Granulomatosis with polyangiitis: Difference between revisions
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==Background== | ==Background== | ||
* | *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 | *Upper airway: Serous [[otitis media]], [[hearing loss]], [[sinusitis]], nasal mucosal ulcerations, septal perforation, [[epistaxis]], laryngotracheal disease | ||
** Subglottic stenosis | **[[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 | *[[Polyarteritis nodosa]], [[Eosinophilic granulomatosis with polyangiitis]] (Churg-Strauss syndrome), [[SLE]], [[Goodpasture syndrome]] | ||
* Lymphoma, lung cancer | *[[Lymphoma]], lung cancer | ||
* [[ | *[[Pneumonia]], infective [[endocarditis]], [[HUS]] | ||
* [[Glomerulonephritis]] | *[[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 | |||
*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: | *[[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== | |||
==See Also== | |||
== | ==References== | ||
<references/> | <references/> | ||
[[Category: | [[Category:Rheumatology]] | ||
[[Category: | [[Category:Renal]] | ||
[[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
- 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
- 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