Sarcoidosis: epidemiology, characteristics, and outcomes over 10 years - a single-center study in Pakistan

Abstract

ABSTRACT Background: Sarcoidosis is a multisystem granulomatous inflammatory disease which remains under-diagnosed in a tuberculosis endemic region such as Pakistan. Rationale: To determine the distribution, clinical characteristics, diagnostic and treatment modalities and the disease course in the Pakistani population. Methods: A cross-sectional review of sarcoidosis patients from Jan-1,2010 to Dec-31,2019 was done. Multivariable logistic and cox-regression models were used to identify the independent risk-factors associated with disease relapse. Kaplan–Meier curves were used to assess the DFS. Results: 222 patients, with mean age 44 ± 12 years, predominantly females (57.7%) and mean BMI 29 ± 6 were diagnosed sarcoidosis. Significant co-morbidities affected 36.5%, 90% were nonsmokers, and 50.3% belonged to moderate SES. Total 178 (80.2%) were symptomatic with 115 (51.8%) having multi-organ involvement. Stage-I radiological disease was predominant (52.5%). Histopathological diagnosis was obtained in 161 (72.5%) patients. Out of 113 mediastinal lymph-nodes, NNGI was present in 99, with highest yield in Station-07 (68.6%). Treatment was instituted in 108/178 (60.7%) symptomatic patients with steroids alone and in 26 (14.6%) with S+IS, with better clinical and radiological response duration in patients receiving steroid monotherapy (p-values=0.01 and 0.001,respectively, along with overall higher survival time (p-value = 0.04). Risk factors identified for relapse included high SES (AOR5.52;95%CI(1.10–28.40),0.04), steroid monotherapy (AOR0.22; 95%CI(0.10–0.87),0.03), symptomatic response after one year (AOR3.40; 95%CI(1.02–11.10),0.04), and radiological response duration (AOR1.10; 95%CI(1.05–1.20),0.04). Conclusion: Sarcoidosis is a dynamic disease with a variable clinical and geographical spectrum but good overall prognosis.