ABSTRACT
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.
Subject(s)
Behcet Syndrome/physiopathology , Musculoskeletal Diseases/physiopathology , Registries , Adolescent , Age of Onset , Algorithms , Behcet Syndrome/genetics , Child , Child, Preschool , Cohort Studies , Databases, Factual , Diagnosis, Differential , Disease Progression , Female , Follow-Up Studies , Genetic Predisposition to Disease , Humans , International Cooperation , Male , Musculoskeletal Diseases/genetics , Pedigree , Severity of Illness Index , Sex Factors , Statistics as Topic , Young AdultABSTRACT
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.
Subject(s)
Still's Disease, Adult-Onset/diagnosis , Adolescent , Adult , Anti-Inflammatory Agents/therapeutic use , Arthritis/etiology , Diagnosis, Differential , Exanthema/etiology , Female , Fever/etiology , Hepatomegaly/etiology , Humans , Hydrocortisone/therapeutic use , Lymphatic Diseases/etiology , Male , Middle Aged , Retrospective Studies , Splenomegaly/etiology , Still's Disease, Adult-Onset/complications , Still's Disease, Adult-Onset/drug therapy , Treatment OutcomeABSTRACT
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.
