Granulomatosis with polyangiitis - pulmonary

Case contributed by Dr Ian Bickle

Presentation

Intermittent hematuria and hemoptysis. No arthralgia. Anti-MPO and Anti-PR3 postive.

Patient Data

Age: 60 years
Gender: Male

Multiple focal areas of consolidation in both lungs measuring upto 2 cm, most of which are subpleural in location.  Several are wedge shaped and have a small amount of surrounding groundglass opacification.

Minor right apical bronchiectasis.

No endobronchial lesion.

No mediastinal lymphadenopathy.

CT-guided lung biopsy

CT

Right upper lobe subpleural 1.4 cm lesion targeted.

20 G core biopsy x 2 taken.

The patient has hemoptysis immediately after the biopsy. Several clots (1-3 mL).

3 mm post procedural pneumothorax.

 

Lung biopsy histology

Pathology

LUNG BIOPSY RESULT:  Granulomatous inflammation with polyangiitis

Case Discussion

Granulomatosis with polyangiitis (GPA) is a multisystem necrotizing non-caseating granulomatous vasculitis involving the small to medium-sized arteries, capillaries, and veins. It most commonly affects the respiratory system and kidneys as in this case.

This patient also had laboratory evidence of renal impairment and a renal biopsy identified concurrent renal involvement.

Four types of pulmonary involvement are recognized, one of which is typified by peripheral wedge-like consolidation as illustrated in this case.

The post lung biopsy hemoptysis self-resolved.   Immunosuppressive treatment was commenced.

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