ABSTRACT
BACKGROUND:In recent years, the application of stem cel s to treat autoimmune diseases has become a hot spot. But, studies on umbilical cord blood mesenchymal stem cel s transplantation for the treatment of polymyositis/dermatomyositis are rarely reported. OBJECTIVE:To explore the immunologic mechanism of Th cytokines on the occurrence and development of polymyositis/dermatomyositis by observing the changes in serum interferon-γ, interleukin-4 and interleukin-17 in patients after umbilical cord blood mesenchymal stem cel s transplantation. METHODS:Eighty-one polymyositis/dermatomyositis patients were selected and divided into conventional therapy group (n=44) undergoing glucocorticoid and immunosuppressants therapy and cel transplantation group (n=37) undergoing intravenous infusion of umbilical cord blood mesenchymal stem cel s at a density of (3.5-5.2 )×107 . Dosing regimen was same in the two groups. After fol ow-up of 1, 3, 6 months, the changes of creatine kinase and myodynamia were evaluated;after fol ow-up of 3 and 6 months, lung imaging was evaluated;in the cel transplantation group, interferon-γ, interleukin-4 and interleukin-17 levels were detected before treatment and at 3 and 6 months after treatment. RESULTS AND CONCLUSION:At 1, 3, 6 months after treatment, the creatine kinase level was significantly decreased, and the muscle force grade was significantly increased in both groups (both P0.05). These findings indicate umbilical cord blood mesenchymal stem cel s transplantation combined with glucocorticoid and immunosuppressants therapy can adjust immune network effects and improve the immune tolerance in polymyositis/dermatomyositis patients, which is safe and effective.
ABSTRACT
Objective We reported 16 eosinophilic fasciitis (EF) patients with eosinophilic fasciitis and performed a systematic review of the literature to improve the disease awareness.Methods The clinical course of 16 patients with eosinophilic fasciitis at the Peking Union Medical College Hospital were described,inclu-ding demographic data,clinical manifest-ations,laboratory tests,pathology and treatment.Results The mean age at diagnosis was (47±8) years,with 13 female and 3 male patients.Three cases had exertion or strenuous sports before the onset of EF.Positive ANA was noted in 6 of 12,positive RF was noted in 3 of 10,hyper-gammaglobulinemia was noted in 6 of 7,elevated IgG was noted in 8 of 13,peripheral blood eosinophilia was noted in 10 of 16,while thrombocytopenia was found in one patient.Conclusion Based on this and other reported cases in the literature,EF may be a kind of autoimmune disease.Genetic influence and environ-mental factors are involved in the development of this disease.Systemic involvement is rare.In general,corticosteroids and immunosuppressive are effective in EF.
ABSTRACT
Objective To analyze the clinical data of diagnosis and treatment of Wegener' s granulomatosis (WG) in order to understand the nature of this disease. Methods The clinical data including clinical manifestations,laboratory findings imaging features,pathological changes and efficacy of treatment of 34 patients with WG admitted from January 2002 to March 2012 were analyzed.Results Of the 34 patients,male to female ratio was 18 to 16,and the average age subjected to WG onset was (45 ± 15)years old ranged from 18 to 81 years old.The average duration before the diagnosis confirmed was ( 140 ±72) mouths,while 29.4％ was diagnosed within 3 months. The presenting symptoms included initial involvements in lung (41.1％),nose (38.2％ ),eyes ( 11.7％ ),ear (8.8％ ) and constitutional symptoms such as fever (26.4％ ). Throughout the whole disease course,the incidence of systemic impairments were as follows:lung (76.4％),nose (73.5％),kidney (67.6),eyes (58.5％),ear (47.0％),nervous system (41.1％),oral ulcer (20.5％ ).Of laboratory findings,C - ANCA/PR3 -ANCA was positive in 61.7％ patients,P - ANCA/MPO - ANCA positive in 20.5％ patients,ANCA negative in 11.7％ patients,P- ANCA positive with MPO- ANCA negative in one patient (2.9 ％ ) and C -ANCA positive with PR3 and MPO positive in one patient (2.9％ ).Of imaging findings,nodules or masses were most commonly observed (34％), followed by fibrotic lesions (20.5％ ), cavitations ( 17.6％ )、opacities ( 17.6％ ).Typically pathological triad ( vasculitis,necross and granuloma formation)was found in one patient ( 4.3％ ),while two pathological changes of the triad occurred in 10 patients (43.4％). Pulmonary infection was highly prevalent (50％), and of them,20.5％ patients were misdiagnosed as tuberculosis,malignancy or abscess.After admission,high dose of corticosteroids and cyclophosphamide were administered as bolus and maintenance therapy. Plasma exchange,intravenous immunoglobulin,and rituximab were added for refractory cases and optimal response were obtained in most cases.Conclusions Wegeners granulomatosis is a clinically complicated entity and sometimes can have progression,leading to misdiagnosis. Early and prompt use of steroids and cyclophosphamide may confer good therapeutic efficacy and avoid life -threatening complications.