Hladr3

Idiopathic adrenal insufficiency h hypoparathyroidism + chronic mucocutaneous candidiasis + gonadal failure Most commonly in childhood

Males and females Unknown (very rare)

No HLA-DR association P450 side-chain cleavage enzyme Steroid 17a-hydroxylase (?)

Idioopathic adrenal insufficiency + insulin-dependent diabetes mellitus + autoimmune thyroiditis All ages, but more commonly in adults Mostly females

Possibly 50% of adult Addisons patients HLA-DR3 and/or DR4 Steroid 21 -hydroxylase P450

T cell responses to

Adrenal antigens (?)

Figure 1 (A) Adrenal gland from patient with Addisons disease, showing atrophy of the cortex and infiltration with mononuclear cells. (B) Another area from same adrenal gland, photographed at higher magnification. (See also color Plate 6.)

Figure 1 (A) Adrenal gland from patient with Addisons disease, showing atrophy of the cortex and infiltration with mononuclear cells. (B) Another area from same adrenal gland, photographed at higher magnification. (See also color Plate 6.)

thelium is present, it is not arranged in three different zones, but in strands or rows of irregular size, contour and direction. There is loss of cellular outline, swelling and vacuolar appearance. Some cells are extremely large, with granular or vacuolated cytoplasm, others are small. In addition, both hyperplasia and hypertrophy can be observed in adrenals from patients with idiopathic Addisons disease. Scattered islands of cells may be seen in the cortex as well as large well-circumscribed adenoma-like nodules, without typical zonal division of the parenchyma. These cells resemble those of the zona fasciculata, less often those of the zona glomerulosa. They may have a bizarre appearance and sometimes are huge, with deeply pigmented cytoplasm and large hyper-chromatic nuclei. A chronic inflammatory infiltrate is usually present, with a prevalence of small lymphocytes. Plasma cells and macrophages are also present, but in lesser numbers. The adrenal medulla is usually intact or infiltrated by a few lymphoid cells. The lymphocytic infiltrate is usually diffuse, but in some areas lymphocytes may form aggregates of various dimensions. Germinal centers are seldom present.

Studies of adrenal immunopathology in patients with Addisons disease have been rather scarce. Direct immunofluorescence of the adrenals has shown dense deposits of immunoglobulin G (IgG) in some areas of the adrenal cortex, with specific fluorescence localized more intensely at the level of the cell membranes. To date, there are no reports on the composition of the adrenocortical lymphocytic infiltrates. However, immunohistopathology studies of micronodules of Cushing's disease have shown that the lymphocytic infiltrates, present around vessel walls but also inside the nodules themselves, were composed mostly of T cells, usually CD4+ with a minority of CDS 1. In addition, B cells were also present in the infiltrates. It is possible that a similar situation also occurs in the adrenals of patients with autoimmune Addisons disease. Class II major histocompatibility complex (MHC) expression by human adrenal cortical cells appears to be a physiological phenomenon. Approximately 10% of normal adrenocortical cells express MHC class II antigenic determinants. These antigens are mostly localized in the zona reticularis and occasionally may extend to the zona glomerulosa or fasciculata. In adrenal glands from Addisons disease patients, the majority of residual cortical cells show MHC class II antigens, with variation in extent from 50 to 100%. Staining is most intense at the periphery of residual nodules. Adrenal glands from patients with tuberculous Addisons disease have a similar pattern to normal glands, but show class II positivity in cortical cells immediately adjacent to foci of caseous necrosis.

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