Severe sarcoidosis: Management and prognosis

Abstract

<b>Introduction:</b> In sarcoidosis, reduction in mortality and the prevention of disability due to major organ involvement are treatment goals. Thus, it is important to recognize severe disease. <b>Aim:</b> To study management and prognosis of severe sarcoidosis. <b>Methods:</b> Retrospective multicenter study conducted between 2000 and 2021 including patients hospitalized with severe sarcoidosis. The factors of severity retained were:pulmonary arterial hypertension (PAH), chronic respiratory insufficiency (CRI), pulmonary fibrosis, cardiac and/or neurological and/or renal localization, resistance and/or dependence to corticosteroids and chronicity. <b>Methods:</b> We enrolled 214 patients with sarcoidosis among whom 92 patients had severe sarcoidosis: 26 patients with pulmonary fibrosis,11 patients with cardiac sarcoidosis,9 patients with neurosarcoidosis ,38 patients with PAH and 8 patients with chronic sarcoidosis. The mean age was 52 years old[24-77years] with a sex ratio equal to 0.39. Systemic corticosteroid therapy was prescribed in 87% of cases over an average period of 19 months, immunosuppressive therapy in 15.2% of cases over an average period of 15 months and synthetic antimalarials drugs in 3.3% of cases. A complete clinical remission was noted in 13% of cases. A relapse was observed in 87% of our patients. It was a pulmonary relapse in 93% of cases and cardiac relapse in 7% of cases. For those patients, immunosuppressive therapy was combined with corticosteroid therapy in 53% of cases. The evolution was marked by recovery in 33% of cases, progression to CRI was in 8.7% of cases, chronicity in 51% of cases and death in 5,3% of cases. <b>Conclusion:</b> Severe sarcoidosis requires more monitoring to prevent the disease progression towards severe outcomes.