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About a quarter of familial Mediterranean fever (FMF) patients have recurrent painful attacks of polyserositis despite regular colchicine treatment. There is no known alternative drug for colchicine-resistant cases. We had previously observed a patient with FMF whose painful attacks disappeared during the 6 month period of interferon alpha (IFN) treatment for his chronic hepatitis B. The objective of the present study was to investigate the possible beneficial effect of IFN on these episodes. Twenty-one consecutive attacks in seven adult patients with FMF were treated at early onset with IFN, the dosage being 3-10 million I U s.c. Eighteen of the 21 attacks could be halted in a mean time of 3.05 h, while the intensity of abdominal pain remained very low. Observed side-effects were generally mild and acceptable. IFN may be a useful adjunct for the treatment of colchicine-resistant attacks in FMF patients. [ABSTRACT FROM PUBLISHER]

We surveyed the 10-yr mortality among 152 Behçet's syndrome (BS) patients who had registered at a BS out-patient clinic and compared it to the expected mortahty in the general population. Information on mortality was available in 79% of the study group, among whom six patients (all male) had died. The observed mortality of two patients in the 15–24 yr age bracket was significantly above that expected in the general population. BS is a cause of increased mortality in the young male patients. [ABSTRACT FROM PUBLISHER]

Purpose: To evaluate satisfaction of acromegalic subjects with their medical treatment and its contribution to their quality of life., Methods: This cross-sectional study included a total of 159 medications used in 133 subjects with acromegaly (controlled n = 84 and uncontrolled n = 49, female/male: 81/52). Subjects were asked to complete questionnaires on symptoms of depression (BDI) and satisfaction with the medical therapy they received for acromegaly (TSQM). Acromegaly cases also completed Acromegaly Quality of Life Questionnaire (AcroQoL)., Results: Subjects on pegvisomant therapy scored lower on convenience (p = 0.007). Cases on combination therapy had lower domain scores for effectiveness, convenience and global satisfaction in comparison to the cases on monotherapy (p = 0.01, p = 0.01 and p = 0.01, respectively). The time elapsed since diagnosis and the duration of medical therapy were positively correlated with effectiveness score (r = 0.2, p = 0.007 and r = 0.2, p = 0.04, respectively). The AcroQoL score was positively correlated with all domains of TSQM (for effectiveness r = 0.2, p = 0.01; for side effects r = 0.3, p = 0.001; for convenience r = 0.3, p = 0.004 and for global satisfaction r = 0.2, p = 0.01). In contrast, the BDI score was inversely correlated with all domains of TSQM (for effectiveness r = -0.3, p = 0.001; for side effects r = -0.2, p = 0.006; for convenience r = -0.3, p < 0.001 and for global satisfaction r = -0.3, p = 0.001)., Conclusion: In acromegaly, quality of life, status of depression and satisfaction of the subjects with their treatment are intercorrelated.

Background: Because there is an increased inflammatory response to trauma, particularly of the skin, in patients with Behçet's syndrome (BS), an alteration in wound healing in BS is expected. The aim of this study was to investigate the healing features of punch biopsy wounds in BS and acne vulgaris (AV) patients used as controls., Method: Full-thickness skin punch biopsies (4 mm) were taken from the hairless sites of the non-dominant forearms in 20 BS and 20 AV patients. Each patient was examined on days 1, 2, 3, 4, 5, 8, and 10, and the biopsy wound area and induration were marked on sterile glass slides. Other inflammatory changes, such as suppuration and pain, were also recorded. No antiseptic solutions or ointment, except saline, were used., Results: The wound area healed similarly in both groups (p > 0.05). Thirteen (65%) BS patients had erythematous haloes around the wound on the first day. The number of patients presenting inflammatory changes reached 18 (90%) on the second day in the BS group. The area of erythema around the wound in BS patients was significantly greater than that in AV patients (5; 25%). It gradually decreased day by day. Purulent changes were observed in four BS patients on day 1, and induration around the biopsy wound in six patients on day 2. Only one patient with AV had suppuration, which appeared on the second day, while no AV patient displayed induration., Conclusion: Biopsy-induced trauma may cause increased inflammation in BS, but wound healing is not altered.

Objective: To assess the effect of azathioprine (AZA) treatment on long-term prognosis in Behçet's syndrome., Methods: Patients (all male) who took part in a double-blind, placebo-controlled trial of AZA a mean +/- SD of 94 +/- 10 months previously were reevaluated., Results: The emergence of blindness (log rank chi2 = 5.6, P = 0.02) and a 2-line drop in the visual acuity of the right eye (log rank chi2 = 5.9, P = 0.015) occurred significantly more frequently among the patients originally allocated to the placebo group compared with patients who originally received AZA, despite posttrial treatment for patients in both groups when needed. There was also a trend toward more frequent occurrence of extraocular complications in the placebo group. The beneficial effect of AZA was especially pronounced among patients who had eye involvement of short duration prior to their entry into the trial., Conclusion: Early treatment with AZA tends to favorably affect the long-term prognosis of Behçet's syndrome.

Background: The surgical therapy of Behçet aneurysms is often unsuccessful, resulting in graft occlusions, anastomoses, and/or new aneurysms., Methods: Twenty-nine aneurysms were documented in 24 Behçet's patients during a period of 19 years. All patients were male, ranging in age from 20 to 53 years (mean, 35 +/- 7.3 years). The mean duration of disease was 9 +/- 5 years. There were nine abdominal aorta, four iliac, three common femoral, five superficial femoral, four popliteal, one subclavian, one carotid, and one posterior tibial artery aneurysm. In addition, in one patient an aneurysm developed from the arterialized venous conduit that had been inserted for a common femoral artery aneurysm elsewhere. Five patients were already under immunosuppressive therapy for ocular problems at the time of diagnosis. Fifteen patients received immunosuppressive therapy after operation. We performed one abdominal aneurysmorrhaphy, two iliac artery PTFE graft interpositions, two aortobiliac bypasses (PTFE), six aortic tube graft (three PTFE, three Dacron) interpositions, one avrtofemoral bypass (PTFE), two iliofemoral bypasses (PTFE), two superficial femoral artery graft (PTFE) interpositions, and three popliteal graft interpositions (one PTFE, two vein graft). Also as an initial procedure one carotid, one subclavian, four superficial femoral, one popliteal, and one posterior tibial artery were ligated., Results: Nineteen patients were followed up for a mean duration of 47.3 +/- 27 months (range, 1 to 108 months). The patient with a subclavian aneurysm died of massive bleeding on postoperative day 15. Four patients were lost to follow-up. In the abdominal aortic aneurysm group one patient died of gastrointestinal bleeding 4 years after the operation. Another patient from the same group died 5 years after operation without any vascular disease. In the common femoral artery group the patient with an occluded iliofemoral graft died of an exsanguinating pulmonary artery aneurysm in the first year after operation. Overall, there were five anastomotic aneurysms. In addition, after the initial operation two iliofemoral, one aortofemoral, and one popliteal interposition graft were occluded without disabling ischemia., Conclusions: Aneurysms limited to the extremities could be ligated without disabling ischemia. Abdominal aortic aneurysms could be treated with tube graft insertion, giving satisfactory results. Patients could tolerate graft occlusion without major ischemia.