[Value of MRI in the treatment of Grave's disease orbital myopathy]. / L'intérêt de l'IRM dans le traitement de la myopathie de l'orbitopathie dysthyroïdienne.

In order to evaluate the predictability of the results in the treatment of myopathy in cases with the clinical signs of muscle involvement, 177 extraocular muscles of 27 cases whose oedematous status was detected by MRI and who were given antiinflammatory treatment according to the data of this method, were studied. The nature of involvement was detected in respect with the signal intensity and thickness of each rectus muscle prior to the treatment and at the end of the sixth month following a three months' application of combined treatment of steroids and irradiation of 2000 rads. When the initial and final results were compared, the signal intensities of four involved recti showed significant decrease at the end of the treatment, as they were evaluated separately or together. Besides the thicknesses of these groups of involved recti which were evaluated separately showed significant decrease. The evaluation of the signal intensities by MRI is a way that enables noninvasive detection of the edema and prediction of the anti-inflammatory treatment's results of dysthyroid myopathy. Therefore a systematic follow up by MRI is recommended for the treatment choice in dysthyroid myopathy.

Subconjunctival cyst extending into the orbit.

A fourteen year old girl who had developed a large subconjunctival mass extending into the orbit is presented. The mass had appeared after a strabismus operation, and it caused a consecutive exotropia and decreased adduction in the affected eye. She had had this cyst for six years. A cranial CT scan confirmed the presence of a massive orbital cyst. This cyst was removed surgically. Postoperatively, exotropia and adduction limitation improved. The pathological report stated that it was an epithelial cyst. This cyst and its differential diagnosis is the subject of this paper.

[Ocular site of pigmentation in oculodermal melanocytosis]. / Localisation oculaire de la pigmentation dans la mélanocytose oculodermique.

Sixteen patients with oculodermal melanocytosis (Nevus of Ota, congenital ocular melanocytosis) were investigated for the location and the density of the ocular pigmentation, the increase in intraocular location and the malignant transformation. In all cases, conjunctivo-scleral dispersion pattern appeared as dispersed patches without any predominant site but a diffuse involvement of the angle. This finding points out that ODM may exhibit a characteristic dispersion pattern of pigmentation which is characteristic for ODM. For 14 patients intraocular pressures were < or = 18 mmHg, and for remaining 2 patients 21 and 22 mm Hg. Glaucomatous optic disc or perimetric changes were not found in any patient. In one case, a histologically verified ciliary-choroidal melanoma at a superior-temporal site co-existed at the same location as the ODM.

The prevalence of Sjögren's syndrome in Behçet's syndrome.

OBJECTIVE: The role of autoimmune mechanisms in Behçet's syndrome (BS) is debated. Sjögren's syndrome (SS) accompanies most autoimmune diseases. Thus we investigated the prevalence of SS in BS in a formal protocol. METHODS: The study was conducted in 2 phases. During the first phase subjective symptoms of dryness were blindly assessed by questionnaires and Schirmer I and Saxon tests were done in 67 patients with BS and 100 healthy and diseased controls. During the 2nd phase 30 patients with BS and 19 with rheumatoid arthritis (RA) had salivary gland biopsies along with rose bengal dye, Schirmer I and tear breakup time tests. RESULTS: During the first phase only patients with primary SS had significantly abnormal findings. However during the 2nd phase pathologic changes in salivary gland biopsies and positive RB tests were highly significantly more prevalent among patients with RA. CONCLUSION: SS is not a feature of BS.

[Malignant melanoma of the choroid in vivo. Comparative biometry using RX scanner and MRI]. / Contribution à l'étude du mélanome malin de la choroïde in vivo. Biométrie comparée par scanner RX et IRM.

Uveal melanomas are unique among the malignant tumors of the eye investigated by MRI in that both T1 and T2 are relatively shortened due to the paramagnetic effect of melanin. Bearing in mind this property, we conducted a comparative study between MRI and CT in 11 patients with histologically proven choroidal malignant melanoma. The results of this study confirm that MRI is far superior to CT in both differential diagnosis and in determining the extent of the tumor which is crucial if conservative treatment is to be undertaken.

Low dose cyclosporin A versus pulsed cyclophosphamide in Behçet'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 Behçet's 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 Behçet's syndrome are warranted.

A controlled trial of azathioprine in Behçet's syndrome.

Cytotoxic agents have long been used in Behçet's 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 Behçet's 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 Behçet's syndrome, especially its most serious manifestation, eye disease.

The prevalence of Behçet's syndrome in a rural area in northern Turkey.

We conducted a field survey to establish the prevalence of Behçet's syndrome among all the inhabitants aged 10 years or older in a village in northern Turkey. The prevalence of Behçet's syndrome was 19/5,131. There were 13 females and 6 males. None had eye involvement. Pathergy test was positive in 6/18 (33%) and 5/19 carried HLA-B5 (26%), suggesting that the previously established association of HLA-B5 in Behçet's syndrome is mainly true for hospital based populations. Moreover, the pathergy test was less commonly positive in milder disease.

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.

Von Willebrand factor in Behçet's syndrome.

Plasma von Willebrand factor F VIII related antigen levels were elevated in 13 patients with Behçet's syndrome and vascular disease compared to 17 with Behçet's syndrome without vascular involvement and 23 healthy controls. As expected 12 diseased controls, 9 of whom had systemic sclerosis, had still higher levels of F VIII related antigen.

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.

Male patients with Behçet's syndrome have stronger pathergy reactions.

The effects of patient's sex and age at onset on the pathergy reaction (cutaneous hypersensitivity to a needle prick) and its correlation with disease activity in Behcet's syndrome was investigated by two independent observers in a blind protocol. Among 92 male patients the pathergy reaction was more strongly positive (p less than 0.025) than among 67 female patients of similar age and disease duration. The age at onset did not affect the severity of the pathergy reaction, although, the early onset females (age at onset 24 years or less) had the lowest prevalence of pathergy positivity (52%), compared to early onset males and late onset (age at onset 25 years or more) males and females (73-75%). As previously reported the disease was more severe among males and among those with early onset of either gender. On the other hand, no correlation between the strength of the pathergy reaction and clinical severity could be discerned.

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.

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

A prospective study of arthritis was performed in 47 patients with Behçet's 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.

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.