Clinical Manifestations Of Sarcoidosis

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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.



Differential Diagnosis



Rheumatoid arthritis

Parotid gland


Sjogren's syndrome


Upper airway








Facial nerve palsy


Lyme disease



Sjogren's syndrome

abnormal chest radiograph. Four types of radiographic appearance have been described: type 0 is normal; type I shows enlargement of hilar, medi-astinal, and occasionally paratracheal lymph nodes; and type II shows the adenopathy seen in type I as well as pulmonary infiltrates (Figure 6.9). Type III demonstrates the infiltrates without the adenop-athy. Type II involvement is the most common among patients with sarcoidosis who have respiratory distress.

Two patterns of arthritis are observed in sar-coidosis, and are classified as to whether the arthritis occurs within the first 6 months after the onset of the disease, or late in the course of the disease. The early form of arthritis often begins in the ankles and may spread to involve the knees and other joints. The axial skeleton is typically spared. Monarthritis in the early phase is unusual.

Erythema nodosum, a syndrome of inflammatory cutaneous nodules frequently found on the extensor surfaces of the lower extremities, occurs in about two-thirds of patients and is strikingly associated with early arthritis. Lofgren's syndrome involves a triad of hilar lymphadenopathy, erythema nodosum, and arthritis. The late form of arthritis occurs at least 6 months after the onset of sarcoidosis, and is generally less dramatic than the early form. The knees are the most common joints to be involved, followed by the ankles. Monarthri-tis can occur in the late form of arthritis, and erythema nodosum is not commonly noted.

Other rheumatic manifestations associated with sarcoidosis include involvement of the larynx, nasal turbinates, and nasal cartilage, thereby resembling the clinical presentation of Wegener's granulomatosis (Figure 6.10). Eye involvement

Figures 6.9a and 6.9b. Posterior-anterior (a) and lateral (b) chest radiographs of a patient with type II sarcoidosis. This patient presented with severe shortness of breath.

Sarcoidosis Arthritis
Men Salivary Glands Year Old
Figure 6.10. Severe nasal cartilage involvement by sar- Figure 6.11. Bilateral parotid enlargement in a 50-year-old coidosis in this elderly woman. (Image courtesy of Dr. black woman (left > right) with a known history of sarcoid-James Sciubba.) osis. (Image courtesy of Dr. James Sciubba.)
Sarcoid Submadnibular Gladn Sarcoidosis Submandibular Gland

Figures 6.12a, 6.12b, and 6.12c. A 55-year-old man with bilateral submandibular gland swellings (a and b), as well as lower lip lesions (c). Excision of the left submandibular gland and biopsy of the lower lip swelling identified non-caseating granulomas. Additional workup identified signs consistent with sarcoidosis.

occurs in 22% of patients, with uveitis being most common (Hellmann 1993). The triad of anterior uveitis in conjunction with parotitis and facial nerve palsy has been referred to as Heerfordt's syndrome, also known as uveoparotid fever.

While salivary gland involvement seems to primarily involve the parotid gland (Figure 6.11), the submandibular gland can also be involved (Vairaktaris, Vassiliou, and Yapijakis et al. 2005; Werning 1991). The Armed Forces Institute of Pathology registry identified 85 cases of sarcoidosis. In the 77 cases in which a gland was specified, parotid involvement occurred in 65% of the cases, while the submandibular gland accounted b a c for 13% of cases (Werning 1991). Submandibular gland enlargement may occur in the absence of parotid swelling, with or without clinical evidence of minor salivary gland involvement (Figure 6.12). Minor salivary gland involvement is occasionally noted histologically in the presence of clinically apparent major salivary gland swelling (Mandel and Kaynar 1994). In fact, enlargement of the major salivary glands may be the first identifiable sign of sarcoidosis (Fatahzadeh and Rinaggio 2006). When this occurs, therefore, it is important to differentiate the parotid swelling associated with sarcoidosis from that of Sjogren's syndrome (Fol-waczny et al. 2002). Salivary gland biopsy with histopathologic examination is one means to make this distinction.

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    Can you have a saliva gland inflammed with sarcoidosis?
    14 days ago

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