Granulomatosis with polyangiitis: Difference between revisions

m (Rossdonaldson1 moved page Wegener's Granulomatosis to Wegener's granulomatosis)
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==Background==
==Background==
* "Granulomatosis with polyangiitis"
*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
 
==Clinical Features==
==Clinical Features==
* 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, [[ICH|cerebral hemorrhage]] or [[cerebral venous thrombosis|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]]
==Classification==
 
* American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria
==Evaluation==
* Nasal or oral inflammation
===Classification===
* Abnormal chest radiograph showing nodules, fixed infiltrate, or cavities
*American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria
* Abnormal urinary sedimentation (microscopic hematuria)
*Nasal or oral inflammation
* Granulomatous inflammation on biopsy of an artery or perivascular area  
*Abnormal chest radiograph showing nodules, fixed infiltrate, or cavities
==Workup==
*Abnormal urinary sedimentation (microscopic hematuria)
* Definitive diagnosis: Biopsy
*Granulomatous inflammation on biopsy of an artery or perivascular area  
* ANCA +, RF+
===Workup===
* CBC: Leukocytosis, normochronic anemia, thrombocytosis
*Definitive diagnosis: Biopsy
* ESR/CRP elevated
*ANCA +, RF+
* BUN/Cr
*CBC: [[Leukocytosis]], normochromic [[anemia]], [[thrombocytosis]]
* UA (hematuria, proteinuria)
*ESR/CRP elevated
* CXR- Pulmonary infiltrates and nodules
*BUN/Cr
* CT chest
*[[Urinalysis]] ([[hematuria]], [[proteinuria]])
* To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood cx to r/o other pathology
*[[CXR]]- Pulmonary infiltrates and nodules
* Other tests: Bronchoscopy, PFT, sinus CT
*CT chest
*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
 
==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: 357 mg/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


==Sources==
==Disposition==
 
==See Also==
 
==References==
<references/>
<references/>
* Rosen's Emergency Medicine 8th edition. 2013. Chapter: Erythematosus and the Vasculitides. p1536-1537.
 
* Falk RJ, et al. Clinical manifestations and diagnosis of granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014. 
[[Category:Rheumatology]]
* Kaplan AA, et al. initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis. In: Post T, ed. UpToDate. Waltham, Mass.: UpToDate; 2014. www.uptodate.com. Accessed December 22, 2014.
[[Category:Renal]]
* Tracy CL, et al. (2014, Sep 25). Granulomatosis with polyangiitis. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/332622-overview
[[Category:Vascular]]

Revision as of 17:36, 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

Management

Disposition

See Also

References