A 61-year-old male with past medical history significant for sarcoidosis was admitted with acute respiratory distress. He had severe baseline lung disease with the most recent forced expiratory volume in 1 second (FEV1) at 18%. Arterial blood gas analysis revealed respiratory acidosis with a pH of 7.27 and CO2 of 57 (on 50% FiO2). Due to his level of respiratory distress, he was intubated with ventilator support. He was treated with antibiotics, steroids, and airway clearance measures. Chest X-ray (CXR) showed extensive fibrocalcific scarring in the upper lobes and compensatory emphysema of the lower lobes (Figure 1). Cultures from the endotracheal tube were negative. Viral Direct Fluorescence Antibody (Viral DFA) was negative. Echocardiogram (ECHO) showed normal left ventricular systolic function with an ejection fraction of 60%, right ventricular enlargement, diastolic dysfunction, and normal pulmonary artery pressures. Ventilation/Perfusion Scan (V/Q Scan) was negative for pulmonary embolism.
Our patient first presented with respiratory symptoms at 28 years of age. His symptoms at that time included intermittent fatigue, shortness of breath, a "sore" tongue, and "facial swelling." While spirometry was normal, his CXR revealed left hilar adenopathy and calcification. Mediastinoscopy was performed, including a mediastinal lymph node biopsy where he was found to have noncaseating granulomas. The fungal cultures and AFB (acid fast bacilli) stains by bronchoscopy were negative. The patient was diagnosed with sarcoidosis and started on prednisone for several months, to which his symptoms responded favorably. For 2 decades, he was treated with inhaled steroids and intermittent courses of prednisone. In his 40s, he presented to an ENT (ear, nose, and throat) specialist with uveitis and a hoarse voice. The ENT physician felt that the cause of the hoarse voice was iatrogenic (secondary to inhaled steroids). No biopsy was performed. At the age of 55, a cavitary lesion was detected on chest CT, and sputum cultures were positive for Aspergillus. For this reason, oral antifungal therapy was instituted.
Initial CXRs at the age of 28 showed left hilar and left paratracheal adenopathy. There were also calcified nodules in right and left lung fields. CXRs from the age of 45 through 52 showed progressive upper lobe fibrotic changes. From the age of 52 to 62, the images were stable. The CT scan during the most recent admission was stable as compared to the one done 3 years prior. It showed extensive calcification and soft tissue within the mediastinum with retraction of the mediastinum consistent with fibrosing mediastinitis. Both upper lobes had extensive calcifications and scarring. The esophagus was dilated above the fibrosing mediastinum (Figures 2 and 3). Pulmonary lung function just before admission showed an FVC of 56% FEV1 22% with a ratio of 35%. There was no postbronchodilator response. DLCO (diffusion capacity) was 69%. TLC was 62%, FRC was 75%, and RV was 69%. These values were unchanged as compared to 5 years ago. MRI of the brain showed no evidence of neurosarcoidosis.
Socially, the patient was a nonsmoker and was not exposed to any known environmental toxins which could cause interstitial lung disease. His immunizations were up to date including BCG. He was born in India and lived in Ohio at the age of 20-30 years and in Illinois thereafter. There was no known family history of lung disease.