History
- 36-year-old male
- Nasal bleeding
- Haemoptysis
- Cough
- Headache
- Ocular proptosis
- Duration: 1 month
- Chest X-ray: Multiple cavitary lesions in both lungs
Examination
- ENT involvement likely
- Possible ocular inflammation
- Respiratory findings depending on pulmonary disease extent
Investigations
- Chest X-ray: Multiple bilateral cavitary lesions
- ANCA testing
- CT chest
- Inflammatory markers
- Biopsy if required
MCQ Questions
Q1. Which is NOT a differential diagnosis?
A. Pneumonia/Lung abscess
B. Pulmonary infarct
C. Granulomatosis with polyangiitis
D. Lobar pneumonia
Q2. Most likely diagnosis?
A. Sarcoidosis
B. Granulomatosis with polyangiitis
C. Lung cancer
D. Progressive massive fibrosis
Q3. Least expected physical finding?
A. Proptosis and diplopia
B. Saddle-shaped nose
C. Finger clubbing
D. Mucosal ulceration
Q4. Most specific investigation?
A. CBC with ESR & CRP
B. cANCA / pANCA
C. CT chest
D. Sputum culture
Q5. NOT a recognised management option?
A. IV immunoglobulin
B. Plasma exchange
C. Broad-spectrum antibiotics alone
D. Rituximab
Brief Case Summary
A young male presented with upper airway symptoms, pulmonary cavitary lesions, haemoptysis, and orbital involvement suggesting a systemic vasculitic disorder affecting small to medium-sized vessels.Answer
Click to Reveal Answers
- Q1 = D. Lobar pneumonia
- Q2 = B. Granulomatosis with Polyangiitis (Wegener's)
- Q3 = C. Finger clubbing
- Q4 = B. cANCA / pANCA
- Q5 = C. Broad-spectrum antibiotics alone
Answer Explanation
Granulomatosis with polyangiitis (GPA) is an ANCA-associated necrotizing vasculitis characterized by upper respiratory tract disease, pulmonary nodules/cavitations, orbital involvement, and renal disease. cANCA directed against PR3 is highly suggestive.Why Not Others?
- Sarcoidosis rarely causes multiple cavitary lung lesions.
- Lung cancer usually does not explain ENT manifestations and proptosis together.
- Progressive massive fibrosis occurs in occupational lung disease.
- Broad-spectrum antibiotics alone do not treat autoimmune vasculitis.
5 Brief Case Scenarios
- Chronic sinusitis, haematuria, positive cANCA.
- Saddle nose deformity with pulmonary nodules.
- Orbital pseudotumor with recurrent epistaxis.
- Rapidly progressive glomerulonephritis with lung infiltrates.
- Haemoptysis and cavitary lung lesions in a young adult.
Pathophysiology Simplified
Autoantibodies activate neutrophils, causing inflammation and destruction of small and medium blood vessels. Granuloma formation leads to tissue injury in the upper airway, lungs, eyes, and kidneys.Physical Examination Pearls
- Epistaxis is common.
- Nasal crusting and ulcers may occur.
- Saddle nose deformity is classic.
- Proptosis suggests orbital involvement.
- Signs of renal disease should be assessed.
Investigation Findings
- Positive cANCA (PR3-ANCA)
- Raised ESR and CRP
- Pulmonary nodules and cavitations
- Urinary RBC casts if kidney involvement
- Biopsy showing necrotizing granulomatous vasculitis
Complications
- Diffuse alveolar hemorrhage
- Renal failure
- Visual loss
- Respiratory failure
- Relapsing disease
Management
- Rituximab
- Corticosteroids
- Cyclophosphamide
- Plasma exchange in selected cases
- Maintenance immunosuppression
Differential Diagnosis
- Tuberculosis
- Lung abscess
- Septic emboli
- Pulmonary infarction
- Fungal infection
- Lung malignancy
Clinical Pitfalls
- Misdiagnosing as pulmonary tuberculosis.
- Ignoring ENT symptoms.
- Delaying ANCA testing.
- Missing renal involvement.
- Treating only with antibiotics.
Clinical Pearls
- Think GPA when ENT and lung disease coexist.
- Multiple cavitary lesions are highly suggestive.
- cANCA is strongly associated.
- Kidney involvement may develop later.
- Early treatment improves outcomes.
Monitoring & Follow-Up
- Serial ANCA levels
- Renal function monitoring
- Urinalysis
- Chest imaging
- Drug toxicity surveillance
Prognosis
With modern immunosuppressive therapy, most patients achieve remission. Relapses are common, requiring long-term follow-up.FAQ
1. What is GPA?
ANCA-associated necrotizing vasculitis.
2. Former name?
Wegener's granulomatosis.
3. Which ANCA is common?
cANCA (PR3).
4. Common ENT symptom?
Epistaxis.
5. Typical lung finding?
Cavitating nodules.
6. Does it affect kidneys?
Yes.
7. Can it cause haemoptysis?
Yes.
8. Can eyes be involved?
Yes.
9. Is biopsy useful?
Yes.
10. First-line therapy?
Rituximab or cyclophosphamide.
11. Are steroids used?
Yes.
12. Is it infectious?
No.
13. Is relapse common?
Yes.
14. Can it be fatal?
Untreated disease may be fatal.
15. Is long-term follow-up needed?
Yes.
References
- Harrison's Principles of Internal Medicine
- Davidson's Principles and Practice of Medicine
- Oxford Handbook of Clinical Medicine
- EULAR Vasculitis Guidelines
- American College of Rheumatology Guidelines
Keywords
Granulomatosis with Polyangiitis Wegener's Granulomatosis GPA Quiz ANCA Vasculitis cANCA Positive Pulmonary Cavitary Lesions Haemoptysis Causes Epistaxis Quiz Internal Medicine MCQ Chest Xray Quiz Rheumatology Quiz Pulmonary Vasculitis Medical Case Challenge FCPS Medicine Quiz Clinical ReasoningInternal Medicine Quiz Facebook
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