Interstitial lung disease associated with diffuse systemic sclerosis. Clinical case description.

Abstract

Introduction: Systemic sclerosis is a heterogeneous, multisystemic, autoimmune connective tissue disease of unknown etiology, characterized by extensive cutaneous fibrosis, vascular alterations, and autoantibodies against various cellular antigens. It presents significant morbidity and the highest mortality rates among autoimmune rheumatic diseases. It is a rare disease with a difficult diagnosis. In the United Kingdom, the prevalence is 8.2 cases per 100,000 inhabitants.

Case Report: A 31-year-old man with a history of hypothyroidism, type 2 diabetes mellitus, epilepsy, hypertension, and severe intellectual disability was evaluated for skin hardening in the hands, arms, and neck, with the presence of redness, edema, and warmth in the right cheek and masseter region, cellulitis was diagnosed. During hospitalization, he developed type 2 respiratory failure requiring intubation. Pneumonia was suspected, and a chest CT scan showed an interstitial fibrosis pattern. Management was established with carboxypenicillins. All cultures and infectious tests were negative. Anti-DNA topoisomerase I (Scl-70) antibodies >200 confirmed the diagnosis of scleroderma, and oral corticosteroids and mycophenolate were initiated.

Conclusions: In this case, the identification of skin signs and pulmonary involvement were the specific details to identify scleroderma with pulmonary involvement. The prognosis in this case is determined by interstitial fibrosis > 20-30%, FVC <70%, DLCO <50%, and the presence of pulmonary hypertension.