Difference between revisions of "Granulomatosis with polyangiitis"

(Text replacement - "*UA" to "*Urinalysis")
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==Background==
 
==Background==
 
*Formerly known as ''Wegener's granulomatosis''
 
*Formerly known as ''Wegener's granulomatosis''
*c-ANCA associated systemic necrotizing vasculitis
+
*c-ANCA associated systemic necrotizing [[vasculitis]]
 
*Small- and medium-sized blood vessels
 
*Small- and medium-sized blood vessels
 
*Predilection for upper and lower respiratory tracts and kidneys
 
*Predilection for upper and lower respiratory tracts and kidneys
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*Upper respiratory, pulmonary and renal disease + constitutional symptoms
 
*Upper respiratory, pulmonary and renal disease + constitutional symptoms
 
*White, older patients
 
*White, older patients
*Constitutional symptoms: Fever, malaise, weight loss
+
*Constitutional symptoms: [[Fever]], malaise, weight loss
*Upper airway: Serous otits 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 MC 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, cerebral hemorrhage or thrombosis
*Cardiac: Pericarditis, myocarditis
+
*Cardiac: [[Pericarditis]], [[myocarditis]]
  
 
==Differential Diagnosis==
 
==Differential Diagnosis==
*[[Polyarteritis nodosa]], [[Churg-Strauss syndrome]], [[SLE]], [[Goodpasture syndrome]]
+
*[[Polyarteritis nodosa]], [[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss syndrome), [[SLE]], [[Goodpasture syndrome]]
*Lymphoma, lung cancer
+
*[[Lymphoma]], lung cancer
*[[Pnuemonia]], infective [[endocarditis]], [[HUS]]
+
*[[Pneumonia]], infective [[endocarditis]], [[HUS]]
 
*[[Glomerulonephritis]]
 
*[[Glomerulonephritis]]
  
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*Definitive diagnosis: Biopsy
 
*Definitive diagnosis: Biopsy
 
*ANCA +, RF+
 
*ANCA +, RF+
*CBC: Leukocytosis, normochronic anemia, thrombocytosis
+
*CBC: Leukocytosis, normochromic anemia, thrombocytosis
 
*ESR/CRP elevated
 
*ESR/CRP elevated
 
*BUN/Cr
 
*BUN/Cr
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==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)
*Methotrexate: 20-25mg weekly PO or SC
+
*[[Methotrexate]]: 20-25mg weekly PO or SC
*Cyclophosphamide: 2mg/kg/d PO or 15mg/kg q2 weeks x3 then q3 weeks
+
*[[Cyclophosphamide]]: 2mg/kg/d PO or 15mg/kg q2 weeks x3 then q3 weeks
*Rituximab: 357mg/m² weekly x 4
+
*[[Rituximab]]: 357mg/m² weekly x 4
*To consider plasma exchange: Severe/rapidly progressive renal disease, concurrent anti-GBM Ab disease, severe pulmonary hemorrhage
+
*To consider [[plasma exchange]]: Severe/rapidly progressive renal disease, concurrent anti-GBM Ab disease, severe pulmonary hemorrhage
  
 
==Disposition==
 
==Disposition==

Revision as of 22:55, 13 November 2016

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

Differential Diagnosis

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, LFT, 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
  • 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:
  • 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

Disposition

See Also

References