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Pulmonary Vasculitis — Advanced Treatment

Inflammatory disorders of pulmonary vasculature including ANCA-associated vasculitides (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA], eosinophilic GPA [EGPA]), anti-GBM disease, and Takayasu arteritis, presenting with diffuse alveolar hemorrhage, pulmonary nodules, or fibrosis, requiring induction with cyclophosphamide or rituximab plus corticosteroids and maintenance with azathioprine, methotrexate, or rituximab per EULAR/ACR guidelines.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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What is Pulmonary Vasculitis — Advanced Treatment?

Pulmonary vasculitis encompasses primary systemic vasculitides with prominent pulmonary involvement, classified by Chapel Hill Consensus Conference: small-vessel ANCA-associated vasculitis (AAV) - granulomatosis with polyangiitis (GPA, formerly Wegener's), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA, formerly Churg-Strauss); anti-GBM (Goodpasture's syndrome) with anti-glomerular basement membrane antibodies binding alpha-3 chain of type IV collagen; large-vessel vasculitis - Takayasu, giant cell arteritis (with pulmonary artery aneurysms in 15%); and immune complex small-vessel vasculitis - cryoglobulinemic, IgA vasculitis, hypocomplementemic urticarial vasculitis.

GPA pathogenesis involves PR3-ANCA (anti-proteinase 3) antibodies activating primed neutrophils, causing necrotizing granulomatous inflammation in upper airway (sinusitis, saddle nose, subglottic stenosis), lower airway (pulmonary nodules, cavitation, DAH), and kidney (pauci-immune crescentic glomerulonephritis). MPA features MPO-ANCA (anti-myeloperoxidase) without granulomas, predominantly causing pulmonary capillaritis with DAH, pulmonary fibrosis (in some), and rapidly progressive glomerulonephritis. EGPA involves asthma (95%), eosinophilia >10% or >1500/uL, vasculitis, neuropathy, cardiac involvement, with MPO-ANCA in 30-40%.

Advanced therapy follows EULAR/ACR 2021 guidelines: severe AAV (organ-threatening) requires induction with cyclophosphamide (IV pulse 15 mg/kg q2-3w for 3-6 doses) OR rituximab (375 mg/m2 weekly x 4 doses or 1 g x 2 doses 2 weeks apart, RAVE/RITUXVAS trials), plus high-dose glucocorticoids (methylprednisolone pulse 500-1000 mg x 3 days then prednisone 1 mg/kg/day with rapid taper per PEXIVAS protocol). Plasma exchange (PLEX) considered in DAH, severe renal disease (Cr >5.7), anti-GBM. Maintenance: rituximab 500 mg every 6 months (MAINRITSAN, RITAZAREM), azathioprine 2 mg/kg/day, or methotrexate 25 mg/week, for 24-48 months. Avacopan (C5a receptor antagonist) approved 2021 as glucocorticoid-sparing for AAV.

Symptoms

Hemoptysis (mild to massive, DAH)
Cough, dyspnea, chest pain
Pulmonary nodules with cavitation (GPA)
Diffuse alveolar hemorrhage with hypoxia
ENT symptoms: chronic sinusitis, saddle nose (GPA)
Asthma and eosinophilia (EGPA)
Glomerulonephritis with hematuria/proteinuria (renal involvement)

Risk Factors

Age 50-70 (peak incidence)
ANCA positivity (PR3-ANCA in GPA, MPO-ANCA in MPA)
Genetic susceptibility (HLA-DPB1*04 for GPA)
Environmental exposures (silica, hydrocarbons)
Drug-induced ANCA vasculitis (hydralazine, propylthiouracil, levamisole-cocaine)
Asthma history (EGPA precursor)
Anti-GBM antibodies in Goodpasture's

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Hemoptysis with respiratory failure (urgent)
  • Pulmonary-renal syndrome (DAH + glomerulonephritis)
  • Chronic sinusitis with pulmonary nodules
  • Late-onset asthma with eosinophilia
  • Mononeuritis multiplex with rash
  • ANCA positivity workup (PR3, MPO testing)
  • Refractory disease despite induction therapy

Treatment Methods

01
Cyclophosphamide IV pulse 15 mg/kg q2-3w for 3-6 doses (severe AAV)
02
Rituximab 375 mg/m2 weekly x 4 or 1 g x 2 (alternative to CYC, RAVE trial)
03
High-dose glucocorticoids: methylprednisolone pulse 500-1000 mg x 3 days, then prednisone 1 mg/kg/day taper
04
Plasma exchange in DAH, severe renal (Cr >5.7), anti-GBM
05
Maintenance rituximab 500 mg q6m (MAINRITSAN), azathioprine, or methotrexate 24-48 months
06
Avacopan (C5a receptor antagonist) glucocorticoid-sparing for AAV (2021)
07
Plasmapheresis emergency in anti-GBM disease

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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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.