Humans ; Arthritis, Juvenile/drug therapy* ; China

Adrenal Cortex Hormones ; administration & dosage ; therapeutic use ; Arthritis, Juvenile ; drug therapy ; Humans ; Injections, Intra-Articular

Arthritis, Juvenile ; drug therapy ; Glucocorticoids ; adverse effects ; pharmacology ; therapeutic use ; Humans

Adolescent ; Arthritis, Juvenile ; diagnosis ; drug therapy ; Child ; Female ; Follow-Up Studies ; Humans ; Male

Child ; Humans ; Arthritis, Juvenile/drug therapy* ; Macrophage Activation Syndrome/etiology* ; Antibodies, Monoclonal, Humanized/therapeutic use*

OBJECTIVE: To explore the clinical characteristics and biological treatment of juvenile Idiopathic arthritis (JIA) after adulthood. METHODS: Selected 358 patients with previous medical history diagnosed by JIA who were hospitalized in the Department of Rheumatology and Immunology, West China Hospital of Sichuan University from January 1, 2009 to January 1, 2019. Perform retrospective analysis of basic information, clinical symptoms, diagnostic indicators, treatment plans, outpatient follow-up (inpatients require outpatient follow-up treatment) and diagnosis and treatment process of 90 eligible cases included, and observe different ages and different courses of disease. The clinical characteristics of young and middle-aged idiopathic arthritis in adults and the outpatient situation of using biological agents for 6 months. RESULTS: According to age, they were divided into ≤26 years old group (42 cases) and >26 years old group (48 cases). Under examination [rheumatoid factor (RF), anti-nuclear antibody (ANA), anti-neutrophil antibody (ANCA), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), interleukin-1β (IL-1β), interleukin 6 (IL-6), hemoglobin (HGB), white blood cell count (WBC), human leukocyte antigen-B27 (HLA-B27), complement 3 (C3), etc.], concurrent in terms of symptoms, treatment and prognosis, the ≤26-year-old group was generally lighter than the >26-year-old group; that was, the older the age, the heavier the onset of inflammation and other symptoms, the more complications, the worse the treatment effect, and the worse the prognosis, and there were statistical differences academic significance (P < 0.05). According to the course of disease, they were divided into ≤19 years group (46 cases) and >19 years group (44 cases). In terms of examination (RF, ANA, ANCA, ESR, CRP, IL-1β, IL-6, HGB, HLA-B27, C3, etc.), complications, treatment and prognosis, the course of disease ≤19 years group was compared with the disease course> 19 years group Overall mild; that was, the longer the course of the disease, the more severe the onset of symptoms such as inflammation, the more complications, the worse the treatment effect, and the worse the prognosis, P < 0.05, the difference was statistically significant. After 6 months of outpatient treatment with biological agents, it was found that biological agents could improve some of the patients' clinical symptoms and delay the further development of the disease. Compared with the non-biological agent treatment group (48 cases), the biological agent group (42 cases) benefited, and the difference was statistically significant (P < 0.05). CONCLUSION Through retrospective analysis, this article believes that although adult JIA is diagnosed as connective tissue disease, it has special clinical characteristics with the course of the disease and age. Therefore, it should be recommended to give special attention to JIA patients after adulthood, require regular medical treatment in the adult rheumatology department, according to the corresponding connective tissue disease or JIA diagnosis, and standard treatment; at the same time, pay attention to the history of JIA. In the comparison of biological and non-biological treatment, it is proved that biological treatment can effectively improve some of the clinical symptoms of JIA patients after adulthood. Therefore, it is recommended that biological treatment be used as soon as possible if economic conditions permit to delay the development of the disease.
Adult ; Arthritis, Juvenile/drug therapy* ; Blood Sedimentation ; China ; Humans ; Infant ; Middle Aged ; Retrospective Studies ; Rheumatoid Factor

OBJECTIVETo evaluate the efficacy of thalidomide in the treatment of juvenile idiopathic arthritis (JIA).

METHODSTwelve children with JIA who did not respond to conventional treatment were administered with thalidomide (2 mg/kg daily). The symptoms, signs, and laboratory test results were compared before and after treatment. The thalidomide-related side effects were observed.

RESULTSThe average dosage of prednisone was reduced from 1.92 ± 0.16 mg/kg•d to 0.49 ± 0.42 mg/kg•d in the 12 patients 6 months after thalidomide treatment (P<0.01). Four patients did not need prednisone treatment any more. White blood cell count, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and serum ferritin (SF) significantly decreased after treatment in all of 12 patients (P<0.01). Hemoglobin level increased to normal in 8 patients after treatment (P<0.01). The number of affected joints decreased from 5 before treatment to zero to 2 after treatment in patients with polyarticular JIA (P<0.01). Signs of hip involvement and Schober's sign turned negative in enthesitis-related cases. No thalidomide-related side effects were observed.

CONCLUSIONSThalidomide is effective in the treatment of JIA in children who do not respond to conventional treatment.

Adolescent ; Arthritis, Juvenile ; blood ; drug therapy ; Child ; Female ; Humans ; Male ; Prednisone ; therapeutic use ; Retrospective Studies ; Thalidomide ; therapeutic use

Macrophage activation syndrome (MAS) is one of the serious complications of juvenile rheumatoid arthritis (JRA) and recently, cyclosporine A has been found to be effective in patients with corticosteroid-resistant MAS. A 29-yr-old male was admitted with high fever and jaundice for one month. He was diagnosed as juvenile arthritis 16 yr ago. Physical and laboratory results showed hepatosplenomegaly, high fever, pancytopenia and impaired liver and renal function tests, elevated triglyceride and serum ferritin levels. Bone marrow biopsy showed hyperplasia of histiocytes with active hemophagocytosis. He was diagnosed as MAS associated with juvenile rheumatoid arthritis and managed with high-dose corticosteroids initially, but clinical symptoms and laboratory findings did not improve immediately. Finally, he completely recovered after treatment with cyclosporine A (3 mg/kg/day).
Treatment Outcome ; Pancytopenia/*drug therapy ; Male ; *Macrophage Activation ; Lymphatic Diseases/*drug therapy ; Immunosuppressive Agents/therapeutic use ; Humans ; Cyclosporine/*therapeutic use ; Arthritis, Juvenile Rheumatoid/*drug therapy ; Adult

OBJECTIVEMacrophage activation syndrome (MAS) is a rare but life-threatening complication in children with rheumatic diseases, particularly systemic-onset juvenile idiopathic arthritis (SOJIA). Because of the potential fatality of this condition, prompt recognition and immediate therapeutic intervention are important. This study reviewed the data of MAS in 13 cases with SOJIA.

METHODSRetrospective review was performed on the precipitating events, clinical manifestations, laboratory data, treatment, and outcome of macrophage activation syndrome in 13 children with SOJIA seen from 1996 to 2005.

RESULTSOver the past 10 years the unit has had 90 new patients with SOJIA. Thirteen of those patients (14.4%) developed MAS during the course of their primary SOJIA, of whom ten were male. All patients were noted to have active SOJIA prior to developing MAS; 3 patients had medications, which were considered as trigger factors; 8 had infections prior to MAS, in two of them the infections were possible triggers. All the patients had high grade fever; 12 cases (92.3%) had hepatomegaly; 10 patients (76.9%) had coagulopathy, and eight patients (61.5%) had central nervous system dysfunction. The counts of platelet, white blood cells and the mean erythrocyte sedimentation rate fell dramatically in all patients; hyperferritinemia was identified in 8 patients, in 5 of whom serum ferritin (SF) was >or= 10,000 microg/L; in 8 (72.7%) of 11 cases fibrinogen was or= 2.5 mmol/L in 9 (69.2%) of 13 cases.

CONCLUSIONMAS is a rare and potentially fatal complication of children with SOJIA. Primary disease activity, medications and infections preceding MAS were all important triggers. The strongest clinical discriminators were hepatomegaly, hemorrhages and central nervous system dysfunction. The strongest laboratory tests were decreased counts of platelet and white blood cells, decreased ESR and fibrinogen, dramatically increased SF and TG. It calls for the immediate treatments, particularly with cyclosporin A, which are often effective.

Arthritis, Juvenile ; complications ; drug therapy ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Macrophage Activation Syndrome ; drug therapy ; etiology ; pathology ; Male ; Retrospective Studies

OBJECTIVETo review and analyze the clinical features, treatment, and outcome of macrophage activation syndrome (MAS) in children with systemic onset juvennil rheumatoid arthritis (SOJRA).

METHODRetrospective review and analysis were performed on cases with MAS from a prospectively collected database of children with SOJRA from the year of 2003 to 2006 in the Hospital.

RESULTSTwenty four patients (21 boys, 3 girls) were diagnosed as having MAS with SOJRA. Mean age of the patients with MAS at diagnosis was 7 years, and the duration prior to diagnosis of MAS was 12 months. No trigger factors were found except in one case whose MAS was triggered by use of methotrexate and in another by parvovirus B19 infection. High grade fever, new onset hepatosplenomegaly and lymphadenopathy, pancytopenia, liver dysfunction were common clinical features in all the 24 cases (100%). Bleeding from skin, mucous membrane and gastrointestinal tract were noted in 9 cases (38%). Twelve (50%) cases had CNS dysfunction (high intracranial pressure, seizure and coma). Six cases (25%) developed ARDS. One patient suffered from renal damage. The laboratory test revealed elevated live enzymes and ferritin, decreased value of ESR, albumin, complete blood count and fibrinogen in all the 24 cases. Bone marrow examination supported the diagnosis of definite hemophagocytosis in the 24 cases. Lymph node biopsy was done for one case and histopathological examination showed that the node was full of activated macrophage. As to treatment, five cases only received high dose steroids (three of them died), 14 cases were treated with high dose steroids plus cyclosporine (one died), two were treated with steroids plus cyclosporine and etoposide (none died). The causes of deaths were ARDS and CNS involvement. In three of the cases who died, treatment was given up by their parents.

CONCLUSIONSMAS is a rare and potentially fatal complication of SOJRA. Most of our patients were male. Bone marrow studies support the diagnosis. CNS involvement and ARDS were poor prognostic signs. Early diagnosis and aggressive therapy are essential.

Adolescent ; Arthritis, Juvenile ; complications ; drug therapy ; pathology ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Macrophage Activation Syndrome ; drug therapy ; etiology ; pathology ; Male ; Retrospective Studies