Granulomatosis with polyangiitis: Difference between revisions
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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 | ||
==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 otits media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease | ||
** Subglottic stenosis MC laryngotracheal lesion (16% patients) | **Subglottic stenosis MC 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]], [[Churg-Strauss syndrome]], [[SLE]], [[Goodpasture syndrome]] | ||
* Lymphoma, lung cancer | *Lymphoma, lung cancer | ||
* [[Pnuemonia]], infective [[endocarditis]], [[HUS]] | *[[Pnuemonia]], infective [[endocarditis]], [[HUS]] | ||
* [[Glomerulonephritis]] | *[[Glomerulonephritis]] | ||
==Diagnosis== | |||
===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, normochronic anemia, thrombocytosis | |||
*ESR/CRP elevated | |||
*BUN/Cr | |||
*UA (hematuria, proteinuria) | |||
*CXR- Pulmonary infiltrates and nodules | |||
*CT chest | |||
*To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood cx to r/o 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: 357 mg/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 | ||
== | ==References== | ||
<references/> | <references/> | ||
[[Category:Rheum]] | [[Category:Rheum]] | ||
[[Category:Nephro]] | [[Category:Nephro]] | ||
[[Category:Vascular]] | |||
Revision as of 17:32, 6 March 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
- Upper respiratory, pulmonary and renal disease + constitutional symptoms
- White, older patients
- Constitutional symptoms: Fever, malaise, weight loss
- Upper airway: Serous otits media, hearing loss, sinusitis, nasal mucosal ulcerations, septal perforation, epistaxis, laryngotracheal disease
- Subglottic stenosis MC 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, Churg-Strauss syndrome, SLE, Goodpasture syndrome
- Lymphoma, lung cancer
- Pnuemonia, infective endocarditis, HUS
- Glomerulonephritis
Diagnosis
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, normochronic anemia, thrombocytosis
- ESR/CRP elevated
- BUN/Cr
- UA (hematuria, proteinuria)
- CXR- Pulmonary infiltrates and nodules
- CT chest
- To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood cx to r/o 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: 357 mg/m² weekly x 4
- To consider plasma exchange: Severe/rapidly progressive renal disease, concurrent anti-GBM Ab disease, severe pulmonary hemorrhage