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Case Fifty Four - Cavitary Sarcoidosis

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Clinical History: 25 year old African American female with six month history of fever, night sweats and weight loss.

Findings: The chest CT shows multiple nodular opacities throughout the lungs. There is evidence of cavitation in several of these opacities. The mediastinum shows evidence of mediastinal and hilar adenopathy.

Diagnosis: Cavitary Sarcoidosis.

Discussion: Sarcoidosis is a common systemic disease of unknown etiology. Various agents have been cited in the pathogenesis of the disorder, including mycobacterial infection, pollen, and inorganic dusts. Pathologically, there is initially a mononuclear infiltrate of cells, followed by the development of mature granulomas of epithelioid cells, macrophages, and multinucleated giant cells. Characteristically, the granulomas distribute along lymphatic pathways.

Sarcoidosis is seen in blacks ten times more commonly than whites, and females are affected two to three times more commonly than males. Patients present with fever, anemia, arthralgias and skin manifestations such as erythema nodosum. Hypercalcemia is seen in 10-20% of patients, and Angiotensin Converting Enzyme (ACE) levels are seen in 60-70% of patients.

The lung is the most commonly affected organ in sarcoidosis. Stage I disease is seen in 45-65% on presentation and consists of isolated lymphadenopathy, commonly involving the right paratracheal region and bilateral hila. Stage II connotes involvement of both parenchyma and adenopathy, while Stage III is isolated to the parenchyma. Stage IV, or end stage sarcoid, refers to the extensive fibrosis and honeycombing seen in late sarcoidosis. Tracheobronchial involvement is seen in 25% of patients, while eggshell calcifications of lymph nodes are seen in 5%. Pleural effusions are quite uncommon, and occur in less than 2%.

Nodular sarcoid is seen in a small percentage of patients, and cavitary sarcoid is extremely rare, with 10 cases in the literature. The cavities range in size from 3-8 cm and are usually thick walled. Patients may be asymptomatic, but life threatening hemoptysis has been reported. The cavitation is felt to probably result from ischemic necrosis of the granulomas, though others postulate infarction secondary to a granulomatous angiitis. It is important to distinguish cavitary sarcoid from cavitary infection, cystic bronchiectasis, and mycetoma formation, common complications of pulmonary sarcoidosis.

References:
Sharma,OP. Sarcoidosis. Unusual Pulmonary Manifestations.
Postgraduate Medicine 1977;61:67-73.

Rohatgi PK and Schwab LE. Primary Acute Pulmonary Cavitation in
Sarcoidosis. AJR 1980;134:1199-1203.

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Submitted by:
R.C. Gilkeson, M.D.
Michelle Garnett, M.D.