SAQ 379 – Rheumatology – GPA with pulmonary renal syndrome
Model Answer
Rheumatology – GPA with pulmonary renal syndrome.
a. GPA manifestations
- Subglottic stenosis, collapsing nasal bridge, upper airway granulomas, or pulmonary fibrosis.
- Pulmonary hypertension.
- Myalgia, arthralgia, or arthritis.
- Conjunctivitis, episcleritis, or uveitis.
- Cranial or peripheral neuritis.
- Myocarditis or pericarditis.
b. Intubation and haemorrhage treatment
| GPA | Solitary lung tumour | |
|---|---|---|
| Rationale for intubation | Primarily respiratory support. | Protect the contralateral lung from soiling; facilitate bronchoscopy/biopsy; provide respiratory support if significant aspiration of blood. |
| Treatment of haemorrhage | Immunotherapy, steroids, plasma exchange, and immunosuppressants. | Interventional therapy such as bronchoscopic injection, vascular embolisation, wedge resection, or lobectomy. |
c. Blood test
- ANCA, typically PR3 or c-ANCA.
d. Infectious causes
- Malaria.
- Leptospirosis.
- Bacterial sepsis such as tricuspid valve endocarditis, lung abscess, or Staphylococcus aureus bacteraemia.
- Influenza H1N1.
- Dengue fever.
- Hantavirus.
- Pulmonary and renal tuberculosis.
e. Non-infective causes
- Goodpasture syndrome/anti-GBM disease.
- Eosinophilic granulomatosis with polyangiitis.
- SLE.
- IgA nephropathy.
- Microscopic polyangiitis.
- Henoch-Schonlein purpura.
- Disseminated malignancy.
- Thrombotic microangiopathy such as HUS/TTP or antiphospholipid syndrome.
- Behcet disease.
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