A 42 year old male, nonsmoker, with no history of diabetes or hypertension, presented with 10 days history of shortness of breath which increased since 1 day. He was diagnosed as a case of sarcoidosis 2 months back based on clinical and radiologic findings when he had presented with history of intermittent fever, shortness of breath, loss of appetite and weight-loss for 2 months. Chest X Ray (CXR) done at that time showed diffuse nodular shadows (Fig. 1A). CT chest demonstrated diffuse micronodules with prediliction for fissures and bronchovascular bundles and associated mediastinal lymphadenopathy (Fig. 1B). MRI abdomen revealed multiple subcentimetric focal lesions diffusely scattered in liver, spleen and renal parenchyma. Serum angiotensin converting enzyme (ACE) was raised (126 U/L). Quantiferon Gold for tuberculosis was negative. Rheumatoid factor and anti nuclear antibodies were also negative. He was started on 40 mg of prednisolone which he was continuing till the time of present admission. He had symptomatic improvement before present episode.
In present admission he was febrile (101  F) with peripheral oxygen saturation of 87%. His CXR and CT chest showed left sided massive pleural effusion (Fig. 2A-C). Blood, urine and sputum cultures were sterile. Pleural fluid aspiration revealed exudative lymphocytic fluid with low adenine deaminase (ADA) (5.93 U/L). Pleural fluid polymerase chain reaction (PCR) for mycobacterium tuberculosis was negative. No cutaneous reaction was observed on tuberculin sensitivity testing. No malignant cells were seen.
In view of acute increase in breathlessness for last one day, pulmonary embolism was suspected. CT pulmonary angiography was done which showed no evidence of embolus. Pleuroscopy revealed multiple large nodules over parietal and visceral surfaces (Fig. 3A and B). Their biopsy demonstrated extensive inflammation with well formed epitheloid granulomas without necrosis. Tuberculosis cultures from biopsy, pleural fluid and bronchoalveolar fluid were negative. 3.5 L of fluid was drained over a period of 24 h.
To confirm the diagnosis of sarcoidosis, patient underwent further investigative work up. On fibreoptic bronchoscopy mucosa in left bronchial tree was grossly oedematous and irregular (Fig. 4A). Endobronchial biopsy showed noncaseating epitheloid cell granulomas with langhans cells (Fig. 4B). Transbronchial lung biopsy revealed nonspecific inflammation of interstitium with ill formed epithelioid granulomas (Fig. 4C). A diagnosis of sarcoidosis with massive pleural effusion was made.
For treatment Prednisolone was continued and Hydroxychloroquine was added. 2 months later effusion resolved completely and he remains asymptomatic.
B: CT-chest on previous admission - showing diffuse micronodules with prediliction for fissures and bronchovascular bundles.