[Vasculitis and diffuse panbronchiolitis-like in systemic lupus erythematosus--case report].

Area of Science:

• Rheumatology
• Pulmonology
• Pathology

Background:

• Systemic lupus erythematosus (SLE) commonly involves renal and cutaneous systems, but pleuro-pulmonary manifestations, including diffuse alveolar damage and hemorrhage, pose significant management challenges.
• Pulmonary involvement in SLE exhibits distinct morphological and clinical differences between pediatric and adult populations, influenced by immunocompetence and treatment regimens.

Observation:

• A 16-year-old male initially presented with symptoms mimicking Epstein-Barr virus (EBV) infection, including asthenia, malaise, and cervical adenopathies.
• Persistent symptoms over eight months led to the emergence of a malar rash, fever, dyspnea, and bibasilar crackles, prompting further investigation.
• Initial lymph node biopsy showed follicular hyperplasia with negative EBV immunostaining, while subsequent lung biopsy revealed bronchovascular lesions consistent with vasculitis and bronchiolitis.

Findings:

• Pulmonary biopsy demonstrated intense macrophage infiltration (CD68+) and intra-alveolar macrophages with septal compromise, indicative of inflammatory lung disease.
• Computed tomography (CT) scans revealed diffuse micronodules throughout the lung parenchyma, a pattern that resolved following corticosteroid therapy.
• Diagnosis of SLE was confirmed by positive serological markers including antinuclear antibodies (ANA), anti-double-stranded DNA (anti-dsDNA), anti-nDNA, and anti-histones antibodies.

Implications:

• This case represents the first reported instance of pulmonary SLE involvement presenting primarily as vasculitis and a diffuse panbronchiolitis-like pattern.
• Early recognition of pulmonary manifestations in SLE is crucial, as they can be the initial clinical sign, preceding other characteristic symptoms.
• The findings underscore the importance of considering SLE in adolescents with unexplained pulmonary disease, even with initial misdiagnosis, and highlight the efficacy of prompt corticosteroid treatment.