Müftüoglu A

A Controlled Trial of Azathioprine in Behçet's Syndrome

Cytotoxic agents have long been used in Behcets syndrome, especially for eye involvement, but their effectiveness has been uncertain. We conducted a two-year randomized, placebo-controlled, double-blind trial of azathioprine (2.5 mg per kilogram of body weight per day) in Turkish men with Behcets syndrome without eye disease (group 1; n = 25) or with eye disease (group 2; n = 48). Corticosteroid treatment remained available to all the patients. All six patients withdrawn from the study because of severe eye disease were receiving placebo (P less than 0.001). Azathioprine was superior to placebo in the prevention of new eye disease in group 1 (1 vs. 8 patients; P less than 0.01) and in group 2 among the 14 patients who at entry had disease in only one eye (P less than 0.001). There were fewer episodes of hypopyon uveitis (1 vs. 15; P less than 0.001) among the group 2 patients who took azathioprine. The patients taking azathioprine also had less frequent oral ulcers, genital ulcers, and arthritis. There were no serious side effects attributable to azathioprine. We conclude that azathioprine is effective in controlling the progression of Behcets syndrome, especially its most serious manifestation, eye disease.

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 Behćet 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.

Low dose cyclosporin A versus pulsed cyclophosphamide in Behcet's syndrome: a single masked trial.

A single masked trial of cyclosporin A 5 mg/kg/day versus monthly 1 g intravenous boluses of cyclophosphamide was conducted among 23 patients with Behçets syndrome and active, potentially reversible uveitis. The trial was unmasked after a mean of 12 (SD 2) months for the cyclosporin A group (n = 12) and a mean of 10 (SD 3) months for the cyclophosphamide group (n = 11). During the initial 6 months the visual acuity significantly improved (p < 0.001) in the cyclosporin A group whereas this was not observed in the cyclophosphamide group. The subsequent follow-up of patients up to 24 months suggested that the initial improvement in visual acuity with cyclosporin A was not sustained. More extensive and especially long-term studies of cyclosporin A in the uveitis of Behçets syndrome are warranted.

The arthritis of Behçet's disease: a prospective study.

A prospective study of arthritis was performed in 47 patients with Behçets disease followed up over a 47-month period (mean 19.25 months, SD 14.09). These patients had a total of 80 episodes of arthritis, which were analysed for joint distribution and symmetry, in 56 of which the duration could also be determined. Attacks were oligoarticular, affecting up to 4 joints per patient, 54 (68%) being monoarticular. Knees, ankles, and wrists were the most commonly involved joints. Involvement of spinal, shoulder, hip, and sacroiliac joints was rare. The arthritis was usually not deforming and subacute; 82% (46/56) of the attacks lasted for 2 months or less and 18% (10/56) for between 3 months and 4 years. The ESR was moderately elevated during the attacks. In 32 specimens the synovial fluid was inflammatory (cell count 14.7 +/- 10.1 x 10(9)/l), but in 19 (59%) a good mucin clot formed. Synovial biopsy in 12 patients revealed superficial ulceration, paucity of plasma cells, and in 5 instances lymphoid follicle formation.

Short term cyclosporin A treatment of Behcet's disease.

Eleven separate three-month courses of cyclosporin A, an oral solution 10 mg/kg/day, were administered to eight patients with Behçets 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 Behçets 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.

Olecranon nodules in a case of Behçet's disease.

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

Lack of Correlation between Skin Reactivity and T Lymphocytes and Monocytes in Hodgkin’s Disease

28 untreated patients with Hodgkins 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 Hodgkins 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 Hodgkins disease.

Polycythemia Vera Associated with Usher's Syndrome

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

Treatment of Ocular Involvement in Behçet’s Disease with Ciclosporin (Preliminary Report)

Behcet’s disease is a multisystem disease characterized by oral and genital ulcers, ocular involvement in various forms, arthritis, thrombophlebitis and neurological involvement. The ocular involvement in Behcet’s disease, encountered in about 50% of the cases, constitutes a major hazard because it may lead to blindness especially in young patients [5]. The ocular disease was first described by Behcet [1] as iridocyclitis sometimes associated with hypopion. In addition to the originally described lesions, conjunctivitis, episcleritis, keratitis, hemorrhage and cellular infiltrations into the vitreous body, retinitis, retinal thrombophlebitis, optic atrophy, macular and papilla-edema have been described [2].

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.