Acute glomerulonephritis. Immunofluorescent and electron-microscopic observations in sporadic cases.

The authors studied by immunofluorescent and electron microscopy renal biopsy specimens from 29 patients with various glomerular diseases. Poststreptococcal glomerulonephritis was characterized by the presence of complement (beta1C) in the mesangium and/or on the basement membrane in all cases. Immunoglobulin G (IgG) was also present in less than half of the cases in the same distribution. Electron microscopy, carried out in six cases, revealed no uniform ultrastructural change: minimal subepi helial deposits were observed in three cases, intramembranous deposits were seen in two cases, and the basement membrane was normal in one case. Glomerular abnormalities during the courses of some systemic diseases were similar. Mild renal involvement was characterized by only beta1C deposition. This finding raises the question whether a mechanism other than or in addition to that involving immune complexes is operative in the pathogenesis of acute glomerulonephritis. There is circumstantial experimental evidence that an alternate pathway of complement activation and deposition may be operative in acute glomerulonephritis.

Polycythemia vera associated with Ushers's symdrome.

A 50-year-old man with Usher's syndrome, progressive retinitis pigmentosa, and congenital sensoneural deafness developed polycythemia vera. Usher's syndrome was associated with a variety of congenital herediatry disorders but there was no evidence to support more than a chance association between Usher's syndrome and polycythemia vera.

Short term cyclosporin A treatment of Behçet's disease.

Eleven separate three-month courses of cyclosporin A, an oral solution 10 mg/kg/day, were administered to eight patients with Behçet's disease with sight-threatening posterior uveitis. It was found to be effective in arresting the inflammatory activity in the eye as well as the mucocutaneous lesions of Behçet's disease. Improvement in visual acuity was observed within one week of starting therapy. Severe exacerbations in the ocular and mucocutaneous lesions occurred on withdrawal of the drug. At this dosage side effects included hirsutism in all women, and a slight rise of serum bilirubins in two patients and of blood urea in one patient. The latter two conditions responded rapidly to dose adjustment.

A case of Whipple's disease complicated by fatal hepatitis.

Whipple's disease was diagnosed in a 42-year-old woman by the histological examination of a mesenteric lymph node taken at laparotomy, and the electron microscopic examination of a peroral intestinal biopsy specimen. She responded well to tetracycline therapy, but succumbed to fatal hepatitis ten weeks after the diagnosis of Whipple's disease

Symposium on membrane markers and antigens of human lymphocytes and leukemic cells.

B lymphocytes, mediators of humoral immunity, carry easily detectable SIg, form rosettes with RBC coated with IgM and complement (EAC rosetting) and bind aggregated Ig (Fc receptor activity). Human T lymphocytes, mediators of cellular immunity, lack a specific antigen found on mice T lymphocytes (theta antigens). They form spontaneous (non-immune) rosettes with SRCB (E rosetting) and they are identified by labelling or cytotoxicity tests wtih heterologous antisera. Apart from B and T lymphocytes, a third population of lymphoid cells carrying no detectable surface markers have been identified by various methods (Null cells).

Lack of correlation between skin reactivity and T lymphocytes and monocytes in Hodgkin's disease.

28 untreated patients with Hodgkin's disease have been studied in respect to skin reactivity to purified protein derivative and dinitrochlorobenzene and blood monocytes and T lymphocytes. Cutaneous anergy as defined by unresponsiveness to both purified protein derivative and dinitrochlorobenzene was found in 43% of patients and in 7% of the control group. Although there was a significant reduction in E-rosetting lymphocytes in Hodgkin's cases, we found no differences in the mean numbers of E-rosetting lymphocytes and monocytes between normergic and anergic patients. We, therefore, conclude that skin reactivity is not directly correlated with the total numbers of E-rosetting lymphocytes or monocytes in Hodgkin's disease.

Heterogenous glycogen storage disease in one family.

Three brothers, aged 17, 14 and 4 ye presented. Deficiency of glucose-6-phosphatase was associated with deficiency of acid maltase in one and debranching enzyme in the other. Enzyme analyses could not be performed in the youngest sibling.

Renal amyloidosis: immunofluorescence and electron microscopy studies.

Renal biopsy specimens of 15 patients with renal amyloidosis were studied by immunofluorescence microscopy. The amyloidosis was associated with chronic pulmonary disease in five, rheumatoid arthritis in one, chronic lymphocytic leukemia in one, and familial Mediterranean fever in five patients. In three patients no associated condition could be determined although the pattern of organ involvement resembled that of secondary amyloidosis. IgG and complement (C3) were demonstrated in the glomerular capillary walls and in the mesangium in all patients. The pattern of the deposits was neither granular nor linear. Ig and C3 appeared as large confluent masses or broad ribbon-like segments. In the six patients studied by electron microscopy the fibrillary formation of amyloid was seen in the mesangium and the glomerular capillary walls corresponding to the Ig deposits. No immunofluorescence or ultrastructural differences were observed among the patients with secondary, inherited and leukemia-associated amyloidosis included in this study.

Olecranon nodules in a case of Behçet's disease.

A 33-year-old male with definite Behçet's disease had rheumatoid-like nodules at his elbows. This finding, we believe, represents another manifestation of vasculitis in Behçet's disease.

Behçet's disease. Relation of serum C-reactive protein and erythrocyte sedimentation rates to disease activity.

In a prospective study of 150 patients with Behçet's disease, significant associations were shown between CRP positivity and the presence of erythema nodosum (p less than 0.02) and acute thrombophlebitis (p less than 0.05) and between ESR and erythema nodosum (p less than 0.01), acute thrombophlebitis (p less than 0.001), and acute arthritis (p less than 0.01). Mucocutaneous, ocular, or central nervous system activities did not show significant associations with these indices of inflammation. In 50 patients in whom quantitative CRP determinations were performed, clinical disease activity was accompanied by slight to moderate increases in CRP and ESR.

Behçet's disease: lack of correlation of clinical manifestations with HLA antigens.

It has been suggested that certain tissue types are associated with different clinical manifestations of Behçet's disease in Japan and England. Regional differences in the associations between HLA antigens and Behçet's disease have been established. We tissue type 119 patients with Behçet's disease in Istanbul and attempted to correlate various clinical manifestations with HLA antigens. The frequency of only one tissue antigen, HLA-B5, was found to be increased in these series. HLA-B5 was present in 94 of 119 patients (77%) compared to 89 of 268 controls (33%), P less than 0.0001, ARR = 6.79. Age of onset, sex, incidences of ocular disease, arthritis, thrombophlebitis and erythema nodosum did not show any positive or negative associations with any of the alleles tested.

Influence of age of onset and patient's sex on the prevalence and severity of manifestations of Behçet's syndrome.

Eye disease, arthritis, folliculitis, and thrombophlebitis were more common among males, and erythema nodosum among females, in 297 patients with Behcet syndrome (BS) at their first visit despite an identical disease duration. Younger males and females (age of onset 24 years or less) had a higher prevalence of eye disease and total clinical activity than did the older patients (age of onset 25 years or more). Among the 51 patients followed up for 52 months (SD 7 months) the total clinical activity became significantly less (p less than 0.05) in the whole group at the end of this period. This was particularly true for older females. While male sex and a younger age of onset are associated with more severe disease in BS, overall, the syndrome ran an improving or stable course over 4 1/2 years.