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An abstract of the conference paper "International cohort study for pediatric Behçet's Disease: Update 2011," by M. Darce Bello and colleagues is presented.

Objective: To set-up an international cohort of patients suspected with Behçet's disease (BD). The cohort is aimed at defining an algorithm for definition of the disease in children., Methods: International experts have defined the inclusion criteria as follows: recurrent oral aphthosis (ROA) plus one of following-genital ulceration, erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis and family history of BD. Onset of disease is <16 years, disease duration is ≤3 years, future follow-up duration is ≥4 years and informed consent is obtained. The expert committee has classified the included patients into: definite paediatric BD (PED-BD), probable PED-BD and no PED-BD. Statistical analysis is performed to compare the three groups of patients. Centres document their patients into a single database., Results: At January 2010, 110 patients (56 males/54 females) have been included. Mean age at first symptom: 8.1 years (median 8.2 years). At inclusion, 38% had only one symptom associated with ROA, 31% had two and 31% had three or more symptoms. A total of 106 first evaluations have been done. Seventeen patients underwent the first-year evaluation, and 36 had no new symptoms, 12 had one and 9 had two. Experts have examined 48 files and classified 30 as definite and 18 as probable. Twenty-six patients classified as definite fulfilled the International Study Group criteria. Seventeen patients classified as probable did not meet the international criteria., Conclusion: The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.

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).

Still's Disease of the adult is a systemic disease that the cause is unknown. If the pediatric forms are frequent, the affection of the adult is rare and its diagnosis is difficult. The purpose of this study is to analyse the features of the clinical evolution of adult Still's disease and to compare our results with the literature. It is a retrospective study about 21 years that permitted to collect 11 cases according the criteria of Yamaguchi. It is a matter of 6 men and 5 women that the mean age is 31 years old (age range: 16 to 48 years old). The fever was constant, the skin rash was noticed in 8 patients (72.7%); a polyarthritis was noticed in all the patients, chronic in 10 cases (90.9%) which 2 erosive forms. Adenopathies were present in 5 patients, a splenomegaly and a hepatomegaly were noticed respectively in 4 and 2 cases. Inflammatory syndrome with hyperleukocytosis was constant, and a hepatic cytolysis was noticed in 80%. The total ferritinemia titrated in 8 patients was constantly high. The hemocultures realized in all the patients were sterile and the complete immunologic examination was negative. The strong dose of corticoid was prescribed with success in all the patients. The immediate evolution was favourable in 10 patients. We deplore one death after a state of deep denutrition. Still's disease of the adult is rare, its diagnosis is difficult, sensitive to corticotherapy and its clinical evolution in our country is comparable to the literature.

Objective: Osteo-articular sarcoidosis may be evoked in the presence of peripheral articular manifestations or bone lesions that are sometimes asymptomatic. The aim of this work is to describe clinical and progressive features of sarcoidosis with osteo-articular involvement., Methods: Our retrospective study concerned 18 patients presenting with osteo-articular sarcoidosis from 1985 to 1999. We included patients with clinical diagnosis suggestive of sarcoidosis and with at least one positive biopsy., Results: Among 35 cases of sarcoidosis, 18 patients had an osteo-articular manifestation (51.42%), which revealed the disease in 2 patients. The female sex was predominant (sex ratio M/F of 0.12), the mean age was 47 years and the time before diagnosis was 3.6 years. Articular involvement was the most frequent. Inflammatory joint pains were present in 11 cases, a Lofgren syndrome in 2 cases, a chronic arthritis in 4 cases and acute monoarthritis of the elbow in 1 case. A female patient exhibited a probable association with a spondylarthropathy. The bone involvement, revealing the disease in 1 case, was also noted in 5 cases, located exclusively on hands; this sarcoidosal dactyly was represented in 2 cases in the form of phalangeal geodes, in wired form (2 cases) and in large bulla form (1 case). The bone biopsy when it was performed was positive in all 3 cases. The patients responded well to corticosteroids., Conclusion: The osteo-articular involvement of sarcoidosis is polymorphic and can reveal the disease or may appear during the course of its progression.

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

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.