S8C12VA3-Angiitides (VA)

Immunopathological Classification

(cutaneous angiitides)

1. evidence of circulating immune complexes:

a. IgA mesangiopathy

b. low serum IgM

c. single component cryglobulin

d. negative findings

(J.J. Cream)

S8C12VA3-1: This is a continuation of the list begun with A8C10VA2-9.

Henoch-Schonlein Purpura

(with renal involvement and IgA)

1. glomerular changes usually focal

2. subepithelial humps rare

3. mesangial involvement common (size of aggregates)

a. fibrin

b. IgA (IgG & IgM +/-)

c. properdin

d. glycine-rich B-glycoprotein

e. C3 - alternate pathway activation

S8C12VA3-2: Henoch-Schonlein purpura is a classic example in which the changes, early on, are those of a leukocytoclastic angiitis.

Henoch-Schonlein Purpura

1. circulating immune complexes (large aggregates), or

2. vascular or mesangial localization of antigen with attraction of antibody

3. mesangial binding antibody

S8C12VA3-3:Three possible explanation for the glomerular lesions of Henoch-Schonlein are listed.

Intermediate Complex Purpura

1. primary

2. secondary

a. keratoconjunctivitis sicca

b. Sjogren’s syndrome

c. systemic LE

d. renal tubular acidosis

e. chronic lymphatic leukemia

S8C12VA3-4: Features of intermediate complex purpura are listed.

Intermediate Complex Purpura

(Cream)

1. polyclonal increase in gamma globulins

(hyper-gammaglobulinemic purpura)

2. complexes of intermediate size

3. analytical centrifugation (13-14S)

4. preponderance of females

5. thyroid antibodies common

6. no glomerulonephritis

S8C12VA3-5: Other features of intermediate complex purpura are listed.

Low IgM Levels and Vasculitis

1. IgM complexes? (removed from serum)

2. rare disorder

3. reported in setting of Henoch-Schonlein purpura

S8C12VA3-6: Some examples of vasculitis are associated with low IgM levels.

Low IgM Levels

1. genetic disorder

2. secondary

a. bone marrow disorders

b. reticuloendothelial neoplasia

c. prolonged anemia

d. gluten-sensitive enteropathy

e. excessive loss (nephrotic syndrome)

S8C12VA3-7: Some of the disorders associated with low IgM levels are listed.

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