Pregnancy and early onset pauciarticular juvenile chronic arthritis.
(9/962)
OBJECTIVES: To study interaction of early onset pauciarticular juvenile chronic arthritis (EOP-JCA) and pregnancy in the Polish population, in particular to confirm the ameliorating effect of pregnancy on disease activity reported by others and to analyse the factors that govern the occurrence of postpartum flare, with emphasis on the potential role of breast feeding. METHODS: The reproductive outcome and disease status in 39 adult women with history of EOP- JCA was examined by means of a questionnaire and an interview. In all patients the disease onset occurred before the 6th birthday, 19 had persistent pauciarticular JCA (PeEOP-JCA) and 20 had extended pauciarticular JCA (ExEOP-JCA). RESULTS: 23 women had at least one successful pregnancy, seven had unsuccessful pregnancies but all of them had also one or more successful pregnancies. Among those who have never been pregnant (n=16) there was a higher frequency of eye disease and ExEOP-JCA compared with the rest of the group. In almost all cases pregnancy was associated with remission of disease activity, however a postpartum flare appeared after 22 pregnancies (52%). The flares were more frequent in women who had an active disease before pregnancy, had a flare after a previous pregnancy and/or were breast feeding. CONCLUSIONS: In EOP-JCA patients pregnancy generally has a good outcome and induces amelioration of disease activity. After delivery, however, a flare of disease often appears, especially in women who were breast feeding, had a postparum flare previously or had an active disease before pregnancy. The pattern of interaction between disease and pregnancy found in EOP-JCA makes EOP-JCA similar in this respect to RA, but different from systemic lupus erythematosus and ankylosing spondylitis. (+info)
Giant cell arteritis associated with rheumatoid arthritis monitored by magnetic resonance angiography.
(10/962)
A 57-year-old Japanese woman with well controlled rheumatoid arthritis visited our hospital with a severe bitemporal headache and marked fatigue. Based on the classification criteria by the American College of Rheumatology, she was diagnosed as having giant cell arteritis. Magnetic resonance (MR) angiography was performed, from which stenotic changes in the bilateral superficial temporal arteries were strongly suspected. Corticosteroid therapy was quickly started. The patient followed an uneventful course with no complications. Therapeutic effect was confirmed by MR angiographic findings obtained 4 weeks after the initiation of therapy. (+info)
Sugar printing rheumatic diseases: a potential method for disease differentiation using immunoglobulin G oligosaccharides.
(11/962)
OBJECTIVE: To look for oligosaccharide structural variants of IgG that may be unique to specific rheumatic diseases. METHODS: Using normal-phase high-performance liquid chromatography technology, a comparison was made of the oligosaccharide pools released from serum IgG from patients with systemic lupus erythematosus (SLE) (n = 10), ankylosing spondylitis (AS) (n = 10), primary Sjogren's syndrome (n = 6), juvenile chronic arthritis (JCA) (n = 13), psoriatic arthritis (n = 9), rheumatoid arthritis (RA) (n = 5), and healthy control individuals (n = 19). RESULTS: The oligosaccharide pools were resolved into 13 peaks and the relative proportions of the peaks in each disease group was significantly different from that in healthy controls (P < 0.0001-0.05). A characteristic serum IgG oligosaccharide profile, or sugar print, for each of the rheumatic diseases was found. The sugar prints exhibited a range of glycosylation patterns whereby all RA (P < 0.0001) and JCA (P < 0.006) patients had predominantly agalactosyl structures, while SLE (P < 0.03-0.0001) and AS (P < 0.025-0.0001) patients had predominantly digalactosyl structures. CONCLUSION: The data suggest that each disease is associated with a specific mechanism that gives rise to alterations in the normal glycosylation pattern of IgG. Sugar printing of IgG is therefore a potential means for the differentiation of rheumatic diseases and may provide insight into disease pathogenesis. (+info)
Prognosis in children with rheumatic diseases: justification for consideration of new therapies.
(12/962)
It is evident that current approaches to the treatment of childhood arthritis, although much improved in the past decade, are still insufficient to halt the destructive progress of these diseases in many children. Furthermore, the treatment itself is associated with toxicity which may be prohibitive. The need for new and innovative treatments is urgent. ABMT is one possible avenue that offers hope to children with severe unresponsive disease. It will be important to select carefully those children who are likely to benefit from such an approach. An appreciation of the prognosis and possible predictors of disease severity should aid in this task. (+info)
The depletion of T cells from haematopoietic stem cell transplants.
(13/962)
OBJECTIVE: In our laboratory, we have developed an immunorosette technique for the depletion of T cells from bone marrow transplants. Tetrameric complexes of monoclonal antibodies are able to form very stable immunorosettes, which are efficiently depleted with the aid of a blood cell separator. Major improvements over the original sheep red blood cell depletion are the use of human (patient or donor derived) erythrocytes instead of sheep-derived cells, and the possibility of using a closed system for separation in a cell separator. In contrast to bone marrow, mobilized haematopoietic stem cell transplants obtained after leucocytapheresis contain higher numbers of T cells. Therefore, a different approach is necessary. METHOD: We have used two CD34 selection systems (Isolex 300SA and the Clinimacs) to perform T-cell depletions from peripheral blood stem cell (PBSC) transplants. RESULTS: Immunorosette T-cell depletion, with CD2/CD3 tetrameric complexes, of bone marrow transplants resulted in a mean 2.5 log depletion of T cells with a yield of 50% of the CD34+ cell population. Stem cell selection of PBSC transplants using one of the CD34 selection procedures resulted in a 4.5 log depiction of T cells for both systems, but with different results for the recovery of CD34+ cells. An increased yield of CD34+ cells was obtained with the Clinimacs procedure (57.9+/-9.0%) in comparison to the Isolex procedure (40.1+/-12.5%). CONCLUSION: Our own immunorosette depletion technique and the two tested CD34 selection methods for stem cell transplants both resulted in a very efficient T-cell depletion with the recovery of 40-60% of the CD34 haematopoietic stem cells present in the transplant. (+info)
Effectiveness and risks of total body irradiation for conditioning in the treatment of autoimmune disease with autologous bone marrow transplantation.
(14/962)
The results of experiments with the induced autoimmune diseases adjuvant arthritis and allergic encephalomyelitis in rats, which led to the discovery of the curative effect of autologous bone marrow transplantation following high-dose myeloablative treatment, are reviewed. The rationale is eradication of the autoreactive lymphocytes and memory cells, and the prevention of relapse due to transfer of lymphocytes with the autograft. Comparison of various conditioning regimens in the animal models indicates that a combination conditioning with low-dose total body irradiation (TBI) and high-dose cyclophosphamide is optimal. These findings were the basis for the conditioning currently employed in the treatment of polyarticular juvenile chronic arthritis (JCA) by the teams in Utrecht and Leiden, which consists of cyclophosphamide 50 mg/kg for 4 days, 4 Gy TBI and anti-thymocyte globulin (ATG). The use of TBI for the treatment of non-malignant disease is regarded as undesirable by many physicians in view of the risks, in particular, of growth inhibition in children and the induction of tumours. Experimental and clinical data show that a dose of 4 Gy does not cause significant inhibition of skeletal growth in infants. The risk of excess cancer due to TBI has been well established in quantitative terms and is compared with the expected risk of high-dose cyclophosphamide and the risk associated with the highly immunosuppressive regimens currently used for the treatment of JCA. (+info)
Autologous stem cell transplantation: a possible treatment for refractory juvenile chronic arthritis?
(15/962)
OBJECTIVE: In adults, autologous stem cell transplantation (ASCT) has been described recently as a possible treatment for severe autoimmune disease refractory to conventional treatment. We report here the four first children with severe forms of juvenile chronic arthritis (JCA) treated with ASCT. METHODS: We studied three children with systemic JCA and one child with polyarticular JCA. Unprimed bone marrow was harvested 1 month prior to ASCT. T-cell depletion of the graft was performed with CD2 and CD3 antibodies. We used a preparative regimen of antithymocyte globulin (ATG; 20 mg/kg), cyclophosphamide (Cy; 200 mg/kg) and low-dose total body irradiation (TBI; 4 Gy). Methotrexate (MTX) and cyclosporin A (CsA) were stopped before ASCT; prednisone was tapered after 2 months. RESULTS: After ASCT, our patients showed an anti-inflammatory-drug-free follow-up of 6-18 months with a marked decrease in joint swelling, pain and morning stiffness. The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and haemoglobin (Hb) returned to near-normal values within 6 weeks. Despite T-cell depletion, there was a very rapid immune reconstitution. Two patients developed a limited varicella zoster virus (VZV) eruption which was treated by acyclovir. (+info)
Consulting the 'experts': children's and parents' perceptions of psycho-educational interventions in the context of juvenile chronic arthritis.
(16/962)
There is little documented information regarding current provision of psycho-educational interventions in the context of juvenile chronic arthritis (JCA). The purpose of the present exploratory study was to gain insight and understanding of the needs and preferences of children with JCA and their parents, and to examine how these may be more adequately addressed by future psycho-educational interventions. Data were collected through focus group discussions with children, parents and health professionals, respectively. Results showed that the voices of children and their parents were as one in calling for greater availability, easier access and more comprehensive psycho-educational interventions in the context of JCA. Greater emphasis is needed upon the social and emotional aspects of chronic disease, and enhancing the self-management skills of children and their families. Moreover, content should be tailored to match broad categories of disease severity (e.g. mild or severe), developmental age and stage in the life course (e.g. child or adolescent). Group education, informal support groups, activity weekends and summer camps would be particularly welcomed by parents and children, and may serve to reduce their sense of isolation whilst providing much needed reassurance. Findings provide a useful set of signposts to guide future development of psycho-educational interventions in the field of psychosocial paediatric rheumatology. (+info)