OBJECTIVE: We investigated the long-term outcomes of autologous peripheral blood stem cell transplantation (PBSCT) to treat refractory rheumatic diseases. METHODS: Patients who underwent PBSCT for refractory rheumatic diseases at our institution between 2002 and 2005 were assessed for outcomes including treatment response, adverse events, damage accrual, and survival at 6 months and last follow-up. RESULTS: Eleven patients, including six with systemic lupus erythematosus (SLE), four with systemic sclerosis (SSc), and one with Still's disease were treated with PBSCT. In SLE patients, two showed complete response, two partial response, and two expired. One patient who expired responded completely two months after transplantation but discontinued treatment by choice and expired at six months due to an SLE flare. Long-term, two patients went into remission without organ damage, one patient went into remission with organ damage, and one had low disease activity with organ damage. Of the four patients with SSc, two showed a complete response, one a partial response, and there was one transplantation-related death at six months. At the last record notation, two remained in remission without relapse and one was lost to follow-up. The Still's disease patient partially responded at six months and was in remission at the last record notation. CONCLUSION: The ten-year survival rate was 70% with a 40% recurrence rate and 20% treatment-related mortality rate.
Follow-Up Studies ; Humans ; Lost to Follow-Up ; Lupus Erythematosus, Systemic ; Mortality ; Peripheral Blood Stem Cell Transplantation* ; Recurrence ; Rheumatic Diseases* ; Scleroderma, Systemic ; Survival Rate

No abstract available.
Chondromatosis, Synovial* ; Knee Joint* ; Knee* ; Osteoarthritis*

No abstract available.

Intraaortic balloon counterpulsation (IAB) has been shown to prolong survival in the critically ill cardiac patients. Originally developed for use in the patients with cardiogenic shock, the indications have been expanded. But despite technical advances, the complication rate associated with IAB remains high. The most commonly reported complications include damage to the femoral artery and distal embolization. Other reported major complications are balloon rupture, limb loss, bleeding, systemic infection and bowel infarction. We report a patient complicated by ischemic colitis causing stenosis and intestinal obstruction after IAB insertion.
Colitis, Ischemic* ; Constriction, Pathologic* ; Counterpulsation* ; Critical Illness ; Extremities ; Femoral Artery ; Hemorrhage ; Humans ; Infarction ; Intestinal Obstruction ; Rupture ; Shock, Cardiogenic

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is a group of systemic necrotising vasculitides, which often involve small vessels, and which lead to few or no immune deposits in affected organs. According to clinical manifestations and pathological features, AAV is classified into three variants: microscopic polyangiitis, granulomatosis with polyangiitis (GPA), and eosinophilic GPA. The American College of Rheumatology 1990 criteria contributed to the classification of AAV, although currently the algorithm suggested by the European Medicines Agency in 2007 and the Chapel Hill Consensus Conference Nomenclature of Vasculitides proposed in 2012 have encouraged physicians to classify AAV patients properly. So far, there have been noticeable advancements in studies on the pathophysiology of AAV and the classification criteria for AAV in Western countries. However, studies analysing clinical features of Korean patients with AAV have only been conducted and reported since 2000. One year-, 5 year-, and 10 year-cumulative patient survival rates are reported as 96.1, 94.8, and 92.8%. Furthermore, initial vasculitis activity, prognostic factor score, age and specific organ-involvement have been found to be associated with either all-cause mortality or poor disease course. The rate of serious infection is 28.6%, and 1 year-, 5 year- and 10 year-cumulative hospitalised infection free survival rates range from 85.1% to 72.7%. The overall standardised incidence ratio of cancer in AAV patients was deemed 1.43 compared to the general Korean population.
Antibodies, Antineutrophil Cytoplasmic ; Classification ; Consensus ; Cytoplasm* ; Eosinophils ; Granulomatosis with Polyangiitis ; Humans ; Incidence ; Korea* ; Microscopic Polyangiitis ; Mortality ; Rheumatology ; Survival Rate ; Vasculitis*

Eosinophilic granulomatosis with polyangiitis (EGPA) is one form of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Identical to what has been called Churg-Strauss syndrome, EGPA exhibits both allergic and vasculitis features. EGPA was first described as a syndrome consisting of asthma, fever, eosinophilia, and organ involvement including heart failure, neuropathy, and kidney damage, by Churg and Strauss in 1951. On the basis of the 2012 Chapel Hill Consensus Conferences Nomenclature of Vasculitis, EGPA comprises three typical allergic components, including asthma, peripheral eosinophilia, and eosinophil-rich granuloma of the respiratory tracts. EGPA has three clinical and histological stages. The first is an allergic stage composed of asthma and sinusitis, and the second is an eosinophilic stage characterised by peripheral hypereosinophilia and intra-organ infiltration of eosinophils. The last is a vasculitic stage, including necrotising inflammation of small vessels and end-organ damage. In this review, we describe the classification criteria for EGPA and recommendations for the evaluation and management of EGPA with conventional and newly suggested drugs for EGPA. Also, we discuss a variety of clinical aspects such as predictive values for prognosis and associations with other Th2-mediated diseases and hepatitis B virus.
Antibodies, Antineutrophil Cytoplasmic ; Asthma ; Churg-Strauss Syndrome ; Classification ; Congresses as Topic ; Consensus ; Eosinophilia ; Eosinophils ; Fever ; Granuloma ; Granulomatosis with Polyangiitis ; Heart Failure ; Hepatitis B virus ; Humans ; Inflammation ; Kidney ; Prognosis ; Respiratory System ; Sinusitis ; Vasculitis

BACKGROUND/AIMS: SKI306X, a mixed extract of three herbs, Clematis mandshurica (CM), Prunella vulgaris (PV), and Trichosanthes kirilowii (TK), is chondroprotective in animal models of osteoarthritis (OA). The objectives of this study were to investigate its effect on interleukin (IL)-1beta-induced degradation of glycosaminoglycan (GAG) and the basis of its action in human OA cartilage, as well as to screen for the presence of inhibitors of matrix metalloproteinase (MMP)-13 and a disintegrin and metalloprotease with thrombospondin motifs (ADAMTS)-4 in SKI306X and its component herbs, as well as in fractions from SKI306X. METHODS: Human OA chondrocytes and cartilage explants were obtained during total knee replacements and incubated with IL-1beta +/- oncostatin M with or without SKI306X or its component herb extracts. GAG degradation was assayed in cartilage explants using a commercial kit. Expression of genes involved in cartilage destruction was measured by real-time polymerase chain reaction using chondrocyte RNA. SKI306X was fractionated by preparative liquid chromatography to test for the presence of inhibitors of MMP-13 and ADAMTS-4. RESULTS: SKI306X and PV inhibited IL-1beta-induced GAG release from cartilage explants, and SKI306X, CM, PV, and TK inhibited IL-1beta-induced MMP gene expression. Unexpectedly, SKI306X greatly stimulated IL-1beta + oncostatin M-induced ADAMTS-4 gene expression, probably due to its TK component. Some fractions of SKI306X also inhibited ADAMTS-4 activity. CONCLUSIONS: SKI306X and its herbal components inhibit GAG degradation and catabolic gene expression in human OA chondrocytes and cartilage explants. SKI306X likely also contains one or more ADAMTS-4 inhibitor.
ADAM Proteins/antagonists & inhibitors ; Cartilage, Articular/*drug effects/*metabolism ; Cells, Cultured ; Chondrocytes/drug effects/metabolism ; Down-Regulation/drug effects ; Drugs, Chinese Herbal/*pharmacology ; Glycosaminoglycans/*metabolism ; Humans ; Interleukin-1beta/metabolism ; Matrix Metalloproteinase 13/metabolism ; Matrix Metalloproteinase Inhibitors/pharmacology ; Oncostatin M/metabolism ; Osteoarthritis, Knee/drug therapy/genetics/metabolism ; Procollagen N-Endopeptidase/antagonists & inhibitors

Posttransplant lymphoproliferative disorder (PTLD) are among the most serious and potentially fatal complications of chronic immunosuppression in organ transplant recipient and also the most common malignancies, accounting for 21 percent of all malignancies in organ transplants versus 5 percent of malignancies in the general population. PTLD is associated with immunosuppression and Epstein Barr virus (EBV). Treatment modality of PTLD includes antiviral agent, interferon, intensive chemotherapy and monoclonal antibody. Choice of treatment modality depends on clinical presentation of PTLD. We report here a case of PTLD involving liver and renal allograft treated with rituximab.
Allografts ; Drug Therapy ; Herpesvirus 4, Human ; Immunosuppression ; Interferons ; Kidney Transplantation ; Liver ; Lymphoproliferative Disorders ; Transplantation* ; Transplants ; Rituximab

OBJECTIVE: Ovarian hyperstimulation syndrome (OHSS) is one of the well known complication of conttolled ovarian hyperstimulation. Though there have been numerous protocols for the prevention of OHSS, it has not been completely preventable until now. This study was performed to identify clinical predictors for early and late OHSS. METHODS: A retrospective analysis of all IVF cycles in 1993 up to June 1996 was performed. OHSS was diagnosed using the criteria of Rabau modified by Schenker. All cases of OHSS reported in this study presented with marked ovarian enlargement, ascites, oliguria, hemoconcentration and electrolyte disturbance. Ovarian stimulation was carried out using a combination of gonadotrophin releasing hormone-agonist, follicle-stimulation hormone and human menopausal gonadotrophin. 27 patients has moderate or severe OHSS presenting 3-7 days post-human chorionic gonadotrophin (hCG), and 21 patients had severe OHSS presenting 12-17 days post-hCG. RESULTS: No patient with early OHSS went onto develop late OHSS, and no patient with late OHSS had demonstrated early OHSS. Logistic regression showed that early OHSS was predicted by the number of oocytes retrieved and the estradiol concentration on the day hCG injection (P<0.05). Late OHSS was predicted by the transferred embryos, B-hCG on 14 day after hCG injection (P<0.05). CONCLUSION: Early OHSS was an acute effect of the hCG administered prior to egg retrieval in women with high estradiol and large number of retrieved oocytes. Our analysis of the risk factors for early OHSS indicates that cryopreservation of all embryos will not alter the risk of early OHSS even though it should prevent late OHSS. Late OHSS was induced by the rising serum concentration of hCG produced by the early pregnancy, the number of transferred embryos must be adjusted carefully, since it was associated with multiple gestation.
Ascites ; Chorion ; Cryopreservation ; Embryonic Structures ; Estradiol ; Female ; Humans ; Logistic Models ; Oliguria ; Oocytes ; Ovarian Hyperstimulation Syndrome ; Ovulation Induction ; Ovum ; Pregnancy ; Retrospective Studies ; Risk Factors