Granulomatosis with polyangiitis: Difference between revisions
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*White, older patients | *White, older patients | ||
*Constitutional symptoms: [[Fever]], malaise, weight loss | *Constitutional symptoms: [[Fever]], malaise, weight loss | ||
*Upper airway: Serous [[otitis media]], hearing loss, [[sinusitis]], nasal mucosal ulcerations, septal perforation, [[epistaxis]], laryngotracheal disease | *Upper airway: Serous [[otitis media]], [[hearing loss]], [[sinusitis]], nasal mucosal ulcerations, septal perforation, [[epistaxis]], laryngotracheal disease | ||
**Subglottic stenosis is most common laryngotracheal lesion (16% patients) | **[[Subglottic stenosis]] is most common laryngotracheal lesion (16% patients) | ||
*Lower respiratory: [[Cough]], [[dyspnea]], pleuritis, [[hemoptysis]], [[diffuse alveolar hemorrhage]] | *Lower respiratory: [[Cough]], [[dyspnea]], pleuritis, [[hemoptysis]], [[diffuse alveolar hemorrhage]] | ||
*[[Renal failure]], [[glomerulonephritis]] | *[[Renal failure]], [[glomerulonephritis]] | ||
*Ophthalmologic: [[scleritis]], [[episcleritis]], [[uveitis]] | *Ophthalmologic: [[scleritis]], [[episcleritis]], [[uveitis]] | ||
*Cutaneous: Palpable [[purpura]], nodules, ulcers | *Cutaneous: Palpable [[purpura]], nodules, ulcers | ||
*Neurologic: Mononeuropathy and polyneuropathy, cerebral vasculitis, cerebral hemorrhage or thrombosis | *Neurologic: Mononeuropathy and polyneuropathy, cerebral vasculitis, [[ICH|cerebral hemorrhage]] or [[cerebral venous thrombosis|thrombosis]] | ||
*Cardiac: [[Pericarditis]], [[myocarditis]] | *Cardiac: [[Pericarditis]], [[myocarditis]] | ||
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*Definitive diagnosis: Biopsy | *Definitive diagnosis: Biopsy | ||
*ANCA +, RF+ | *ANCA +, RF+ | ||
*CBC: Leukocytosis, normochromic anemia, thrombocytosis | *CBC: [[Leukocytosis]], normochromic [[anemia]], [[thrombocytosis]] | ||
*ESR/CRP elevated | *ESR/CRP elevated | ||
*BUN/Cr | *BUN/Cr | ||
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*[[CXR]]- Pulmonary infiltrates and nodules | *[[CXR]]- Pulmonary infiltrates and nodules | ||
*CT chest | *CT chest | ||
*To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, | *To consider ANA, C3 or C4, cryoglobulins, [[viral hepatitis|hepatitis serology]], HIV, [[LFTs]], blood culture to rule out other pathology | ||
*Other tests: Bronchoscopy, PFT, sinus CT | *Other tests: Bronchoscopy, PFT, sinus CT | ||
==Management== | ==Management== | ||
*Priority: Manage pulmonary hemorrhage and renal insufficiency | *Priority: Manage pulmonary hemorrhage and renal insufficiency | ||
**[[Difficult airway]]: Diffuse alveolar hemorrhage and subglottic stenosis | **[[Difficult airway]]: [[Diffuse alveolar hemorrhage]] and [[subglottic stenosis]] | ||
***Fiberoptic intubation through LMA advocated | ***Fiberoptic intubation through LMA advocated | ||
*Rheumatology consult + multidisciplinary consults | *Rheumatology consult + multidisciplinary consults | ||
*Mild disease: [[Corticosteroids]] and [[methotrexate]] | *Mild disease: [[Corticosteroids]] and [[methotrexate]] | ||
**No active glomerulonephritis or organ-threatening disease | **No active glomerulonephritis or organ-threatening disease | ||
*Mod-Severe disease: Corticosteroids and [[cyclophosphamide]] or [[rituximab]] | *Mod-Severe disease: [[Corticosteroids]] and [[cyclophosphamide]] or [[rituximab]] | ||
*Corticosteroids: | *[[Corticosteroids]]: | ||
**[[Methylprednisolone]] (7-15mg/kg/d with max 1000mg) | **[[Methylprednisolone]] (7-15mg/kg/d with max 1000mg) | ||
**[[Prednisone]] 1mg/kg/d (max 80mg) | **[[Prednisone]] 1mg/kg/d (max 80mg) | ||
Revision as of 16:43, 16 October 2019
Background
- Formerly known as Wegener's granulomatosis
- c-ANCA associated systemic necrotizing vasculitis
- Small- and medium-sized blood vessels
- Predilection for upper and lower respiratory tracts and kidneys
Clinical Features
- Upper respiratory, pulmonary and renal disease + constitutional symptoms
- White, older patients
- Constitutional symptoms: Fever, malaise, weight loss
- Upper airway: Serous otitis media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease
- Subglottic stenosis is most common laryngotracheal lesion (16% patients)
- Lower respiratory: Cough, dyspnea, pleuritis, hemoptysis, diffuse alveolar hemorrhage
- Renal failure, glomerulonephritis
- Ophthalmologic: scleritis, episcleritis, uveitis
- Cutaneous: Palpable purpura, nodules, ulcers
- Neurologic: Mononeuropathy and polyneuropathy, cerebral vasculitis, cerebral hemorrhage or thrombosis
- Cardiac: Pericarditis, myocarditis
Differential Diagnosis
- Polyarteritis nodosa, Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome), SLE, Goodpasture syndrome
- Lymphoma, lung cancer
- Pneumonia, infective endocarditis, HUS
- Glomerulonephritis
Evaluation
Classification
- American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria
- Nasal or oral inflammation
- Abnormal chest radiograph showing nodules, fixed infiltrate, or cavities
- Abnormal urinary sedimentation (microscopic hematuria)
- Granulomatous inflammation on biopsy of an artery or perivascular area
Workup
- Definitive diagnosis: Biopsy
- ANCA +, RF+
- CBC: Leukocytosis, normochromic anemia, thrombocytosis
- ESR/CRP elevated
- BUN/Cr
- Urinalysis (hematuria, proteinuria)
- CXR- Pulmonary infiltrates and nodules
- CT chest
- To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFTs, blood culture to rule out other pathology
- Other tests: Bronchoscopy, PFT, sinus CT
Management
- Priority: Manage pulmonary hemorrhage and renal insufficiency
- Difficult airway: Diffuse alveolar hemorrhage and subglottic stenosis
- Fiberoptic intubation through LMA advocated
- Difficult airway: Diffuse alveolar hemorrhage and subglottic stenosis
- Rheumatology consult + multidisciplinary consults
- Mild disease: Corticosteroids and methotrexate
- No active glomerulonephritis or organ-threatening disease
- Mod-Severe disease: Corticosteroids and cyclophosphamide or rituximab
- Corticosteroids:
- Methylprednisolone (7-15mg/kg/d with max 1000mg)
- Prednisone 1mg/kg/d (max 80mg)
- Methotrexate: 20-25mg weekly PO or SC
- Cyclophosphamide: 2mg/kg/d PO or 15mg/kg q2 weeks x3 then q3 weeks
- Rituximab: 357mg/m² weekly x 4
- To consider plasma exchange: Severe/rapidly progressive renal disease, concurrent anti-GBM Ab disease, severe pulmonary hemorrhage