Difference between revisions of "Granulomatosis with polyangiitis"

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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, LFT, blood culture to rule out other pathology
+
*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

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, LFTs, blood culture to rule out other pathology
  • Other tests: Bronchoscopy, PFT, sinus CT

Management

Disposition

See Also

References