can involve the spine and cause lytic, granulomatous lesions and should be included in the differential diagnosis.

Inflammatory Diseases

Inflammation may be caused by chemical or physical agents or infections. In this section infective endocarditis (IE), syphilis, and sarcoidosis will be discussed. Sarcoidosis Patients with sarcoidosis may have skin and cardiac involvement. The skin lesions that involve the face may take two forms red papules around the eyes, nose, and mouth, which are pruritic and do not ulcerate purple plaques that produce a bulbous nose, thickened cheeks, and thickened ears (lupus pernio) (101). There may also be erythema nodosum (red nodules on the legs) (101). Twenty percent of patients with sarcoidosis have cardiovascular findings at autopsy (102,103). Clinical manifestations include congestive heart failure, ventricular tachycardia, complete heart block, or cor pulmonale (102,103).

Approach to the Reoperative Patient

When the initial operation fails to return a patient to the nor-mocalcemic state or if hypercalcemia recurs after initial but temporary postoperative normocalcemia, the patient joins a special selective group of patients quite distinct from patients being considered for primary operation. It is extremely important that the diagnosis of primary HPT be reconfirmed rigorously. The confirmation of primary HPT should be based only on the stringent criterion of elevated, or inappropriately elevated, PTH in the presence of elevated ionized serum calcium. In some instances of suspected primary HPT, another cause for hypercalcemia may be present, such as sarcoidosis, vitamin D excess, metastatic malignant disease, multiple myeloma, and so forth. A repeated careful history taking and physical examination should be conducted. A detailed family history usually helps determine whether multiple-gland disease or BFHH should be suspected. Patients with a positive family history of less severe disease...

Primary Sensory Neuropathy Pure Sensory Distal Nerve Lesions

Examples of causes of diffuse sensory neuropathy include a dysproteinemic state (IgM monoclonal gammopathies with antimyelin-associated glycoprotein), amyloidosis (generally small fiber), hereditary, diabetes mellitus, uremia, hypothyroidism, immunological (scleroderma, sarcoid), and toxins. A generalized peripheral neuropathy can show selective involvement of certain fiber types such as large myelinated fibers. In these circumstances there will be a dissociated sensory loss with a deficit of vibration and proprioception while sparing pain and temperature on sensory examination. Examples of these conditions include Friedreich's ataxia, Charcot-Marie-Tooth disease, uremia, and Guillain-Barre syndrome.

Restrictive cardiomyopathy

Restrictive cardiomyopathy (rCMP) is diagnosed echocardiographically if there is enlargement of both atria, normal systolic function and wall thickness and a restrictive filling pattern (deceleration time < 150 ms) 59 . The cause of rCMP may be idiopathic or rCMP may be a cardiac manifestation of amyloidosis, sclerodermia, sarcoidosis, hemochromatosis, Churg-Strauss syndrome, hypereosinophilic syndrome, hyperoxaluria, cystinosis, Gaucher's disease, Fabry's disease, Noonan's syndrome, Werner's syndrome, reactive arthritis, pseudoxanthoma elasticum, various neuromuscular disorders, carcinoid or lymphoma 59, 60 . Compared to dCMP, patients with rCMP are assumed to have a worse prognosis regarding morbidity and mortality 60 . Whether rCMP is associated with an increased risk of ischemic stroke is unknown, but single cases with rCMP have been described who developed an ischemic stroke 61 . In a study of 15 patients with amyloidosis 60 developed an arterial thromboembolic event, three

Mecanism Of Centripetal Lipid Accumulation Supraclavicular Fat And Face

Hypothalamic Tumors Sarcoid Hypocortisolemia can be primary, in which there is a defect intrinsic to the adrenal gland, or secondary, when pituitary or hypothalamic dysfunction causes decreased secretion of CRH or ACTH. Primary adrenal insufficiency was described by Thomas Addison in 1855 and is most commonly associated with destruction of the adrenal glands, either by tuberculosis, acquired immunodeficiency syndrome (AIDS), autoimmune disorder, adrenal hemorrhage, or tumor. In such cases, ACTH levels are high in response to the low plasma levels of gluco-corticoids. Secondary adrenal insufficiency is most often caused by suppression of the hypothalamic-pituitary axis by exogenous glucocorticoid therapy. Endogenous causes are a result of pituitary destruction by large tumors, apoplexy (hemorrhage into a pituitary adenoma), pituitary infarction (Sheehan's syndrome), inflammatory process (lymphocytic hypophysitis, Langerhans cell histiocytosis), or granulomatous disease (sarcoidosis)....

Yamamoto Cranioacupuncture Paris

Infected Salivary Gland Scan

With either squamous cell carcinoma or melanoma there is also a concern for perineural invasion and spread. Tumors commonly known to have perineural spread in addition to the above include adenoid cystic carcinoma, lymphoma, and schwannoma. The desmoplastic subtype of melanoma has a predilection for neurotropism (Chang, Fischbein, and McCalmont et al. 2004). The peri-neural spread along the facial nerve in the parotid gland and into the skull base at the stylomastoid foramen must be carefully assessed. MRI with contrast is the best means of evaluating the skull base foramina for perineural invasion. Gadolinium enhanced T1 MRI in the coronal plane provides optimal view of the skull base (Chang, Fischbein, and McCalmont et al. 2004). There may also be symptomatic facial nerve involvement with lymph-adenopathy from severe infectious adenopathy or inflammatory diseases such as sarcoidosis. Bacterial infections Acute bacterial parotitis Chronic bacterial parotitis Chronic recurrent...

Parotid Gland Tumour Mri Radiology

Scan Mono Lymph Nodes

The etiology of chronic inflammatory states of the salivary glands varies by the particular gland in question. Chronic inflammatory changes in the parotid gland tend to be related to autoimmune disease (Sjogren's syndrome), recurrent suppurative parotitis, or radiation injury. Other etiologies include granulomatous infections such as tuberculosis or sarcoidosis. Chronic inflammation of the submandibular gland and to a lesser degree the sublingual gland is more commonly due to obstructive disease, particularly sialolithiasis. In the chronically inflamed state the glands are enlarged but over longer periods of time progressively reduced in size, and heterogenous density may be seen on CT with extensive fibrosis and small focal (punctate) calcification. The density on CT is often increased due to cellular infiltration and edema during acute phases of exacerbation. The surrounding subcutaneous fat may not show signs of edema as is seen with acute sialadenitis. MRI demonstrates similar...

Pupillary Syndromes Anisocoria

The most important disorder that produces Horner's syndrome is malignancy involving the preganglionic sympathetic pathways in the neck or next to the apex of the lung. A minority of these preganglionic Horner's syndrome cases are secondary to trauma, usually resulting from penetrating neck wounds and root involvement in spinal injuries, and they should be obvious from the history and physical findings. In stellate ganglia anesthetic blocks, a transient Horner's syndrome predictably occurs because the preganglionic ocular sympathetic fibers go through this area. Inflammation, caused by suppurative infections and granulomatous diseases such as sarcoidosis or tuberculosis in cervical lymph nodes, is an occasional nonmalignant cause for preganglionic ocular sympathetic palsy. This etiology

Central Hypothyroidism

Abnormalities of the pituitary gland, such as pituitary tumor, ischemic lesion (Sheehan's syndrome), and iatrogenic events (surgical removal or radiation therapy), can cause central hypothyroidism with decreased pituitary TSH secretion. Other rare causes of pituitary lesions include tuberculosis, syphilis, hemochromatosis, sarcoidosis, histiocytosis, and aneurysms of the internal carotid artery.

Ocular Misalignment Syndromes

Functions of that ipsilateral nerve are affected. Localizing the lesion along the fascicular portion of the nerve depends on the presence of associated neurological features. Nuclear lesions are caused primarily by small infarcts secondary to occlusion of the medial penetrating vessels from the basilar artery (see Chapter22 ), '571 or rarely by small hemorrhages. Other intramedullary lesions associated with cranial nerve III dysfunction include masses, including neoplastic (lymphoma, metastatic), inflammatory (sarcoidosis), and infectious (tuberculosis, fungal) etiologies.

Nuclear Brain Stem Syndromes

Tolosa-Hunt syndrome, sarcoidosis, lupus, orbital pseudotumor Other pathological processes within the brain stem can produce trigeminal dysfunction. Tumors, hemorrhage (hypertensive, ruptured arteriovenous malformation), infarctions, demyelinating disease such as multiple sclerosis, infections such as brain stem abscesses and brain stem encephalitis, and inflammatory conditions such as tuberculosis or sarcoidosis affecting the lateral pons or midbrain may result in ipsilateral or contralateral facial sensory loss, respectively, as well as severe paroxysmal hemifacial pain. Facial weakness, muscle atrophy, difficulty chewing, and diminished jaw jerk reflex may be identified in amyotrophic lateral sclerosis as Figure 10-5 Left-sided trigeminal nerve involvement from sarcoid involving the skull base and meninges. Weakness of the pterygoids and masseters manifested as ipsilateral (leftward) jaw deviation and weakness of jaw opening.

Mikuliczs Disease And The Benign Lymphoepithelial Lesion

The pathologic entity known as the benign lym-phoepithelial lesion was once referred to as Miku-licz's disease. The German surgeon Johann Mikulicz first described the benign lymphoepithelial lesion in 1888 in a report of a single case of lacrimal gland involvement (Daniels 1991). The lacrimal gland enlargement was followed by enlargement of the submandibular and parotid glands, as well as minor salivary gland tissue. The term Mikulicz's disease was subsequently applied to a variety of cases of bilateral salivary or lacrimal gland enlargement, including those caused by sarcoidosis, lymphoma, tuberculosis, or syphilis. The term lymphoepithelial lesion was proposed by Godwin in 1952 to describe parotid gland lesions previously called Mikulicz's disease, ade-nolymphoma, chronic inflammation, lymphoepi-thelioma, or lymphocytic tumor (Godwin 1952). One year later, Morgan and Castleman observed numerous similarities of the benign lymphoepithe-lial lesion to the histopathology of Sjogren's...

Clinical Summary

Hard exudation and cotton wool spots are associated with vascular diseases such as diabetes mellitus, hypertension, and collagen vascular diseases but can be seen with papilledema and other intrinsic ocular conditions. Inflammatory exudates are seen in patients with such diseases as sarcoidosis and toxoplasmosis.

Clinical History

Oculomotor dysfunction may occur in the setting of any systemic disorder. One of the most common diseases to affect ocular motility is thyroid orbitopathy. The past history is particularly important because thyroid function studies are commonly normal when the patients present with orbital findings. A history of fatigue, weight change, skin alterations, heat intolerance, and other constitutional symptoms is thus important to elicit. A large contingent of patients who present with cranial mononeuropathies have a presumed ischemic etiology based on putative small vessel occlusive disease. These can usually present in persons older than 60 years of age, and many have other arteriosclerotic risk factors such as systemic hypertension or diabetes mellitus. A history of these disorders should be elicited by the interviewer. A full inquiry about multisystem signs and symptoms is essential to identify collagen vascular diseases such as periarteritis nodosa or systemic lupus erythematosus,...

Sinusitis

The most important risk factor for the development of sinusitis is rhinitis (e.g., viral, allergic). Other risk factors include anatomic abnormalities (abnormality within the sinuses, septal deviation, choanal atresia, foreign body, adenoid hypertrophy), nasal polyps (which can also occur secondary to chronic sinusitis), conditions of local or systemic immunodeficiency, cystic fibrosis, primary ciliary dysfunction (Kartagener's syndrome), secondary ciliary dysfunction (cigarette smoking, nasal decongestant abuse, cocaine abuse), gastroesophageal reflux disease (GERD), systemic inflammatory conditions (sarcoidosis, Wegener's granulo-matosis), dental disease, and nasal or sinus tumors. Any of these conditions can mimic or cause rhinosinusitis. Further

Inspect the Eyes

Examination of the eyes may reveal an arcus senilis. An arcus (see Figs. 10-53 and 10-54) seen in a patient younger than 40 years should raise the suspicion of hypercholesterolemia. Opacities in the cornea may be evidence for sarcoidosis, which may be responsible for cor pulmonale or myocardial involvement. Displacement of the lens is frequently seen in patients with Marfan's syndrome, an important cause of aortic regurgitation (see Fig. 10-69). Conjunctival hemorrhages are commonly seen in infective endocarditis. Hypertelorism, or widely set eyes, is often associated with congenital heart disease, especially pulmonic stenosis and supravalvular aortic stenosis. Retinal evaluation may furnish valuable information about diabetes (see Figs. 10-88 to 10-100), hypertension (see Figs. 10-101 to 10-105), and atherosclerosis. Roth's spots may develop in patients with infective endocarditis (see Fig. 10-109).

Preferential Atrophy

Myopathic process include rounded fibers, central nucleation, muscle fiber size variability, fiber splitting, segmental necrosis, muscle fiber necrosis (degeneration), cellular inflammation, myophagocytosis, regeneration (i.e., basophilic fibers), an increase in connective tissue elements, and structural abnormalities (e.g., vacuoles glycogen, lipid , ragged-red fibers mitochondrial myopathies , granulomas sarcoid , and microorganisms toxoplasmosis, trichinosis , as well as those structural changes associated with various congenital myopathies e.g., rod bodies, central cores ). 8 Centrally located nuclei may be observed in up to 3 percent of normal muscle tissue specimens, but when present in a higher percentage, this indicates an underlying myopathy. This finding is especially prominent in the muscle fibers of patients with myotonic dystrophy (see Ch.a.pie.L3.6 ). In fact, when internal nuclei appear in most of the muscle fibers of the sample, this diagnosis is strongly implicated....

Specific Indications

HIV encephalitis, opportunistic infections (toxoplasma, cytomegalovirus, herpes simplex, cryptococcus), bacterial abscess, cysticereosis, tuberculosis, sarcoidosis lesion abnormalities, and diffuse abnormalities. Mass lesions include most neoplasms and neoplastic or non-neoplastic cysts, some forms of infection (abscesses, cerebritis, tuberculomas, some encephalitides), specific vascular disorders (hemorrhages, early infarcts), malformations or developmental lesions (hamartomas, some migrational abnormalities), and a variety of miscellaneous conditions (sarcoidosis, acute multiple sclerosis, and adrenoleukodystrophy). Conditions that produce imaging study abnormalities that are not necessarily mass lesions include (1) forms of neoplasia (infiltrating gliomas, gliomatosis, lymphomas) (2) cerebrovascular disorders (vasculitides, vasculopathies) (3) infections (viral encephalitis, progressive multifocal leukoencephalopathy, CNS syphilis) (4) degenerative diseases (Creutzfeldt-Jakob...

Trauma

Pincer nail (severe form enclosing bone) Post-cryosurgery (may be prolonged bone pain) Prosector's wart (tuberculosis) Sarcoid dactylitis Subcutaneous abscess Subungual foreign body Ventral pterygium Tumours (soft tissue and bone) Aneurysmal bone cyst Bowen's disease Enchondroma Fibroma Glomus tumour Keratoacanthoma Leiomyoma Metastases Myxoid cyst Osteoid osteoma Osteoma, exostosis

Laboratory Studies

A complete cell count (CBC) with differential, urinalysis, and renal and liver function tests should be performed if asymptomatic rheumatic disease is suspected. Importantly, the frequency of abnormal laboratory results increases with increasing age in the normal population, even in the absence of disease, including common tests such as erythrocyte sedimentation rate (ESR), uric acid, antinuclear antibodies (ANAs), and rheumatoid factor (RF). Thus, arthritis panels can confuse the situation and should not be performed routinely. For example, only 80 of patients with RA have a positive RF. RF is a serum autoantibody against immunoglobulin G (IgG). Up to 4 of the healthy population has a positive RF, which is also frequently positive in patients with chronic obstructive pulmonary disease (COPD), viral hepatitis, and sarcoidosis, and can also be positive in malignancy, and primary biliary cirrhosis and other autoimmune diseases. The higher the RF titer, however, the more likely it is...

Exclusions

Sarcoidosis, preexisting lymphoma, HIV infection, hepatitis virus B or C infection, primary fibromyalgia, and other known causes of autonomic neuropathy, keratitis sicca, or salivary gland enlargement A diagnosis of definite SS requires a minor salivary gland biopsy a probable SS diagnosis can be based on demonstrating decreased salivary function. Exclusions to the diagnosis based on the San Diego Criteria include patients with HIV, primary fibromyalgia, sarcoidosis, preexisting lymphoma, keratitis sicca, hepatitis B or C, or salivary gland enlargement (Box 32-4). Objective evidence of ocular dryness can be obtained by the Schirmer II test, which involves stimulating the nasolacrimal reflex by inserting a cotton swab into the nostril the increase in tear flow is then measured for both eyes (Tsubota, 1991). Rose bengal staining of the corneal or conjunctiva epithelial layer is another objective test. Serologic evidence of SS includes an elevated RF (> 1 320), elevated ANA (> 1...

Erythema Nodosum

Erythema nodosum is an acute inflammatory process involving the fatty tissue layer underlying the skin (pan-niculitis). The condition is more frequently seen in women, and although often idiopathic, many cases are associated with streptococcal infections of the upper respiratory tract, drugs such as estrogens oral contraceptives, sarcoidosis, and inflammatory bowel disease. Other, less frequent bacterial causes include tuberculosis, brucellosis, mycoplasma, and chlamydia. Fungal infections such as blastomycosis and his-toplasmosis may also cause erythema nodosum. Rare causes are Behcet's disease, acute myelogenous leukemia, and Hodgkin's disease.

Lymphocytopenia

When a paucity of lymphocytes occurs, because most lymphocytes are T cells, it is preponderantly a reduction in the T-cell count. Lymphocytopenia is defined as a total lymphocyte count of 1000 cells L or lower. Inherited causes of lymphocytopenia include severe combined immunodeficiency, ataxia-telangiectasia, Wiskott-Aldrich syndrome, and idiopathic CD4+ T-cell lymphocytopenia. Acquired causes of decreased lymphocyte counts include mostly viral infections, such as HIV, hepatitis, influenza, or respiratory syncytial virus (RSV), and unusual bacterial infections, such as typhoid fever or tuberculosis. Miscellaneous causes include aplastic anemia, autoimmune diseases, Hodgkin's disease, sarcoidosis, chronic alcohol ingestion, immunosuppressive agents, particularly corticosteroids, and radiation.

Uveitis

Patients suspected of an anterior uveitis should be referred to an ophthalmologist for consultation and treatment. The most common cause of anterior uveitis is idiopathic other common causes include ankylosing spondylitis, inflammatory bowel disease, sarcoidosis, juvenile rheumatoid arthritis, Reiter's syndrome (urethritis, polyarteritis, and ocular inflammation), herpetic keratitis, and Lyme disease. Patients with posterior uveitis usually present with a reduction in vision and vitreous floaters. They may have clinical signs of retinal vasculitis, retinal ischemia, optic nerve edema, and exudative retinal detachment. On careful inspection, cells may be visible floating in the vitreous. Common causes of posterior uveitis are toxoplasmosis, sarcoidosis, cytomegalovirus, Epstein-Barr virus, Beh et's disease, and Bartonella infection. Toxoplasmosis accounts for up to 30 of cases and may destroy the macula or other important visual structures. Characteristically, there is an exudation in...

Laboratory Test

Infectious causes of problems, including sarcoidosis, histiocytosis X, bacterial meningitis, tuberculosis, or abscess, are typically resolved by the presence of other findings of these diseases. Lymphocytic hypophysitis and Sheehan's syndrome often occur in the peripartum. Finally, one must always be vigilant to detect prescription or recreational drugs that interfere with normal pituitary function.

Norwegian Scabies

Pictures Norwegian Scabies

Erythema nodosum is a common reaction associated with streptococcal infections, sarcoido-sis, tuberculosis, inflammatory bowel diseases, and fungal diseases. It is infrequently associated with rheumatic disorders. Patients, primarily young women, seek medical attention after the appearance of extremely painful, erythematous nodules on the lower legs, especially over the anterior tibia. The lesions range in size from 1 cm to several centimeters in diameter. The lesions can then coalesce and spread over the entire leg. The lesions of erythema nodosum begin to regress after 1 to 2 weeks. As they disappear, they undergo a series of characteristic color changes bright erythema to shades of purple, yellow, and green. Figure 8-97 shows the early lesions of erythema nodosum in a 33-year-old woman in whom sarcoidosis was diagnosed 3 months later.

Sialosis

Sialosis, also known as sialadenosis, represents a bilateral enlargement of the parotid gland that is multifactorial in its etiology (Table 6.3). It is not commonly associated with an autoimmune phenomenon, as is the case for Sjogren's syndrome and sarcoidosis, although it can easily be confused with these two pathologic processes due to its clinical presentation (Figure 6.14). Quite commonly, sialo-sis is caused by nutritional disturbances such as alcoholism, bulimia, or in the rare case of achalasia (Figure 6.15). Chronic alcoholism with or without

Lymphadenopathy

Sarcoidosis, amyloidosis Chronic granulomatous disease Histiocytic disorders Necrotizing lymphadenitis The character of the node, overall size, and relation to other lymph node groups help determine whether biopsy is indicated. When there is thoracic adenopathy, unilateral hilar adenopathy, or mediastinal adenopathy, particularly in older adults, lung cancer, sarcoidosis, or lymphoma must be strongly considered, depending on the clinical setting. Mediastinal adenopathy in young persons, however, may be associated with sarcoidosis or infectious mononucleosis. Enlarged nodes found in the abdomen or retroperitoneal space are more likely to be malignant and are characteristic of lymphomas or germ cell tumors in men.

Causes

Infiltrative diseases of the pituitary, and more frequently of the hypothalamus, can cause hypopituitarism. Sarcoidosis can present with hypopituitarism along with polydipsia and polyuria. Histiocytosis X may present as a suprasellar tumor. Lipid storage diseases and hemochromatosis can cause hypopituitarism, often with hypogonadotropin deficiency.

Neuromyelitis Optica

An occasional patient may need prone and supine myelography to exclude a spinal dural-based AVM. Laboratory investigations reveal an elevated erythrocyte sedimentation rate in one third, positive antinuclear antibodies in nearly one half, and occasionally other autoantibodies. y It is reasonable to exclude syphilis, Lyme disease, and human immunodeficiency virus by laboratory testing. A chest radiograph helps to exclude pulmonary tuberculosis and sarcoidosis. CSF examination is an essential part of the evaluation for Devic's syndrome, and repeated studies are sometimes necessary to ensure that there is no infection in that the CSF findings are sometimes atypical for inflammatory demyelination. In contrast to MS, a minority of patients with Devic's syndrome (17 percent) have a normocellular CSF during the acute phase. A marked pleocytosis is often present, sometimes exceeding 100 cells. Moreover, neutrophils are commonly seen in CSF and may predominate, a...

Optic Nerve

The most common compressive lesions causing optic neuropathies are carotid-ophthalmic artery aneurysms and sellar masses (craniopharyngioma, meningioma, or pituitary adenoma apoplexy). Primary neoplastic processes affecting the optic nerve include optic nerve gliomas (often associated with neurofibromatosis type 1), and optic nerve sheath meningiomas. Infiltrative processes (sarcoidosis, carcinomatous meningitis, leukemia, and lymphoma) nutritional

Multiple Sclerosis

The eye is the only organ outside the nervous system that is sometimes involved in MS. Uveitis and retinal periphlebitis each occur in at least 10 percent of MS patients. The uveitis can involve the posterior, intermediate (pars planitis), or rarely anterior portion and resembles that seen in other inflammatory (e.g., sarcoid, Reiter's syndrome, Behcyet's syndrome, inflammatory bowel disease, systemic lupus erythematosus) and infectious (e.g., syphilis, tuberculosis, Lyme disease) conditions. Periphlebitis is seen as venous sheathing on funduscopic examination and is histologically identical to the perivascular inflammation present in brain white matter. It is interesting that inflammation commonly occurs in the retina, which has a peripheral type of myelin produced by Schwann cells. Several systemic or organ-specific inflammatory conditions can involve the CNS white matter. Optic neuritis, myelitis, and other syndromes sometimes occur with systemic lupus erythematosus. Whether this...