Systemic vasculitis – special analysis

Tests in package

IgA quantification, PiZ analysis of alpha 1 antitrypsin

Indication

In Goodpasture syndrome and Wegener’s granulomatosis and other renopulmonary syndromes. May indicate patients who should be followed with extra care and may predict relapses.

Clinical background

The most frequent subclasses of IgG in these diseases are IgG1 and IgG4. There is only a low number of IgG2 and IgG3. Patients with MPO-ANCA have more often IgG2 and patients with PR3-ANCA have more often IgG3. An unusual subclass distribution may indicate a different clinical course. Changes in the subclass distribution can be used to study relapses of the disease.

A heterozygous form of alpha-1-antitrypsine occurs in about 4.7% of the normal population and a homozygous form occurs in about 6 out of 10000 individuals. Heterozygotes have a somewhat lower level of alpha-1AT in the blood, whereas homozygotes only have 5-10% of the normal concentration of 0.9-1.7 g/L. Patients with PR3-ANCA have an overrepresentation of PiZ. About 20% can have the gene and in the group of biopsi-verified granulomae only 1/3 had the Piz gene. These patients often have a more widespread disease (more organ engagements) and a shorter survival.

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June 27, 2019
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June 19, 2019
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