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Case Sixty Three - Sarcoidosis

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Clinical History: Chronic shortness of breath.

Findings: Classically divided into stages for descriptive purposes.

This case demonstrates classic findings such as marked bilateral hilar adenopathy with associated paratracheal adenopathy. In addition, there is diffuse pulmonary abnormality with an upper zone predominance (which is not uncommon). The interstitium demonstrates a reticulonodular pattern with associated traction bronchiectasis and the development of upper lobe bulla.

Diagnosis: Sarcoidosis.

Discussion: Sarcoidosis is a disease of unknown etiology characterized by granulomatous inflammation of multiple organ systems with a high predilection for the lungs. The first manifestations of the disease occur in the age group 20-40 with a definite female and black predominance.

Symptoms develop in approximately 50% of those afflicted with the disease. Of those with symptoms, the most common are weight loss, fatigue, and malaise. With progression of the disease to involve the pulmonary parenchyma, dry cough and shortness of breath may develop.

The earliest radiographic manifestations generally consist of bilateral hilar adenopathy with associated right paratracheal adenopathy - the so-called "1,2,3 sign" on chest x-ray. Approximately 80% of those with hilar adenopathy (with or without associated visible pulmonary abnormality) will eventually show complete radiographic resolution. It is generally agreed that approximately 20% of patients will go on to develop irreversible pulmonary fibrosis with associated bronchiectasis, bulla formation, and emphysema. When severe, pulmonary hypertension and cor pulmonale may ensue. The prognosis in patients with sarcoidosis varies considerably, but it is generally thought that the overall mortality rate ranges from 5-10% with most of these patients dying from cardiac decompensation secondary to pulmonary fibrosis.

References:
Fraser, Pare: Synopsis of Diseases of the Chest. pp 829-844. W. B. Saunders, Co.

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Submitted by:
Vince Keiser, M.D.
Joseph P. LiPuma, M.D.