ANCA-Associated Vasculitis with Pulmonary Involvement
Small-vessel necrotizing vasculitis (granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis) with diverse pulmonary manifestations from cavitary nodules to alveolar hemorrhage to obstructive airway disease; managed with rituximab or cyclophosphamide induction.
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What is ANCA-Associated Vasculitis with Pulmonary Involvement?
ANCA-associated vasculitis (AAV) is a group of three small-vessel systemic vasculitides linked by anti-neutrophil cytoplasmic antibodies: (1) granulomatosis with polyangiitis (GPA, Wegener) — necrotizing granulomatous inflammation of upper and lower respiratory tract with necrotizing glomerulonephritis, classically c-ANCA/PR3 positive (90 percent); (2) microscopic polyangiitis (MPA) — necrotizing vasculitis of small vessels without granulomas, predominantly p-ANCA/MPO positive (75 percent), prominent renal and pulmonary capillaritis; (3) eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss) — eosinophil-rich granulomatous inflammation with asthma, eosinophilia, polyneuropathy, eosinophilic infiltrates, p-ANCA/MPO positive in 40 percent.
Pulmonary manifestations: GPA — cavitary nodules and masses (60 percent — multiple, bilateral, frequently cavitating, can mimic infection or malignancy), subglottic and tracheobronchial stenosis (25 percent), endobronchial inflammation, pleural effusion, alveolar hemorrhage; MPA — diffuse alveolar hemorrhage with pulmonary capillaritis (30–50 percent — life-threatening, often co-presents with rapidly progressive glomerulonephritis = pulmonary-renal syndrome), interstitial fibrosis (20–30 percent — usually MPO-ANCA positive, often UIP pattern, may precede vasculitis); EGPA — late-onset asthma (often years before vasculitis), eosinophilic pulmonary infiltrates (mimics Loffler syndrome), pleural effusions with eosinophils, alveolar hemorrhage rare.
Diagnosis: ANCA serology (indirect IF for c-ANCA / p-ANCA pattern + ELISA for PR3 / MPO antigen specificity), tissue biopsy (renal biopsy with pauci-immune necrotizing glomerulonephritis, lung biopsy with vasculitis or granulomas, sinus / nasal biopsy, skin biopsy), HRCT chest (pattern depends on entity), bronchoscopy with BAL (rule out infection, document DAH with progressively bloody sequential aliquots), urinalysis (RBC casts, proteinuria), comprehensive autoimmune panel to rule out alternative diagnoses (anti-GBM, ANA, anti-dsDNA, complement, hepatitis B/C, HIV, cryoglobulins). EGPA workup: peripheral eosinophilia > 1,500/µL or > 10 percent, late-onset asthma, eosinophilic pneumonia, mononeuritis multiplex; ACR / EULAR 2022 classification criteria for accurate phenotyping.
Symptoms
Risk Factors
When to See a Doctor?
If you experience any of the following symptoms, seek medical attention promptly:
- Hemoptysis with hematuria — pulmonary-renal syndrome emergency
- Chronic sinusitis with crusting, bleeding, perforation, or saddle-nose deformity
- Adult-onset asthma with peripheral eosinophilia
- Mononeuritis multiplex (foot drop, wrist drop)
- Constitutional symptoms with multiorgan involvement
- Persistent cavitary lung nodules in non-smoker
- Subglottic stenosis with stridor (urgent ENT)
- Known AAV with new symptoms (relapse)
- Drug exposure (levamisole-cocaine, hydralazine) with new vasculitis symptoms
Treatment Methods
Which Department to Visit?
You can visit our Göğüs Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.
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Related Health Topics
Other articles from the same department you may want to explore.
Asthma
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Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.
COPD (Chronic Obstructive Pulmonary Disease)
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COPD is an irreversible lung disease characterized by shortness of breath and chronic cough; quitting smoking slows its progression.
Pneumonia
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Pneumonia presents with high fever, cough and shortness of breath; the vast majority recover with appropriate antibiotic treatment.
Tuberculosis (TB)
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Tuberculosis presents with weeks-to-months of cough, fever, and night sweats; early diagnosis and treatment lead to full recovery.
Pleural Effusion
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Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.
Pneumothorax
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Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.
Bronchitis (Acute and Chronic)
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Acute bronchitis is mostly viral and resolves spontaneously, while chronic bronchitis is a smoking-related component of COPD.
Bronchiectasis
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Bronchiectasis is a chronic respiratory disease characterized by permanent, abnormal dilation of bronchi with associated destruction of muscular and elastic components of airway walls, resulting in impaired mucociliary clearance and recurrent infection.
Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.