Autoimmune hepatitis (AIH) is an unresolving, predominantly periportal hepatitis that is usually displays hypergammaglobulinemia, and tissue autoantibodies, and this malady is responsive to immunosuppressive therapy. Our understanding about this clinical entity has been greatly expanded since the first description by Waldenstrom 50 years ago. The codified diagnostic criteria of AIH prepared by International Autoimmune Hepatitis Group are still valid, but new attempts are being made to overcome the shortcomings of this scoring system. Immunosuppressive therapies using prednisone and azathioprine are currently the mainstay for the treatment of AIH, but there are still many practical questions to be solved.
Adolescent ; Adult ; Aged ; Autoantibodies ; Azathioprine/administration & dosage/*therapeutic use ; Child ; Child, Preschool ; Drug Therapy, Combination ; Female ; Hepatitis, Autoimmune/*diagnosis/*drug therapy/etiology ; Humans ; Immunosuppressive Agents/administration & dosage/*therapeutic use ; Male ; Middle Aged ; Prednisone/administration & dosage/*therapeutic use

BACKGROUNDAn increasing incidence of Crohn's disease has been found in China in recent years. Our study has been focused on evaluating the diversity of the clinical manifestations of Crohn's disease in order to improve early diagnostic accuracy and therapeutic efficacy.

METHODSThirty patients with active Crohn's disease were enrolled and their clinical data, including diagnostic and therapeutic results, were analyzed. Endoscopy combined with histological examination of biopsy specimens provided characteristic features of the disease. Transabdominal bowel sonography (TABS) was used for detecting intestinal complications. Nutritional supportive therapy was given to 20 subjects with active cases of the disease.

RESULTSMost patients were young adults with a higher proportion of females to males (ratio: 1.14:1). The disease affects any segment or a combination of segments along with the alimentary tract (from the mouth to the anus). In this study, the colon and small bowel were the major sites involved. Recurrent episodes of abdominal pain in the right lower quadrant and watery diarrhea were the most common symptoms. Granulomas were identifiable in nearly one-third (30.8%) of all biopsy specimens. In moderate cases of the disease, remission was achieved more quickly through the use of oral prednisone therapy than with SASP or 5-ASA. Beneficial effects on the host's nutritional status were observed. Immunosuppressives were used on an individual basis and showed variable therapeutic effects. Sixteen patients had surgery due to intestinal obstruction or failure to respond to drug therapies. Rapid improvement after surgery was reported.

CONCLUSIONEndoscopy (with biopsy) and TABS were both crucial procedures for diagnosis. SASP (or 5-ASA) and prednisone were effective as inductive therapies. Azathioprine has demonstrable benefits after induction therapy with prednisone. Surgery, as an alternative treatment, provided another effective choice in selected patients.

Adolescent ; Adult ; Aged ; Azathioprine ; therapeutic use ; Biopsy ; Child ; Crohn Disease ; diagnosis ; diagnostic imaging ; therapy ; Endoscopy, Gastrointestinal ; Female ; Humans ; Male ; Mesalamine ; administration & dosage ; Middle Aged ; Prednisone ; administration & dosage ; Sulfasalazine ; administration & dosage ; Ultrasonography

OBJECTIVETo explore methods of preventing and reversing rejection after simultaneous pancreas-kidney transplantation (SPK).

METHODSSeventeen patients performed SPK operation from Sep, 1999 to Sep, 2003 were reviewed retrospectively. Immunosuppression was achieved by triple regimen consisting of cyclosporine, mycophenolate mofetil (MMF)/azathioprine and steroid. 2 patients were treated with Dalizumab, the other three patients used OKT3 as immune induction.

RESULTS1 patient experienced the accelerated rejection, the pancreas and kidney grafts were resected because of failure of conservative therapy. 8 patients experienced renal acute rejection, 2 cases suffered from pancreas acute rejection at the same time. All these patients received daily high dose pulse steroid for 3 days. OKT3 was administered in 2 patients with steroid resistance rejection. All the grafts were successfully rescued.

CONCLUSIONSReasonable application of immunosuppression after SPK operation and adoption of systemic measures which can reduce sensitivity of high risk receptor before SPK operation are the effective methods of preventing and treating rejection.

Administration, Oral ; Adult ; Azathioprine ; administration & dosage ; Cyclosporine ; administration & dosage ; Diabetic Nephropathies ; surgery ; Drug Therapy, Combination ; Female ; Glucocorticoids ; administration & dosage ; Graft Rejection ; prevention & control ; Humans ; Immunosuppressive Agents ; administration & dosage ; Kidney Transplantation ; immunology ; Male ; Middle Aged ; Pancreas Transplantation ; immunology ; Prednisolone ; administration & dosage ; Retrospective Studies ; Transplantation, Homologous

We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.
Autoimmune Diseases/diagnosis/drug therapy ; Azathioprine/administration & dosage/*therapeutic use ; Brain/blood supply/pathology ; Female ; Hearing Loss ; Humans ; Immunotherapy ; Magnetic Resonance Imaging ; Republic of Korea ; Retinal Artery Occlusion/diagnosis/drug therapy/pathology ; Susac Syndrome/*diagnosis/*drug therapy/pathology ; Young Adult

Pemphigus vulgaris is autoimmune disease characterized by acantholysis and blister formation in the skin and mucosa. The introduction of corticosteroids has greatly reduced mortality, and a number of immunosuppressive agents, such as azathioprine and cyclophosphamide are recommended as adjuncts to oral steroids. A 38-year-old female patient of pemphigus vulgaris has been managed with daily oral cyclophosphamide as adjunct to prednisolone for about 11 months, and then transfered to our department due to amenorrhea for 4 months. Hormonal profile of FSH, LH and estradiol revealed in postmenopausal range and follow-up results of hormonal profile were not changed, and amenorrhea continues for total 13 months until now. So we report a case of premature ovarian failure complicated by long-term oral administration of cyclophosphamide.
Acantholysis ; Administration, Oral ; Adrenal Cortex Hormones ; Adult ; Amenorrhea ; Autoimmune Diseases ; Azathioprine ; Blister ; Cyclophosphamide* ; Estradiol ; Female ; Follow-Up Studies ; Humans ; Immunosuppressive Agents ; Mortality ; Mucous Membrane ; Pemphigus* ; Prednisolone ; Primary Ovarian Insufficiency* ; Skin ; Steroids

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
Azathioprine/administration +ACY- dosage ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/pathology ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis+ACo- ; Case Report ; Creatine Kinase/blood+ACo- ; Dermatomyositis/pathology ; Dermatomyositis/enzymology ; Dermatomyositis/drug therapy ; Dermatomyositis/diagnosis+ACo- ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisone/administration +ACY- dosage ; Tomography, X-Ray Computed

Ulcerative colitis (UC) is a chronic inflammatory bowel disorder characterized by a relapsing and remitting course. The quality of life can decreases significantly during exacerbations of the disease. The incidence and prevalence of UC in Korea are still lower than those of Western countries, but have been rapidly increasing during the past decades. Various medical and surgical therapies are currently used for the management of UC. However, many challenging issues exist and sometimes these lead to differences in practice between clinicians. Therefore, Inflammatory Bowel Diseases (IBD) Study Group of Korean Association for the Study of Intestinal Diseases (KASID) set out the Korean guidelines for the management of UC. These guidelines are made by the adaptation using several foreign guidelines and encompass treatment of active colitis, maintenance of remission and indication for surgery in UC. The specific recommendations are presented with the quality of evidence. These are the first Korean treatment guidelines for UC and will be revised with new evidences on treatment of UC.
Administration, Oral ; Adrenal Cortex Hormones/therapeutic use ; Anti-Inflammatory Agents/therapeutic use ; Antibodies, Monoclonal/therapeutic use ; Antimetabolites, Antineoplastic/therapeutic use ; Azathioprine/therapeutic use ; Colitis, Ulcerative/*drug therapy/surgery ; Humans ; Hydrocortisone/therapeutic use ; Injections, Intravenous ; Mesalamine/therapeutic use ; Methylprednisolone/therapeutic use ; Severity of Illness Index ; Sulfasalazine/therapeutic use

INTRODUCTIONAtopic dermatitis is a common, chronic pruritic condition affecting both children and adults, which has a negative impact on the quality of life. These guidelines were developed by an expert workgroup appointed by the Dermatological Society of Singapore, to provide doctors with information to assist in the management of their patients with atopic dermatitis. The workgroup members are experienced dermatologists with interest and expertise in eczemas.

MATERIALS AND METHODSWorkgroup members arrived at a consensus on the topics to be included. Relevant studies from the literature were assessed for best evidence, supplemented by the collective experience of the workgroup.

RESULTSFor mild atopic dermatitis, emollients, mild potency topical steroids and topical calcineurin inhibitors are recommended. For moderate-to-severe atopic dermatitis, the use of emollients, moderate-to-potent topical steroids, topical calcineurin inhibitors, wet dressings, antimicrobials for secondary skin infection, phototherapy, and systemic therapy (e.g. prednisolone, cyclosporine, azathioprine or methotrexate) may be warranted. Patients with moderate-to-severe atopic dermatitis should be managed in conjunction with a dermatologist.

CONCLUSIONGood outcomes can be achieved with an individualised therapeutic approach combined with adequate patient and parental education.

Administration, Cutaneous ; Adrenal Cortex Hormones ; therapeutic use ; Anti-Bacterial Agents ; therapeutic use ; Azathioprine ; therapeutic use ; Calcineurin Inhibitors ; therapeutic use ; Coinfection ; complications ; drug therapy ; Cyclosporine ; therapeutic use ; Dermatitis, Atopic ; complications ; immunology ; therapy ; Dermatology ; Disease Management ; Emollients ; therapeutic use ; Food Hypersensitivity ; immunology ; Humans ; Immunosuppressive Agents ; therapeutic use ; Methotrexate ; therapeutic use ; Patient Education as Topic ; Phototherapy ; Practice Guidelines as Topic ; Referral and Consultation ; Severity of Illness Index ; Singapore

Administration, Cutaneous ; Adrenal Cortex Hormones ; therapeutic use ; Azathioprine ; therapeutic use ; Calcineurin Inhibitors ; therapeutic use ; Cyclosporine ; therapeutic use ; Dermatitis, Atopic ; immunology ; therapy ; Disease Management ; Food Hypersensitivity ; immunology ; Humans ; Immunosuppressive Agents ; therapeutic use ; Patient Education as Topic ; Phototherapy ; Practice Guidelines as Topic ; Severity of Illness Index ; Singapore