HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Rare, severe, p-ANCA- or c-ANCA positive (60% of patients), necrotizing "small vessel" vasculitis (microscopic) as (minus) variant of the classic polyarteritis nodosa mostly without infestation of medium or large arteries.
MPA is usually accompanied by necrotizing glomerulonephritis and pulmonary capillaritis. Diagnosis is often difficult to distinguish from Churg-Strauss syndrome and granulomatosis with polyangiitis (= Wegener's granulomatosis).
The life-threatening pulmo-renal full picture usually requires intensive treatment.
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Occurrence/EpidemiologyThis section has been translated automatically.
The incidence is 0.6-0.8/100,000 per year.
EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
LocalizationThis section has been translated automatically.
Clinical featuresThis section has been translated automatically.
- Prodromal stage with adynamia, subfebrile temperatures, night sweats and weight loss. 50% of patients develop rheumatic complaints (myalgia, arthralgia).
- Kidneys:in the further course of the disease renal extinction possible (80%) which can manifest itself as Rapid Progressive Glomerulonephritis.
- Lungs: pulmonary capillaritis (25%), haemoptysis, pleuritides, pneumonia.
- Heart: cardiovascular system involvement
- Other: Gastrointestinal tract and peripheral nervous system (polyneuritis) are possible.
- skin (40%; the dermatologist is usually only involved in consultation with the patient in this clinical picture):
LaboratoryThis section has been translated automatically.
Positive (MPO-ANCA) p-ANCA in about 60% of patients. About 20% of patients may also have cANCAs.
Note: pANCAs are not specific for MPA, but are also found in other vaculitides.
HistologyThis section has been translated automatically.
Inconspicuous epidermis, extensive filling of the corium by dense, perivascularly accentuated infiltration of lymphocytic cells with numerous neutrophilic granulocytes and nuclear dust. Pronounced erythrocyte extravasation. Distinctly swollen vascular endothelia and pronounced fibrin deposits within the vessel walls. Leucocytoclastic vasculitis without immune complex deposits.
|Accentuates around arterioles and small arteries in skin and subcutis|
|Capillaries omitted or less strongly involved|
|perivascular and intramural leukocytoclasia|
|Damage to endothelial cells|
|Fibrin in/around vessel walls|
|Perivascular extravasation of erythrocytes|
|No/mild edema in the papillary dermis|
|Pathologist. Alterations limited to vascular position, no palisade granulomas|
|Variable (rather low) eosinophilia|
|No plasma cells or fibrosclerosis to a variable degree|
|Reorganization due to lymphocytic vasculitis|
Differential diagnosisThis section has been translated automatically.
Leucocytoclastic vasculitis: vasculitis of small vessels, no positive ANCA serology
Drug and virus exanthema: no positive ANCA serology
Pyoderma gangraenosum: Clinic, localized infestation, ulcer
Granulomatosis with polyangiitis (GPA) - histological detection of granulomas
Eosinophilic granulomatosis with polyangiitis (EPGPA)- pioneering eosinophilic infiltrates
Microscopic polyangiitis (MPA)- no histological evidence of granulomas
In larger collectives with systemic vasculitis the following distributions were found: 66.7% with GPA, 17.0% with MPA, 16.3% with EGPA(Wójcik K et al. 2019).
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
5-year survival rate: approx. 70%; ANCA and CRP increases indicate a recurrence and a worsened prognosis.
Note(s)This section has been translated automatically.
The dermatological symptoms are extremely variable and not "'diagnosis-specific". Thus, the diagnosis can only be made by evaluating the histopathology in conjunction with the complex overall problem.
LiteratureThis section has been translated automatically.
- Agard C et al (2003) Microscopic polyangiitis and polyarteritis nodosa: how and when do they start? Arthritis Rheum 49: 709-715
- Arkin A (1930) A clinical and pathological study of periarteritis nodosa. A report of five cases, one histologically healed. Am J Pathol 6: 401-410
- Frankel SK et al (2002) Vasculitis: Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa, and Takayasu arteritis. Crit Care Clin 18: 855-879
- Guillevin L, Lhote F (1995) Polyarteritis nodosa and microscopic polyangiitis. Clin Exp Immunol 101(Suppl1): 22-23
- Harper L, Savage CO (2000) Pathogenesis of ANCA-associated systemic vasculitis. J Catholic 190: 349-359
- Hattori N et al (2002) Mortality and morbidity in peripheral neuropathy associated Churg-Strauss syndrome and microscopic polyangiitis. J Rheumatol 29: 1408-1414
- Ratzinger G et al. (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. JDDG 1092-1118
- Wagner et al (2002) Microscopic polyangiitis. Nude Dermatol 28: 370-373
- Wohlwill P (1923) On the only microscopically recognizable form of periarteritis nodosa. Virchows Arch 246: 377-411
Wójcik K et al (2019) Clinical characteristics of Polish patients with ANCA-associatedvasculitides-retrospective
analysis of POLVAS registry. Clin Rheumatol doi: 10.1007/s10067-019-04538-w.
Incoming links (9)Anca; Angiitis, necrotizing, non-granulomatous; Microscopic polyangiitis; Microscopic polyangiitis; Microscopic polyarteritis nodosa; Periarteritis nodosa cutanea; Polyarteritis nodosa systemic; Rapid progressive glomerulonephritis; Renal diseases skin changes;
Outgoing links (9)Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Livedo racemosa (overview); Microscopic polyangiitis; Polyarteritis nodosa systemic; Purpura (overview); Pyoderma gangraenosum; Rapid progressive glomerulonephritis; Vasculitis leukocytoclastic (non-iga-associated);
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.