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Microscopic polyangiitis
Classification and external resources
ICD-10 M31.7
DiseasesDB 8193
eMedicine med/2931

Microscopic polyangiitis (MPA) is an ill-defined autoimmune disease characterized by pauci-immune, necrotizing, small-vessel vasculitis without clinical or pathological evidence of necrotizing granulomatous inflammation.

Contents

Presentation

Because many different organ systems may be involved, a wide range of symptoms are possible in MPA.

Purpura and livedo may be present.[1]

Diagnosis

Laboratory tests show an increased sedimentation rate, reduced red blood count, antineutrophil cytoplasmic antibodies (p-ANCA) directed against myeloperoxidase[2] (a constituent of neutrophil granules), and protein and red blood cells in the urine.

The test for anti-glomerular basement membrane antibody (GBM), which is positive in Goodpasture's syndrome, is negative.

Cause

This condition, the clinical picture of which may include many features of systemic lupus erythematosis, has been reported. While the mechanism of disease has yet to be fully elucidated, the leading hypothesis is that the process is begun with an autoimmune process of unknown etiology that triggers production of ANCA. These antibodies will circulate at low levels until a pro-inflammatory trigger - such as infection, malignancy, or drug therapy. The trigger upregulates production of ANCA. Then, the large number of antibodies make it more likely that they will bind a neutrophil. Once bound, the neutrophil degranulates. The degranulation releases toxins that cause endothelial injury, that is, damage to vessels of glomeruli.[3]

Treatment

The customary treatment involves long term dosage of prednisone, alternated or combined with cytotoxic drugs, such as cyclophosphamide or azathioprine.

Plasmapheresis may also be indicated in the acute setting to remove ANCA antibodies.

Rituximab has been investigated.[4]

See also

References

External links








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