Vasculitis: Difference between revisions
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* Small-vessel: often have palpable purpura |
* Small-vessel: often have palpable purpura |
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** ANCA-associated |
** [[ANCA-associated vasculitis]] |
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*** Microscopic polyangiitis: anti-MPO/p-ANCA; pulmonary-renal syndrome |
*** [[Microscopic polyangiitis]]: anti-MPO/p-ANCA; pulmonary-renal syndrome |
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*** Granulomatosis with polyangiitis: anti-PR3/c-ANCA; pulmonary-renal syndrome |
*** [[Granulomatosis with polyangiitis]]: anti-PR3/c-ANCA; pulmonary-renal syndrome |
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*** Eosinophilic granulomatosis with polyangiitis: more often anti-MPO; rhinosinusitis, asthma, eosinophilia, glomerulonephritis |
*** [[Eosinophilic granulomatosis with polyangiitis]]: more often anti-MPO; rhinosinusitis, asthma, eosinophilia, glomerulonephritis |
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** Non-ANCA |
** Non-ANCA |
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*** Immune-complex |
*** Immune-complex |
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**** Anti-GBM disease |
**** [[Anti-GBM disease]] |
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**** Cryoglobulinemic vasculitis: often associated with hepatitis C |
**** [[Cryoglobulinemic vasculitis]]: often associated with [[hepatitis C]] |
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*** IgA |
*** [[IgA vasculitis]] (HSP): abdominal pain, arthritis, rash, sometimes IgA nephropathy |
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*** Anti-C1q vasculitis (hypocomplementemic urticarial vasculitis |
*** [[Anti-C1q vasculitis]] ([[hypocomplementemic urticarial vasculitis]]) |
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* Medium-vessel: typically cause a lacy rash |
* Medium-vessel: typically cause a lacy rash |
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** Polyarteritis nodosa: often associated with hepatitis B |
** [[Polyarteritis nodosa]]: often associated with hepatitis B |
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** Kawasaki disease: usually in children, associated with mucocutaneous lymph node syndrome |
** [[Kawasaki disease]]: usually in children, associated with mucocutaneous lymph node syndrome |
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* Large-vessel |
* Large-vessel |
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** [[Takayasu arteritis]]: usually aortitis |
** [[Takayasu arteritis]]: usually aortitis |
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* Variable-vessel |
* Variable-vessel |
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** [[Behçet syndrome]]: recurrent oral and genital ulcers with skin, eye, joint, GI, or CNS involvement |
** [[Behçet syndrome]]: recurrent oral and genital ulcers with skin, eye, joint, GI, or CNS involvement |
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** Cogan syndrome: ocular inflammatory lesions |
** [[Cogan syndrome]]: ocular inflammatory lesions |
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** Primary CNS vasculitis |
** [[Primary CNS vasculitis]] |
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* Associated with a systemic disease |
* Associated with a systemic disease |
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** Lupus |
** [[Lupus]] |
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** Rheumatoid arthritis |
** [[Rheumatoid arthritis]] |
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** Relapsing polychondritis |
** [[Relapsing polychondritis]] |
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** Etc. |
** Etc. |
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== Investigations == |
== Investigations == |
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* ANA: lupus and other rheumatologic diseases |
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* Complements: lupus and mixed cryoglobulinemia |
* Complements: lupus and mixed cryoglobulinemia |
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* ANCA: for ANCA-associated vasculitis |
* ANCA: for ANCA-associated vasculitis |
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Revision as of 22:17, 11 November 2021
Differential Diagnosis
- Small-vessel: often have palpable purpura
- ANCA-associated vasculitis
- Microscopic polyangiitis: anti-MPO/p-ANCA; pulmonary-renal syndrome
- Granulomatosis with polyangiitis: anti-PR3/c-ANCA; pulmonary-renal syndrome
- Eosinophilic granulomatosis with polyangiitis: more often anti-MPO; rhinosinusitis, asthma, eosinophilia, glomerulonephritis
- Non-ANCA
- Immune-complex
- Anti-GBM disease
- Cryoglobulinemic vasculitis: often associated with hepatitis C
- IgA vasculitis (HSP): abdominal pain, arthritis, rash, sometimes IgA nephropathy
- Anti-C1q vasculitis (hypocomplementemic urticarial vasculitis)
- Immune-complex
- ANCA-associated vasculitis
- Medium-vessel: typically cause a lacy rash
- Polyarteritis nodosa: often associated with hepatitis B
- Kawasaki disease: usually in children, associated with mucocutaneous lymph node syndrome
- Large-vessel
- Takayasu arteritis: usually aortitis
- Giant cell arteritis: usually carotids and its branches
- Variable-vessel
- Behçet syndrome: recurrent oral and genital ulcers with skin, eye, joint, GI, or CNS involvement
- Cogan syndrome: ocular inflammatory lesions
- Primary CNS vasculitis
- Associated with a systemic disease
Clinical Manifestations
History
- Fever, fatigue, weight loss, arthralgias
- Scleritis
- Nasal crusting, epistaxis, or upper airway disease: GPA
- Acute foot or wrist drop
- Limb claudication, especially unusual patient or location: Takayasu or giant cell arteritis
- Hemoptysis: ANCA-associated vasculitis
- Glomerulonephritis: ANCA-associated vasculitis or anti-GBM disease
- Drug exposure in past 6-12 months
- Cocaine/Levamasole
- Hydralazine
- Propylthiouracil (PTU) more than methimazole (MMI)
- Minocycline
- Allopurinol, penicillamine, procainamide, thiamazole, clozapine, phenytoin, rifampicin, cefotaxime, isoniazid, indomethacin
- Hepatitis B or C
- Lupus or other rheumatologic diseases
Physical exam
- Mononeuritis multiplex: sensory or motor neuropathy, which can be subtle, and symmetric or asymmetric
- Palpable purpura: small-vessel
- Diminished peripheral pulses: large-vessel vasculitis
Investigations
- ANA: lupus and other rheumatologic diseases
- Complements: lupus and mixed cryoglobulinemia
- ANCA: for ANCA-associated vasculitis
- CXR if respiratory symptoms
- EMG if concern for mononeuritis multiplex
- Lumbar puncture if concern for primary CNS vasculitis
