Pulmonary Scleroderma, Clinical Manifestations, and Associated Radiological Patterns: A Case Report

Abstract

Systemic sclerosis or scleroderma, as a systemic disease, typically starts as a limited skin condition (manifesting as cutaneous scleroderma or morphea) before progressing to internal organs, primarily the gastrointestinal tract and lungs. In the latter, the disease finds its primary cause of death. It is crucial to understand the patterns of disease progression to prevent irreversible damage in affected patients.

We present a case report of a 40-year-old adult patient, diagnosed with scleroderma 10 years ago, who seeks consultation after a long period of absence due to a worsening of his initially mild respiratory symptoms. The patient had been absent from consultations since 2019 due to the
pandemic. He currently reports worsening of his usual dyspnea (from functional class 1 to functional class 3 at present), associated with a cough. He presents typical skin stigmata of the disease but denies dyspnea at rest, dysphagia, reflux, or other symptoms suggestive of involvement in other organs. Spirometry demonstrated a restrictive pattern consistent with interstitial lung disease. The computed tomography scan at the pleuropulmonary level shows a subpleural reticular pattern, with patchy ground-glass opacities and traction bronchiectasis observed in both lower lobes in their posterior sectors and the internal segment of the middle lobe, related to interstitial disease. The radiological pattern of the interstitial disease is characterized as “NSIP” (nonspecific interstitial pneumonia), a characteristic but nonspecific pattern of this pathology. In consultation between the pulmonology and rheumatology services, treatments with progressive doses of mycophenolate and bosentan were established with good clinical progression.

The main differential diagnoses are idiopathic pulmonary fibrosis and interstitial lung disease associated with rheumatoid arthritis. The history of his diagnosis and the distribution of lesions are the main supporting elements for this diagnosis, which otherwise would require serological and histopathological studies. It is of utmost importance to correctly interpret radiological patterns in patients with interstitial diseases to ensure appropriate treatment and improve the quality of life for these highly disabling diseases.