Therapeutic challenges in a case of sarcoidosis

Abstract

Sarcoidosis is a chronic systemic inflammatory disease with unknown etiology, whose diagnosis is based on suggestive symptoms/imaging, histopathological examination with non-caseous granuloma, and exclusion of other etiologies. Corticosteroid therapy is the treatment of choice for moderate/severe forms, but medication intolerance or unfavorable evolution may require immunomodulatory therapy. A 40-year-old patient known with poly-allergy was hospitalized for resting dyspnoea, dry cough, night sweats, and itching. Objective examination identifies roughened breathing, wheezing and SpO₂ = 98%. Spirometry shows a medium mixed ventilatory dysfunction. Computed tomography (CT) describes multiple mediastinal lymphadenopathies and inhomogeneous hepatomegaly. Osteomedullary biopsy refutes the suspicion of lymphoma. Bronchoscopy detects bronchial hypervascularization. Due to the suggestive imaging context, a transbronchial biopsy was performed (EBUS-TBNA) from the mediastinal lymphadenopathy. Histopathological examination suggested sarcoidosis: granulomas with epithelioid histiocytes, without caseous necrosis, associated with Langerhans cells and lymphocytes. We initiated corticosteroid therapy, but the patient had an allergic reaction that required the interruption of treatment and administration of methotrexate. After eight months, the clinical and imaging aspect worsened, and we replaced the treatment with cyclophosphamide. However, side effects occurred after a few months, it was replaced with azathioprine. The patient’s condition deteriorates significantly with desaturation. We requested a consultation with the ‘Sarcoidosis Center’ in Rotterdam, which recommended treatment with anti-TNF-α or human immunoglobulin. The patient shows a slightly favorable evolution under human immunoglobulin. The poly-allergy and drug intolerance, including corticosteroid therapy, led to the initiation of immunomodulatory therapy for a young patient with moderate/severe sarcoidosis. The choice of medication was difficult due to the side effects.