Granulomatosis with Polyangiitis (GPA) (Older Terminology: Wegener’s Granulomatosis; Necrotizing Granulomatous Vasculitis = NGV)


Epidemiology


Etiology


Physiology

Necrotizing Vasculitis Involving Small Arteries and Veins

Airway Involvement


Pathologic Features

(histologic material usually obtained from OLB)


Diagnosis

Complete Blood Count (CBC) (see Complete Blood Count)

Erythrocytes Sedimentation Rate (ESR) (see procxx

Urinalysis (see Urinalysis)

Arterial Blood Gas (ABG) (see xxxxx)

Serology – ANA: variable – Anti-DNA: negative – RF: variable (may be mildly elevated) – C3/C4/CH50: normal – c-ANCA (cytoplasmic staining, typically directed against proteinase 3, PR3): positive (>1:20) in >90-95% of untreated active systemic Wegener’s Granulomatosis cases – However, c-ANCA is only positive in 60-65% of limited Wegener’s Granulomatosis cases – ANCA positivity declines with remission but is not sensitive enough to follow disease course – Anti-GBM: negative

Chest CT (see Chest Computed Tomography)

WEG DAH

Pulmonary Function Tests (PFT’s) (see Pulmonary Function Tests)

Bronchoscopy (see Bronchoscopy)

Fluorodeoxyglucose-Positron Emission Tomography (FDG-PET) (see Fluorodeoxyglucose-Positron Emission Tomography)

Video-Assisted Thoracoscopic Surgery (VATS) with Lung Biopsy (see xxxx)

Upper Airway Biopsy

Skin Biopsy (see xxxx)

Renal Biopsy (see xxxx)


Clinical Diagnostic Criteria (with >2 of these criteria, sensitivity is 88% and specificity is 92%)

Limited Wegener’s Granulomatosis


Clinical Features

Cardiovascular Manifestations

Dermatologic Manifestations

Gastrointestinal Manifestations

General Comments

Intestinal Ischemia/Infarction (see Acute Mesenteric Ischemia)

Gastrointestinal Perforation

Ulcerative Lesions

Weight Loss (see Weight Loss)

Neurologic Manifestations (22%)

Fatigue (see Fatigue)

Peripheral Neuropathy (see xxxx)

Mononeuritis Multiplex (see xxxx)

Cranial Neuropathies

External Ophthalmoplegia

Seizures (see xxxx)

Cerebritis

Cerebrovascular Accident (CVA) (see xxxx)

Posterior Reversible Encephalopathy Syndrome (PRES) (see Posterior Reversible Encephalopathy Syndrome)

Ophthamlogic Manifestations (25-60%)

Pulmonary Manifestations

General Comments

Acute/Chronic Bronchiolitis (see Bronchiolitis)

Bronchiolitis Obliterans (BO) (see Bronchiolitis Obliterans)

Diffuse Alveolar Hemorrhage (DAH) (see Diffuse Alveolar Hemorrhage)

Endobronchial Ulcerating Lesions or Stenosis

Interstitial Lung Disease (see Interstitial Lung Disease): less common presentation

Lipoid Pneumonia (see Lipoid Pneumonia)

Lung Nodules (see Lung Nodule or Mass)

Mediastinal Lypmhadenopathy (see Mediastinal Mass)

Pleural Effusion/Thickening (see Pleural Effusion-Exudate)

Pneumonia-Like Picture (see Pneumonia)

Pulmonary Hypertension (see Pulmonary Hypertension)

Renal Manifestations

General Comments

Glomerulonephritis

Rheumatologic Manifestations

Upper Airway Manifestations (75-90% of cases)

Other Manifestations


Treatment

Induction in Mild Disease

Criteria for Mild Disease

Regimens

Pneumocystis Jirovecii Prophylaxis (see Pneumocystis Jirovecii)

Induction in Moderate-Severe Disease

Criteria for Moderate-Severe Disease

Regimens

Pneumocystis Jirovecii Prophylaxis (see Pneumocystis Jirovecii)

Maintenance Therapy

Relapse

Disease Markers to Follow


Prognosis


References

General

Classification

Treatment