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Progressive obstructive lung disease associated with microscopic polyangiitis
O Brugiere1, O Raffy, C Sleiman
1Service de Pneumologie et Reanimation Medicale, Hopital Beaujon, Clichy, France.
Abstract:
Small airway involvement and progressive severe airflow obstruction are unexpected features in patients with microscopic polyangiitis. We report the case of a patient with microscopic polyangiitis and circulating anti-neutrophil cytoplasmic antibodies (ANCA), who developed pulmonary hyperinflation and airflow obstruction over a 7-yr period. Systemic manifestations of this vasculitis improved under corticosteriods and cyclophosphamid therapy, a treatment that did not influence either the very high level of anti-myeloperoxidase antibodies or the ventilatory impairment. Small airway involvement was suspected on the basis of pathologic small airway lesions and a mild emphysematous pattern on computed tomography (CT) scan, which was out of proportion with the severity of the obstructive lung disease.
Insights
Microscopic polyangiitis can cause unexpected severe airflow obstruction and pulmonary hyperinflation. Standard treatments improved systemic symptoms but not lung function or anti-myeloperoxidase antibodies, indicating small airway disease.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Immunology
Background:
- Microscopic polyangiitis (MPA) is a systemic vasculitis characterized by inflammation of small blood vessels.
- Pulmonary involvement in MPA typically includes diffuse alveolar hemorrhage or interstitial lung disease, not primarily small airway obstruction.
Observation:
- A patient with MPA and anti-neutrophil cytoplasmic antibodies (ANCA) presented with progressive pulmonary hyperinflation and severe airflow obstruction over seven years.
- Despite treatment with corticosteroids and cyclophosphamide, systemic vasculitis improved, but ventilatory impairment and high anti-myeloperoxidase antibody levels persisted.
Findings:
- Pathological examination revealed small airway lesions and a CT scan showed a mild emphysematous pattern.
- The extent of small airway disease was disproportionate to the severity of the obstructive lung disease observed.
Implications:
- This case highlights an unusual presentation of MPA with significant small airway involvement and progressive airflow obstruction.
- It suggests that small airway disease may be an under-recognized complication of MPA, potentially requiring different therapeutic strategies.
- Monitoring ventilatory function and considering small airway pathology are crucial in managing MPA patients with respiratory symptoms.