Chest
Volume 106, Issue 3, September 1994, Pages 672-676
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Clinical Investigations: Granulomatous Disease
Necrotizing Sarcoid Granulomatosis With Pleural Involvement: Clinical and Radiographic Features

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The clinical, functional, radiologic, and pathologic characteristics of seven cases of necrotizing sarcoid granulomatosis (NSG) are presented. The population consisted of five women and two men, with an average age of 36 years. Each patient's predominant presenting complaint was pleuritic chest pain. Pulmonary function testing demonstrated a variety of abnormal patterns. Computed tomography (CT) of the chest showed solitary or multiple nodules in all patients, occasionally associated with pulmonary infiltrates in the lower lobes. Pleural involvement was seen on CT scanning in six patients and mediastinal adenopathy was present in five. Biopsy specimens of the lung lesions revealed confluent epithelioid granulomata associated with necrosis and vasculitis. Pleural involvement by confluent granulomata was a prominent feature in four patients. Follow-up has ranged from 6 months to 4 years. All patients are now asymptomatic, the majority having received prednisone. One patient received methotrexate as a steroid-sparing measure. We conclude that NSG is distinguishable from sarcoidosis as a clinicopathologic entity in which pleural involvement is a frequent finding. Treatment with steroids appears to hasten recovery.

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Materials and Methods

We report on seven patients with NSG who presented to the Division of Respirology at the University of Western Ontario from 1988 to 1992. The cases were identified through a retrospective chart review. All patients underwent open lung and/or mediastinal lymph node biopsy for diagnosis. The histologic criteria of Liebow1 were used for inclusion. Briefly, these include sarcoid-like granulomata undergoing necrosis, possibly associated with a vasculitis of granulomatous, giant cell, or lymphocytic

Results

All patients were well and were receiving no medication prior to presentation. The population consisted of five women and two men ranging in age from 24 to 57 years with a mean age of 36 years. Patient 1 represented the only smoker in the group. All patients presented for evaluation of dyspnea, fever, nonproductive cough, weight loss, and night sweats. Six of seven patients also complained of pleuritic chest pain. The duration of symptoms prior to presentation ranged from 2 to 6 months. Patient

Discussion

In the original description of NSG, Liebow1 reported on 11 patients. Since that time, an additional 75 cases have been described.2, 3, 4, 5, 6, 7, 8, 9 Patients tend to be young to middle aged with a female predominance. Symptoms are nonspecific but frequently are a combination of cough, fever, malaise, and dyspnea. A minority of patients are asymptomatic. Our patients were similar, showing a female preponderance, but with pleuritic chest pain as a major component of their clinical presentation.

ACKNOWLEDGMENT

The authors wish to thank Dr. R. Inculet for providing data on two of the patients enrolled in the study, and Sandra Reiehstein for her help in preparing the manuscript.

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