Granulomatosis with polyangiitis: Difference between revisions
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==Clinical Features== | ==Clinical Features== | ||
* Upper respiratory, pulmonary and renal disease + constitutional symptoms | * Upper respiratory, pulmonary and renal disease + constitutional symptoms | ||
* White | * 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 | ||
| Line 17: | Line 17: | ||
* Cardiac: Pericarditis, myocarditis | * Cardiac: Pericarditis, myocarditis | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
* Polyarteritis nodosa, Churg-Strauss syndrome, SLE, Goodpasture syndrome | |||
* Lymphoma, lung cancer | |||
* Pnuemonia, infective endocarditis, HUS | |||
* Glomerulonephritis | |||
==Classification== | ==Classification== | ||
* American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria | * American College of Rheumatology: 88% sensitivity and 92% specificity for ≥2 criteria | ||
| Line 26: | Line 29: | ||
==Workup== | ==Workup== | ||
* Definitive diagnosis: Biopsy | * Definitive diagnosis: Biopsy | ||
* ANCA | * ANCA +, RF+ | ||
* CBC: Leukocytosis, normochronic anemia, thrombocytosis | * CBC: Leukocytosis, normochronic anemia, thrombocytosis | ||
* ESR/CRP elevated | * ESR/CRP elevated | ||
| Line 34: | Line 37: | ||
* CT chest | * CT chest | ||
* To consider ANA, C3 or C4, cryoglobulins, hepatitis serology, HIV, LFT, blood cx to r/o other pathology | * 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: | * 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 | ||
* Corticosteroids and cyclophosphamide | * 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 | |||
==Sources== | ==Sources== | ||
<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. | |||
* 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. | |||
* Tracy CL, et al. (2014, Sep 25). Granulomatosis with polyangiitis. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/332622-overview | |||
Revision as of 06:25, 23 December 2014
Background
- "Granulomatosis with polyangiitis"
- 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
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
Sources
- 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.
- 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.
- Tracy CL, et al. (2014, Sep 25). Granulomatosis with polyangiitis. eMedicine. Retrieved 12/22/2014 from http://emedicine.medscape.com/article/332622-overview