Microscopic polyangiitis M31.7

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Angiitis necrotizing non-granulomatous; IMPA; Microscopic polyangiitis; Microscopic polyarteritis; Microscopic polyarteritis nodosa; MPA; necrotizing non-granulomatous angiitis; Periarteritis nodosa cutanea; Polyangiitis microscopic; polyarteritis nodosa; Polyarteritis nodosa microscopic

History
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Wohlwill, 1923; Arkin, 1930

Definition
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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.

Occurrence/Epidemiology
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The incidence is 0.6-0.8/100,000 per year.

Etiopathogenesis
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Manifestation
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Men are more frequently affected than women; the majority of patients are > 50 years old at the beginning of the disease.

Localization
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Mostly lower extremities and trunk are affected.

Clinical features
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  • 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):
    • Clinically difficult to define in individual cases due to the high variability of dermatological symptoms:

Laboratory
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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.

Histology
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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.

Histopathological algorithm of microscopic polyangiitis (polyarteritis) (lowest common denominator:italics, leading symptoms:bold) varies according to Ratzinger et al. 2105
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 diagnosis
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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).

Therapy
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Identical with the therapy of polyarteritis nodosa, see there.

Progression/forecast
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5-year survival rate: approx. 70%; ANCA and CRP increases indicate a recurrence and a worsened prognosis.

Note(s)
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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.

Literature
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  1. Agard C et al (2003) Microscopic polyangiitis and polyarteritis nodosa: how and when do they start? Arthritis Rheum 49: 709-715
  2. 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
  3. 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
  4. Guillevin L, Lhote F (1995) Polyarteritis nodosa and microscopic polyangiitis. Clin Exp Immunol 101(Suppl1): 22-23
  5. Harper L, Savage CO (2000) Pathogenesis of ANCA-associated systemic vasculitis. J Catholic 190: 349-359
  6. Hattori N et al (2002) Mortality and morbidity in peripheral neuropathy associated Churg-Strauss syndrome and microscopic polyangiitis. J Rheumatol 29: 1408-1414
  7. Ratzinger G et al. (2015) The Vasculitis Wheel-an algorithmic approach to cutaneous vasculitis. JDDG 1092-1118
  8. Wagner et al (2002) Microscopic polyangiitis. Nude Dermatol 28: 370-373
  9. Wohlwill P (1923) On the only microscopically recognizable form of periarteritis nodosa. Virchows Arch 246: 377-411
  10. 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.


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Last updated on: 29.10.2020