Introduction :

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.

Aim:

To study management and prognosis of severe sarcoidosis.

Methods :

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.

Methods :

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.

Conclusion :

Severe sarcoidosis requires more monitoring to prevent the disease progression towards severe outcomes.