Uncommon manifestations of sarcoidosis

Abstract

Objective: To study the uncommon manifestations in patients of sarcoidosis. Methods: This is a prospective study of uncommon manifestations observed in 93 of the 210 biopsy proved patients (mean age 43.8 ± 6.4 years; 58 males) of sarcoidosis seen by us over the last 22 years at the All India Institute of Medical Sciences, New Delhi. Results: Clinical presentation of acute sarcoidosis in the form of Lofgren's syndrome (n = 5) was uncommonly observed. Rare pulmonary manifestations included seasonal dyspnoea with audible wheezing mimicking bronchial asthma (n = 30); narrowing of main bronchi, fixed upper airways obstruction (n = 1); pleural involvement (n = 7); bullous lung disease (n = 2); chronic respiratory failure (n = 2); and vocal cord involvement (n = 4). Other notable features were glaucoma (n = 4); digital clubbing (n = 3); sarcoid nephritis (n = 1); and sicca syndrome (n = 1). Uncommon neurological manifestations included bilateral sequential facial nerve palsy (n = 2); optic atrophy (n = 2); optic neuritis, proximal myopathy, multiple brainstem lesions, pituitary stalk lesion (one patient each). Cardiac involvement occurred in 15 patients. This included complete heart block (n = 2); congestive heart failure (n = 4); supraventricular ectopics (n = 6); ventricular ectopics (n = 2); and recurrent ventricular tachycardia requiring radiofrequency ablation (n = 1). Contrast-enhanced computerised tomographic scan (CECT scan) of the abdomen revealed intrabdominal lymphadenopathy (n = 5); infiltrates in the liver and spleen (n = 3). Portal hypertension was observed in four patients with grade IV (n = 2) and grade II oesophageal varices (n = 2). One of the patients with grade IV oesophageal varices had recurrent haematemesis and successfully underwent endoscopic sclerotherapy while another patient with grade IV varices succumbed to massive haematemesis. CECT scan was found to be useful in localising the lesions in patients with extrapulmonary sarcoidosis. Conclusion: Awareness regarding uncommon manifestations of sarcoidosis will facilitate early confirmation of diagnosis.