Vasculite e padrão de panbronquiolite difusa no lúpus eritematoso sistémico – Caso clínico

Vasculitis and diffuse panbronchiolitis-like in systemic lupus erythematosus – Case report

 

Lina Carvalho¹

Sara Freitas²

 

Resumo

O compromisso visceral pelo lúpus eritematoso sistémico (LES) estende-se para além do rim e da pele. Lesões pleuropulmonares são reconhecidas e as formas de destruição alveolar difusa e hemorragia alveolar são as mais difíceis de controlar. O compromisso pulmonar na evolução clínica do LES difere nas crianças e nos adultos, tanto nos padrões morfológicos como nas apresentações clínicas, dependendo da imunoincompetência do doente e do tratamento instituído.

Um rapaz de 16 anos apresentou um quadro clínico de astenia, cansaço e pequenos gânglios linfáticos cervicais bilaterais e não dolorosos, entendido como infecção pelo EBV, com serologia concordante (IgG e IgM de EBV e EBNA positivos). Os sintomas persistiram durante oito meses e progressivamente instalou-se eritema nasal e malar, discreto e descamativo e também febre persistente, dispneia e estertores basais à auscultação. Foram efectuadas biópsia de um gânglio linfático cervical e biópsia cirúrgica pulmonar.

Observou-se hiperplasia folicular no gânglio linfático e ausência de células LMP1 (EBV) positivas. Na biópsia pulmonar eram evidentes fenómenos de bronquiolite e vasculite à custa de células macrofágicas identificadas pelo marcador CD68. Os macrófagos dissociavam as paredes vasculares e bronquiolares e também estavam presentes nos septos interalveolares peribroncovasculares e nos espaços alveolares, observando-se assim um padrão de panbronquiolite difusa e vasculite. Não se identificaram células LMP1 (EBV) positivas.

O padrão pulmonar micronodular bilateral observado na TAC resolveu com corticoterapia. O diagnóstico de LES foi confirmado pela positividade dos anticorpos ANA, anti-dsDNA, anti-nDNA e anti-histonas.

Este é o primeiro caso divulgado na literatura médica de compromisso pulmonar sob a forma de vasculite e padão de panbronquiolite difusa como primeira manifestação clínica do lúpus eritematosos sistémico.

Palavras-chave: LES, bronquiolite, vasculite, panbronquiolite difusa.

 

Abstract

Visceral involvement in systemic lupus erythematosus (SLE) extends beyond renal and cutaneous management. Pleuro-pulmonary lesions have been recognised and diffuse alveolar damage and hemorrhage are the most difficult patterns to control.

Pulmonary compromise in clinical evolution of SLE differs from children to adults, both in morphological patterns and in clinical presentation, depending on immunocompetence and the treatment prescribed.

A 16-year-old boy presented asthenia, malaise and bilateral cervical painless adenopathies understood as EBV infection as serological EBV IgG, IgM and EBNA were positive. The symptoms persisted for eight months when discrete erythematous and desquamative nasal and malar rash expressed together with persistent fever, dispnoea and bibasilar crackles. Lymph node and pulmonary biopsises were performed.

Lymph node presented follicular hyperplasia and LMP1 (EBV) immunostaining was negative. In lung biopsy bronchovascular lesions were consistent with vasculitis and bronchiolitis due to intense macrophage infiltration, validated with CD68 antibody and intra-alveolar macrophages were also present with septal compromise; LMP1 (EBV) positive cells were not visualized.

The lung pattern seen in CAT as diffuse micronodules all over the lung parenchyme resolved after corticosteroid therapy. The diagnosis of SLE was confirmed by ANA, anti-dsDNA, anti-nDNA and anti-histones positivity.

To the best of our knowledge this is the first reported case of pulmonary SLE involvement with vasculitis and diffuse panbronchiolitis – like pattern as the first clinical sign of the disease.

Key-words: SLE, bronchiolitis, vasculitis, diffuse panbronchiolitis.

 

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¹ Professora de Anatomia Patológica. Faculdade de Medicina da Universidade de Coimbra

² Pneumologista Eventual. Hospitais da Universidade de Coimbra

 

Recebido para publicação/received for publication: 06.11.18

Aceite para publicação/accepted for publication: 06.12.19