[Vogt-Koyanagi-Harada syndrome. Eight cases]. / Syndrome de Vogt-Koyanagi-Harada. A propos de huit cas.

PURPOSE: Vogt-Koyanagi-Harada disease is a rare systemic affection characterised by the association of ocular inflammatory manifestations and extra ocular lesions such as meningismus, tegumentary or auditory findings observed in pigmented population. Auto-immune origin of this syndrome is probable; a T-lymphocyte-mediated autoimmune process is directed against an unidentified antigen associated with melanocytes. METHODS: The aim of this retrospective study is to determine their clinical profile in our country. RESULTS: Eight consecutive cases of Vogt-Koyanagi-Harada disease were studied during a 22-year period. All the patients fulfilled the criteria of American Uveitis Society. All patients were female and had bilateral ocular involvement as panuveitis. Retinal serous detachment was observed in 4 patients, meningitis in 6 cases. 7 patients had hearing loss and 5 patients had cutaneous lesions represented by poliosis. Corticosteroids and/or immunosuppressive therapy were administered in all patients leading to improvement in 5 patients. CONCLUSION: Our series are concording with a usually good ocular prognosis. Immunosuppressive therapy should be used early in posterior segment involvement.

[Neurological involvement in Behçet's disease. 154 cases from a cohort of 925 patients and review of the literature]. / Manifestations neurologiques de la maladie de Behçet.

INTRODUCTION: Neurological involvement is well described in Behçet's Disease (BD), with variable prevalence of 5.3 to 30p.cent. The purpose of this retrospective study was to analyze the clinical patterns of neuro-Behçet (NB) and to compare them with different clinical features of 925 BD registered in the same period and the literature. METHODS: All patients of NB fulfilled the International Study Group Criteria for the diagnosis of BD. 93 patients with headache, without other neurological symptoms were excluded. The findings were supported by cerebrospinal fluid, computed tomography scan, magnetic resonance imaging and angiography. RESULTS: NB was present in 16.64p.cent of BD. A sex ratio male/female: 4.31. The mean age of patients with NB was 31.76 years. The average age of onset of NB was 29.83. The mean duration of the NB disease was 3.81 years. The findings were categorised in 2 main types: parenchymal and non-parenchymal involvement. Some patients had features of both types (mixed patterns). 94 patients (61.03p.cent) presented with parenchymal central nervous system (CNS) involvement. The most common findings were pyramidal signs, cranial nerve palsies, pseudobulbar syndrome and cerebellar signs. 27 patients (17.53p.cent) without parenchymal CNS which were divided into: intracranial hypertension in 24 patients (15.58p.cent) presenting headache, vomiting and bilateral papilloedema; cerebro-arterial involvement in 3 cases (one of them had cerebrovascular aneurysms). Mixed patterns were observed in 9 cases. Erythema nodosum and vascular involvement were more frequent in intracranial hypertension than in BD. Other clinical features were reported: pure meningeal pattern in 15 cases, pure peripheral nervous system involvement in 4 cases, isolated cranial nerves in 8 cases, chorea in one patient and pseudotumor of cervical medulla in other patient. 6 cases of juvenile BD and one case of familial Behçet were observed. 133 patients were treated (86.36p.cent). Mean duration of treatment was 7.46 month. We used corticosteroids, immunosuppressive agents (cyclophosphamid, azathioprine, chlorambucil) and anticoagulant in intracranial hypertension. The course of disease was good in only 54.13p.cent of cases, and was bad in 18.79p.cent. CONCLUSION: NB occurred frequently in men and is more serious especially in parenchymal CNS involvement: 7 deaths (6 from parenchymal CNS).

[Macular damage in Behçet's disease]. / La maculopathie dans la maladie de Behçet.

INTRODUCTION: The macular damage in Behçet's disease requires particular attention because of its poor prognosis. PURPOSE: The purpose of our study was to specify the frequency of macular alterations in Behçet's disease and discuss the clinical, physiopathological and therapeutic aspects. MATERIALS AND METHODS: Our report is based on 244 patients with Behçet's disease examined between January 1995 and December 1997. The authors studied macular alterations in detail. RESULTS: Sixty-nine patients had a maculopathy affecting 108 out of 432 eyes (24%). The mean progression of Behçet's disease in these cases was 4 years. Visual acuity was

[Rheumatic manifestations of Behçet's disease]. / Manifestations rhumatismales de la maladie de Behçet.

UNLABELLED: Rheumatologic manifestations are common in Behçet's disease. Joint involvement takes the third place after mucocutaneous and ocular lesions and can be the inaugural manifestation. Monoarthritis and oligoarthritis affect essentially knees and ankles, with a marked male bias. They usually run an acute or recurrent course, with chronic forms being rare. Polyarthritis is not rare and involves the large limb joints and the small joints of the hands and feet. Arthritis in Behçet's disease heels usually without sequelea and the aggressive treatment is not necessary. Unusual forms include arthritis with deformities and/or destruction, pseudogout, rupture of popliteal cyst (imitating deep vein thrombosis) and myositis. The association with spondylarthropathy is not common. Behçet's disease is rarely associated with rheumatoid arthritis, Sjögren's syndrome, systemic lupus erythematosus, relapsing polychondritis and amyloidosis. Children are more likely than adults to have joint manifestations and polyarthritis. CONCLUSION: Joint manifestations are common in Behçet's disease. Their unusual forms deserve to be known since they can raise diagnostic problems when they are inaugural.

Joint manifestations in Behçet's disease. A review of 340 cases.

OBJECTIVES: To gain additional knowledge on the joint manifestations of Behçet's disease, with special attention to unusual forms. PATIENTS AND METHODS: Retrospective review of 340 cases with joint manifestations identified among 601 cases of Behçet's disease seen over a 15-year period. All the patients met International Study Group for Behçet's disease criteria. Radiographs of all joints with arthritis were obtained. Starting ten years ago, a radiograph of the sacroiliac joint was taken routinely. RESULTS: Joint manifestations were present in more than half the patients (56.57%) and were inaugural in 18.23% of cases. The knees and ankles were the joints most commonly affected. Monoarthritis and oligoarthritis were seen in 16.17% and 11.76% of cases, respectively and polyarthritis involving the large limb joints and the small joints of the hands and feet in 17.05% of cases. Unusual forms included polyarthritis with deformities and/or destruction (n: 8, including two patients who also met criteria for rheumatoid arthritis), pseudogout (n: 5), popliteal cyst (n: 3, including one case imitating deep vein thrombosis), myositis (n: 1), spondylarthropathy (n: 5), and Sjögren's syndrome (n: 2). Children were more likely than adults to have joint manifestations (73.68%) and polyarthritis (35.7%). CONCLUSION: Joint manifestations are common in Behçet's disease. Their unusual forms deserve to be known since they can raise diagnostic problems when they are inaugural.

[Ophthalmological involvement in Behçet disease. Apropos of 520 cases]. / L'atteinte ophtalmologique dans la maladie de Behçet. A propos de 520 cas.

PURPOSE: The aim of this paper is to compare our results with the literature and to discuss some therapeutical aspects of the disease. METHODS: This retrospective study concerned 520 cases of Behçet's disease followed by internal diseases department and ophthalmology service of U.H.C. Averroes (Casablanca, Morocco) during 10 years. RESULTS: There were 432 men (83%) and 88 women (17%). The mean age of the patients was 20 years. Ophthalmological involvements are found in 80%, bilateral in 60%. The disease occurred at the rate of 2 or 3 episodes a year in 5% of cases. Irreversible blindness was noted in 24.4% of cases, predominately panuveitis in 37% of cases, followed by anterior uveitis in 36.3% and retinal vasculitis in 37% of cases. Good results were obtained by a medical care with corticoids and chloraminophen in case of threatening blindness. CONCLUSION: Behçet's disease remains frequent in our country. It requires early diagnosis and steady ophthalmological surveillance in order to delay onset of blindness.

[Systemic lupus erythematosus and Salmonella enteritidis osteomyelitis]. / Lupus érythémateux disséminé et ostéomyélite à Salmonella enteritidis.

Infections in systemic lupus erythematosus are frequent. However, osteoarticular Salmonella infections are rarely reported. We report a case of systemic lupus erythematosus diagnosed in a 15 year-old girl. Seven months later, she presented with fever and a localized collection of the upper extremity of the left tibia related to a Salmonella enteritidis acute osteomyelitis (sub periosteal abscess). The out-come was chronic and led to death. The authors emphasize the severity of non typhoidal salmonellosis in systemic lupus erythematosus.

[Rheumatoid arthritis in Morocco. Apropos of 404 observations]. / Polyarthrite rhumatoïde au Maroc. A propos de 404 observations.

A retrospective study of 404 cases of rheumatoid arthritis seen in a department of internal medicine in Casablanca highlights a number of specific features of the disease in Morocco. Onset occurred early and mean age of patients was 34.4 years. Analysis of joint manifestations showed that the disease tended to be mild in the hips and perhaps in the cervical spine. Thirty-five percent of patients were Steinbrocker's class II and 25.5% had carpal bone fusion. Only 20 patients had severely erosive disease, which manifested as giant geodes in 8 cases and as main en lorgnette deformity in one case. Subcutaneous nodules (7.9%) and systemic visceral disorders were fairly infrequent. Only three cases of malignant rheumatoid arthritis were found. Gougerot-Sjögren syndrome was present in 13.6% of patients. Among comorbid conditions, thyroid gland diseases and tuberculosis were fairly common. Serologic tests were positive in 61.14% of cases, often in low titres. Gold salt therapy was well tolerated. No patients in this group had surgical treatment. These data suggest that in Morocco rheumatoid arthritis may be less aggressive than in Europe.

[Desensitization by autologous saliva and Behçet's disease]. / Désensibilisation à la salive autologue et maladie de Behçet.

Among 240 patients with Behçet's disease seen in the Internal Medicine Department of the Ibnou Rochd University Hospital, Casablanca, between January 1981 and April 1988, 44 were selected to study the effects of desensitization by autologous saliva on oral and genital aphthae and on articular manifestations. Thirty of these 44 patients received gradually decreasing dilutions of their own saliva, and 14 control patients received physiological saline. Injections were administered intradermally twice a week during 15 weeks, then once a week during one year, so that the study lasted one and a half year, from October 1986 to April 1988. No improvement of the arthritis was observed, whereas the oral aphthae were improved in 50% of the patients desensitized by their own saliva. This percentage would have raised some hopes for the treatment of these sometimes disabling lesions were it not for the fact that 50% of the controls were similarly improved. This suggests that desensitization by saliva has a placebo effect.

[Behçet's disease. 316 cases]. / Maladie de Behçet. 316 cas.

The authors have reviewed the records of 316 patients with Behcet's disease diagnosed between 1981 and 1989 in the Internal Medicine Department of Casablanca's University Hospital, Morocco. Males aged between 20 and 40 years (78 percent) were most frequently affected (71 percent of the cases). All patients presented with muco-cutaneous lesions, including aphthous stomatitis (100 percent) and genital ulcers (85 percent). Skin hyperirritability was present in 77 percent. Ocular lesions were frequent (72 percent) and led to blindness in 88 patients. Articular manifestations occupied third rank in our series, with 60 percent of the cases. Three patients showed polyarthritis deformans, with destructive lesions in two cases and associated with Sjögren's syndrome in one case. Thrombophlebitis was present in 69 patients (superior vena cava thrombosis in 8 patients and Budd-Chiari syndrome in one), and it was complicated by pulmonary infarction in 6 cases. Other vascular lesions included middle cerebral artery thrombosis (1 case), cerebral arterial aneurysm (1 case) and pulmonary arterial aneurysm (1 case). Neurological manifestations were present in 50 patients (16 percent), in 13 of them related to mild intracranial hypertension. Ulcerative colitis was diagnosed in 2 cases and proteinuria in 4 others, with a nephrotic syndrome in 2 cases.

[Articular manifestations of Behçet's disease. Apropos of 73 cases]. / Les manifestations articulaires de la maladie de Behçet. A propos de 73 cas.

The authors report 73 cases of Behçet's disease with articular manifestations. Polyarthritis, generally considered to be rare, was found in 20.5 per cent of cases in this series. The unusual features are illustrated. Amongst them, two cases presented deforming and destructive lesions, which have been only exceptionally reported in the literature. A third case is unusual because of the association of Gougerot-Sjögren's syndrome with Behçet's disease and polyarthritis deformans. No cases of ankylosing spondylitis were observed. Lastly, the data of this series are compared with those of the literature.

[Selective immune deficiency in IgA. Intestinal malabsorption syndrome and mixed connective tissue disease]. / Déficit immunitaire sélectif en IgA. Syndrome de malabsorption intestinale et connectivite mixte.

We report the case of a 22-year old unmarried woman who presented with selective IgA immune deficiency, moderate intestinal malabsorption syndrome with pseudo-atrophy of the villi, and mixed connective tissue disease. Although immune deficiency and malabsorption syndrome are frequently associated, association with an autoimmune disease is rare and we were unable to find any case of association with a connective tissue disease in the literature.