Masakazu Hanatani

Focal and Segmental Glomerulosclerosis in Preeclamptic Patients with Nephrotic Syndrome

Etiology and pathogenesis of focal and segmental glomerulosclerosis (FSGS) in patients with toxemia of pregnancy remain controversial. We examined 15 preeclamptic patients presenting with nephrotic syndrome. None of the patients had urinary abnormalities and hypertension before pregnancy. Clinically, proteinuria first developed during pregnancy and disappeared completely in all but one patient lost to follow-up after 1-30 months from delivery. Renal dysfunction, hypertension and edema rapidly resolved in the postpartum period. None of the patients had a progressive clinical course. Renal biopsy specimens obtained postpartum revealed typical features of preeclamptic nephropathy. In addition, findings compatible with FSGS were observed in 13 patients including 4 in which such lesions were unearthed by additional serial sectioning. These results indicate that FSGS may not only be induced by preeclampsia but also be one of the representative glomerular changes in preeclamptic patients with nephrotic syndrome. A favorable clinical course ensues in a manner similar to that of patients with the garden - variety of preeclampsia.

Anaphylactoid purpura and familial IgA nephropathy

I gA nephropathy has become recognized worldwide as a fairly common form of glomerulonephritis. The diagnosis is established by the predominance of IgA in the mesangial immune-complex deposits in the glomeruli, usually accompanied by C3 and sometimes b IgG and IgM. Since the original description in 1968 [l , 3 it has been generally assumed that IgA nephropathy develops randomly in persons. However, increased, awareness of instances of biopsy-proven IgA nephropathy in two or more members of the same family [Z-4] has strengthened the hypothesis that a hereditary factor is important in some patients. A significant development within the past decade has been the immunologic studies of these cases and the discovery of similar changes in patients with anaphylactoid purpura; namely, the presence of IgA in the mesangium of renal biopsy specimens [5-71. This similarity prompted Baart de la Faille-Kuyper et al [B] and Meadow and Scott [9] to suggest that isolated primary IgA nephropathy may be a monosymptomatic form of anaphylactoid purpura. The difference between these two entities is primarily based on the vascular involvement of the skin, joints, and gastrointestinal tract present only in, patients with anaphylactoid purpura. An argument for a relationship between the two diseases is their occasional, although rare, occurrence in the same family [g-11]. We herein report on a family with IgA nephropathy, one member of which had recurrent purpuric rashes accompanied by severe involvement of the kidneys and eventual death ,from cerebral hemorrhage after development of renal failure. This is the first description of anaphylactoid purpura associated with familial IgA nephropathy in an adult.

Anti-nuclear RNP antibodies in two sisters

Two sisters with clinical elements of mixed connective tissue disease were found to have anti‐nuclear RNP (nRNP) antibodies. These antibodies were not found in the six other family members examined. The sisters had inherited an identical HLA haplotype A2‐BW61CW3‐DR1 from their mother who had had Raynauds phenomenon for several years. Analysis of peripheral lymphocyte subsets in the patients and their immediate relatives showed decreased OKT‐4‐positive cells in one of the patients and increased OKT‐8‐positive cells in both patients, their father and their brother, resulting in lower OKT 4/OKT 8 ratios in these members of the family. This is the third description of the familial occurrence of anti‐nRNP antibodies and it adds further evidence for the implication of genetic factors in the development of anti‐nRNP antibodies.