Natural Scleroderma Relief
For patients with a positive ANA titer, further testing for specific nuclear antibodies can be obtained, guided by the pattern of ANA staining and the clinical findings. The interpretation of testing for specific nuclear antigens can also be difficult most of the specific antigens are not 100 specific for a particular disease and need to be interpreted in the clinical context. The anti-DNA test is highly specific for SLE, with about 95 specificity but only 50 to 60 sensitivity, and it can be used as a confirmatory test in patients with a positive ANA. Similarly, the anti-Sm (Smith) test is also highly specific for SLE, but with 30 sensitivity. Anti-SSA Ro and anti-SSS La are often used to diagnose Sjogren's syndrome but can also be found in SLE. Anti Scl-70 is found in scleroderma but is not a requirement for diagnosis.
Autoantibodies in SLE include ANA and anticytoplas-mic antibodies (including lipoproteins), antibodies against blood cells, antibodies against various organs and structures (e.g., gastric mucosa, neurons, muscle sarcolemma, thyro-globulin), and antibodies against collagen. Different antibodies against different parts of the cell nucleus appear as the four patterns of ANA staining. These include membranous antibodies against single-stranded DNA, speckled antibodies against extractable ribonucleoproteins, dsDNA antibodies against native dsDNA, nucleolar antibodies against nucleo-lar antigens and sometimes associated with scleroderma, and homogeneous antibodies against deoxyribonucleoproteins.
The technique is most commonly used for oral and nasal tumours that are not amenable to surgical resection. The prognosis is, however, still guarded and many tumours recur 6-9 months after treatment. Side effects, such as erythema of exposed skin and mucous membranes, alopecia, skin thickening and abnormal hair coloration, may also occur. Owners must therefore be given careful advice before embarking on a treatment programme so that they are fully aware of the possible outcomes.
Primary Raynaud's usually affects both hands and both feet and is not connected to another disorder. Secondary Raynaud's symptoms are part of other conditions or medications such as scleroderma, a thickening of the skin systemic lupus, chronic inflammation of the skin rheumatoid arthritis, chronic inflammation and swelling of tissue in the joints nerve problems or the side effects of heart, blood, or migraine medications.
One study has examined the effects of treatment with stanozolol (10 mg daily) or placebo for 24 weeks in primary Raynaud's phenomenon and systemic sclerosis (Jayson et al. 1991). Although 43 patients (19 Raynaud's, 24 systemic sclerosis including only 4 men) entered, only 28 patients (11 Raynauds, 17 systemic sclerosis) completed the study. Compared with placebo, stanozolol significantly improved ultrasonic Doppler index as well as finger pulp and nail bed temperatures but there was no difference in reported frequency or severity of vasospastic attacks, scleroderma skin score or grip strength. The clinical significance of the changes in digital small vessel function recorded in the absence of vasospasm and without reduction in attack rates is unclear.
Mediated diseases of the connective tissue (systemic lupus erythematosus SLE , scleroderma, polyarteritis nodosa, rheumatic fever, ankylosing spondylitis, and Reiter's syndrome) are discussed. Fig. 13. (Color Plate 9, following p. 270) Mixed connective tissue diseases (lupus, rheumatoid arthritis, scleroderma). (A) The patient had a mask facies with puckering of skin around lips and malar depigmentation. (B) The patient's hand showed ulnar deviation of the metacarpophalangeal (MP) joints as well as a taut shiny skin. Fig. 13. (Color Plate 9, following p. 270) Mixed connective tissue diseases (lupus, rheumatoid arthritis, scleroderma). (A) The patient had a mask facies with puckering of skin around lips and malar depigmentation. (B) The patient's hand showed ulnar deviation of the metacarpophalangeal (MP) joints as well as a taut shiny skin. Scleroderma Patients with scleroderma may initially develop Raynaud's phenomenon and then skin changes involving the face and hands. Facial edema...
Vasomotor disorders in the limbs include Raynaud's phenomenon, acrocyanosis, livedo reticularis, vasomotor paralysis, and erythromelalgia. Raynaud's phenomenon is the episodic, bilateral, symmetrical change in skin color (pallor, followed by cyanosis and terminating in rubor after rewarming) that is provoked by cold or emotional stimuli. This response is due to episodic closure of the digital arteries. There is, however, no consistent evidence of exaggerated sympathetic outflow to the skin. Raynaud's phenomenon may be associated with connective tissue disease (e.g., scleroderma, rheumatoid arthritis, psoriasis), occupational trauma (such as the use of pneumatic hammers, chain saws producing vibration), the thoracic outlet syndrome, the carpal tunnel syndrome, or certain drugs (e.g., beta blockers, ergot alkaloids, methysergide, vinblastine, bleomycin, amphetamines, bromocriptine, and cyclosporine). y
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.
SS is seen in 0.5 to 2.0 percent of the population among the collagen vascular diseases, it is second in prevalence only to rheumatoid arthritis. 107 Ninety percent of patients are women. Onset usually occurs during middle age. 107 Genetic susceptibility is suggested by an association with HLA-B8, HLA-DR3, HLA-DR2, and HLA-DRw52. SS occurs in isolation in 50 percent of cases and in the context of rheumatoid arthritis, systemic lupus erythematosus, scleroderma, or another connective tissue disease in the remaining patients. 107
Differential diagnoses include other vasculopathies and autoimmune diseases such as systemic lupus erythematosus, scleroderma, polyarteritis nodosa, antiphospholipid antibody syndrome, GCA or TA. Other conditions such as pseudoxanthoma elasticum and Ehlers-Danlos may be considered as well. It is important to exclude proximal sources of emboli and atherosclerosis. y
A Department for Work and Pensions (2005) review concluded that there was consistent evidence that the inhalation of VCM in PVC production workers causes a characteristic clinical triad of osteolysis of the terminal phalanges, scleroderma, and Raynaud's phenomenon, but not all three are invariably present together (Department for Work and Pensions 2005). These effects occurred in workers who had been exposed to levels of VCM very much higher than the current control limits. Surveys of factory workforces have shown that among those exposed to VCM who do not have radiological evidence of osteolysis, the prevalence of Raynaud's phenomenon and scleroderma is greater than in the general population (by a factor of two). The mechanisms of toxicity for non-cancer VCM effects are not completely elucidated. VCM disease exhibits many characteristics of autoimmune diseases (e.g. Raynaud's phenomenon and scleroderma). B-cell proliferation, hyperimmunoglobulinemia, and complement activation, with...
In a patient with a possible inflammatory myopathy, the diagnosis is strengthened or confirmed by serum CK, electromyography, and most important, muscle biopsy to help exclude overlap syndromes. ESR is neither a sensitive nor a specific indicator of PM or DM but does correlate in general with inflammatory diseases. Testing for autoantibodies such as antinuclear antibodies and anti-Sm (SLE), SS-A and SS-B (Sjogren's), PM-Scl and anti-Ku (scleroderma), anticentromere (CREST syndrome), and anti-U1snRNP (MCTD and SLE) may be useful in considering the overlap syndromes.
Scleroderma, or progressive systemic sclerosis, is a chronic multisystem disease manifested by thickening of the skin and varying degrees of organ involvement. There is a broad spectrum of disease manifestations of scleroderma, ranging from limited skin lesions associated with calcinosis, Raynaud's phenomenon, esophageal motility problems, sclerodactyly, and telangiectasia (CREST variant) to full encasement of the body by diffuse sclerosis. Calcification of the soft tissues can produce a stony-hard tissue and can range from a small area of involvement to
One or more additional features of the POEMS syndrome often are observed, but in many cases, these are not prominent and must be carefully sought. Hepatomegaly may be found in up to 50 percent of patients, whereas lymphadenopathy and splenomegaly are less common. Manifestations of endocrinopathy include diabetes mellitus, hypothyroidism, impotence, gynecomastia, testicular atrophy, amenorrhea, hyperprolactinemia, and hyperestrogenemia. Skin changes include hyperpigmentation, thickening of the skin, hypertrichosis, skin angiomas, clubbing, and white nails. Some skin changes are suggestive of scleroderma. Peripheral edema, ascites and pleural effusions may also be seen. POeMs syndrome, sometimes referred to as Crow-Fukase syndrome, is not unique to osteosclerotic myeloma. It has been observed in multiple myeloma, Waldenstrom's macroglobulinemia, and angiofollicular lymph node hyperplasia, also referred to as Castleman's disease.y
The number of involved joints and presence or absence of symmetry are criteria for further diagnosis of articular pain (Figs. 32-1 and 32-2). Monoarticular (one joint) or oligoarticular (several joints) arthritides can be caused by conditions such as osteoarthritis (OA), gout, pseudogout, or septic arthritis. Asymmetric polyarthritis occurs in ankylosing spondylitis, psoriatic arthritis, Reiter's disease, and spondyloarthropathies. Symmetric arthritis, meaning that the same joint is affected on the contralateral side but not necessarily to the same degree, is characteristic of rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren's syndrome, polymyositis, and scleroderma. Fibromyalgia, reflex sympathetic dystrophy, and predominantly psychological
Dispose tissue to scarring or nerve damage must be taken into consideration when designing splints and splint wearing schedules. These variables may include, but are not limited to, therapeutic intervention factors, such as medication or radiation systemic diseases like peripheral vascular disease, poor nutritional status, diabetes mellitus, rheumatoid arthritis, scleroderma, or Raynaud's disease use of tobacco, alcohol, other substances including the effects of many widely available herbal supplements that may effect coagulation or wound healing and the possibility or presence of infection. Patients with associated risk factors that undermine tissue nutrition or viability require splints that disperse pressure and minimize shear through contiguous fit over large contact areas. Narrow splint components and constricting circumferential straps are to be avoided. If used, mobilizing forces should be cautiously monitored to ensure that force magnitude is gentle and force angle of...
Other conditions usually considered with this group of diseases are other autoimmune or inflammatory states such as polyarteritis nodosa, rheumatoid arthritis, allergic granulomatosis, hypersensitivity vasculitis, scleroderma, or parasitic infections. History, organ involvement, serologic studies, and tissue biopsy may be necessary to distinguish among these conditions.
Digital display of a magnetic audio recording taken at the xiphoid area of the sternum from a patient with severe pulmonary hypertension secondary to scleroderma with right ventricular decompensation. During inspiration (identified by the noise in the baseline) an S3 is seen clearly, which is not seen during expiration, confirming the right-sided origin of the sound. Fig. 40. Digital display of a magnetic audio recording taken at the xiphoid area of the sternum from a patient with severe pulmonary hypertension secondary to scleroderma with right ventricular decompensation. During inspiration (identified by the noise in the baseline) an S3 is seen clearly, which is not seen during expiration, confirming the right-sided origin of the sound.
Other autoimmune diseases with pulmonary manifestations include mixed connective tissue disease (MCTD), Sjogren's syndrome, and progressive systemic sclerosis (scleroderma). Progressive systemic sclerosis can manifest with thickening and tightening of the skin of distal extremities, sclerodactyly, Raynaud's phenomenon, and multiorgan involvement of lungs, skin, kidney, heart, and gastrointestinal tract. Sixty percent of patients have shortness of breath others have cough, pleuritic chest pain, or hemoptysis. Lung CT scan in progressive systemic sclerosis and other autoimmune conditions can show pleural scarring and ILD, especially a ground-glass fibronodular pattern and honeycombing.
Thoracic patients often exhibit the respiratory symptoms of cough, sputum, haemoptysis, breathlessness, wheeze and chest pain, or oesophageal symptoms of dysphagia, pain and weight loss. Other common chest features are hoarseness, superior vena cava obstruction, pain in the chest wall or arm, Horner's syndrome, cyanosis and pleural effusion. Lung tumours may cause extratho-racic symptoms by metastatic spread, principally to brain, bone, liver, adrenals and kidneys, or by endocrine effects such as finger clubbing, hypertrophic pulmonary osteoarthropathy, Cushing's syndrome, hypercalcaemia, myopathies (e.g. Eaton-Lambert syndrome), scleroderma, acanthosis and thrombophlebitis.
The nomenclature for the disease differs somewhat, but in general it is called Raynaud disease, or primary Raynaud phenomenon, if these symptoms occur without evidence of any other associated disease process. In contrast, secondary Raynaud phenomenon occurs when the symptoms occur in association with a related disease process such as systemic lupus erythematosus or scleroderma.
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.
Scleroderma associated with Raynaud's phenomenon, disseminated lupus erythematosus, and causalgia of the median nerve. The condition may be idiopathic, congenital, and familial or acquired. A congenital, aberrant, painful hyponychium has been described associated with oblique, deep fractures of the nails. In one case an unusual acquired association of pterygium inversum unguis and lenticular atrophy of the palmar creases was recorded.
Attack (often triggered by cold or stress), one or more digits turn white. Some minutes later, the color changes to bluish red. Normal color returns slowly. In severe cases, gangrene may ensue in fingers or toes. Once known as relapsing gangrene, Raynaud's syndrome may occur alone (Raynaud's disease) or with another condition such as scleroderma, lupus, or rheumatoid arthritis. It may be an occupational hazard for people who operate vibratory machinery such as jackhammers.
Some lesions are obviously benign or malignant. For example, a dense, spiculated mass with surrounding distortion, skin thickening and clustered, irregular micro-calcifications in a 70-year-old is almost certainly malignant, whereas a smooth, oval, well-defined isodense mass with popcorn calcification in a 35-year-old is most likely to be a fibroadenoma.
Scleroderma, also known as progressive systemic sclerosis, is an important rheumatic disease characterized by hardening of the skin. Vascular changes occur with visceral involvement and involve the microvessels and small arteries. The onset of the disease is often heralded by the development of Raynaud's phenomenon, which is discussed in Chapter 15, The Peripheral Vascular System. The cutaneous manifestations of scleroderma involve tightening of the skin, especially on the face and hands. As a result of tendon contractures, flexion of the fingers results. The fingers of a patient with scleroderma are shown in Figure 8-95 notice that the skin is bound tightly and obscures the superficial vasculature. Skin lines are absent. Figure 8-96 shows the face of the same patient. Notice the tightening and wrinkling of the skin around her mouth and the fixed, expressionless countenance as a result of flattening of the nasolabial folds. The patient had great difficulty in opening her mouth. The...
Patients with Raynaud's phenomenon have reversible digital artery spasm precipitated by cold or emotional stress. The digits become pallid, then blue, and on rewarming or relief of the emotional stress become hyperemic. It is most commonly associated with collagen vascular disease (scleroderma or disseminated lupus erythematosus), but may also be associated with Buerger's disease, primary pulmonary hypertension, or thoracic outlet syndromes (81). Patients with scleroderma and Raynaud's phenomenon may show digital ischemia, fingertip necrosis (rat-bite lesions), and even autoamputation (82).
Parkinsonism, scleroderma, or myotonia dystrophica, whereas a vacant expression suggests Down syndrome. Marfan and Ehlers-Danlos syndromes are associated with aortic aneurysms rupture. Sleep apnea, sarcoidosis, scleroderma, and tricuspid regurgitation are associated with pulmonary hypertension. The Shy-Drager syndrome gives rise to postural hypotension. Myocardial and or pericardial disease will be the cardiac lesion to be considered when physical signs suggest some of the following disorders hemochromatosis, muscular dystrophy, Friedreich's ataxia, hyperthyroidism, amyloidosis (cardiomyopathy), rheumatic fever, Reiter's syndrome (myocarditis), scleroderma, dermato-myositis, sarcoidosis (pericarditis), and myxedema (pericardial effusion and possibly tamponade).
The term filariasis refers to several diseases of both humans and animals caused by infection with a specific group of parasitic nematodes called filarial worms (named for the hairlike appearance of the adult form). Those worms that affect humans belong to the Order Filarioidea, Family Dipetalonematidae. They include (1) Wuchereria bancrofti and Brugia malayi, which are common causes of elephantiasis (extreme swelling and skin thickening of the legs, scrotum, labia, or arms) and chyluria (lymph and emulsified fat globules in the urine) (2) Loa loa, the eye worm and (3) Onchocerca volvulus, the cause of onchocerciasis. Depending upon their species, adult filarial worms of both sexes reside in the lymphatic system, subcutaneous tissues, or peritoneal and pleural cavities. Sexual reproduction results in embryos (microfilariae) that enter blood or skin, where they are ingested by a particular intermediate host (certain species of mosquitoes, horse fly, black fly, or other arthropods). The...