The World Health Organization classifies lupus nephritis as class I to V or VI. However, a few cases of minimal change glomerulopathy have been reported in association with systemic lupus erythematosus (SLE). Mycophenolate mofetil has been shown to be effective for treatment of minimal change disease and lupus nephritis. A 24-year-old woman diagnosed with SLE five years prior to presentation complained of a mild generalized edema. The urinalysis showed microscopic hematuria and proteinuria. The assessed amount of total proteinuria was 1,618 mg/24 hours. A renal biopsy demonstrated diffuse fusion of the foot processes of podocytes on electron microscopy. Mycophenolate mofetil was started in addition to the maintenance medications of prednisolone 10 mg/day and hydroxychloroquine 400 mg/day. After six months of treatment, the microscopic hematuria and proteinuria resolved, and the total urine protein decreased to 100 mg/24 hours.
Antirheumatic Agents/therapeutic use ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hydroxychloroquine/therapeutic use ; Immunosuppressive Agents/*therapeutic use ; Lupus Erythematosus, Systemic/complications/*pathology ; Mycophenolic Acid/*analogs & derivatives/therapeutic use ; Nephrosis, Lipoid/*drug therapy/etiology/pathology ; Prednisone/therapeutic use ; Young Adult

Acquired hemophilia is a rare disorder associated with serious bleeding in nonhemophilic patients. We had a 40-year-old woman who was diagnosed as acquired hemophilia with a factor VIII inhibitor level of 27.5 BU/mL. She was presented with gross hematuria and severe right shoulder pain, and was successfully treated with daily oral cyclophosphamide and prednisone for 2 weeks. After the remission, the doses of prednisone and cyclophosphamide were slowly decreased and she remained in complete remission without further bleeding episodes.
Administration, Oral ; Adult ; Case Report ; Cyclophosphamide/therapeutic use+ACo- ; Female ; Hemophilia A/drug therapy+ACo- ; Human ; Immunosuppressive Agents/therapeutic use+ACo- ; Prednisone/therapeutic use+ACo-

No abstract available.
Angiogenesis Inhibitors/administration & dosage/*therapeutic use ; Antineoplastic Agents, Alkylating/administration & dosage/*therapeutic use ; Humans ; Melphalan/administration & dosage/*therapeutic use ; Multiple Myeloma/*drug therapy ; Prednisone/therapeutic use ; Thalidomide/administration & dosage/*therapeutic use ; Treatment Outcome

Autoimmune hepatitis is characterized by autoantibodies, hypergammaglobulinemia, and interface hepatitis on histological examination. The features lack diagnostic specificity, and other diseases that may resemble autoimmune hepatitis must be excluded. The clinical presentation may be acute, acute severe (fulminant), or asymptomatic; conventional autoantibodies may be absent; centrilobular necrosis and bile duct changes may be present; and the disease may occur after liver transplantation or with features that suggest overlapping disorders. The diagnostic criteria have been codified, and diagnostic scoring systems can support clinical judgment. Nonstandard autoantibodies, including antibodies to actin, α-actinin, soluble liver antigen, perinuclear antineutrophil antigen, asialoglycoprotein receptor, and liver cytosol type 1, are tools that can support the diagnosis, especially in patients with atypical features. Prednisone or prednisolone in combination with azathioprine is the preferred treatment, and strategies using these medications in various doses can ameliorate treatment failure, incomplete response, drug intolerance, and relapse after drug withdrawal. Budesonide, mycophenolate mofetil, and calcineurin inhibitors can be considered in selected patients as frontline or salvage therapies. Molecular (recombinant proteins and monoclonal antibodies), cellular (adoptive transfer and antigenic manipulation), and pharmacological (antioxidants, antifibrotics, and antiapoptotic agents) interventions constitute future directions in management. The evolving knowledge of the pathogenic pathways and the advances in technology promise new management algorithms.
Anti-Inflammatory Agents/*therapeutic use ; Autoantibodies/*blood ; Azathioprine/therapeutic use ; Biomarkers/blood ; Diagnosis, Differential ; Drug Therapy, Combination ; Hepatitis, Autoimmune/*diagnosis/*drug therapy ; Humans ; Immunosuppressive Agents/*therapeutic use ; Prednisolone/therapeutic use ; Prednisone/therapeutic use

Lymphocytic interstitial pneumonitis (LIP) is characterized by diffuse bilateral pulmonary infiltrations in both lower lobes. Pleomorphic lymphohistiocytes including mature lymphocytes, variable admixture of plasma cells and other mononuclear cells infiltrate within the pulmonary interstitium, ranging from widened septa to confluent masses. We report a case of LIP associated with Epstein-Barr virus in a patient with SLE and Sj gren's syndrome. A 50-year-old woman was admitted with insidious onset of progressive dyspnea for 20 days. She suffered from arthritis 10 years earlier without specific diagnosis. A radiography of chest has showed bilateral consolidative infiltrations with pleural effusion in both lower lung fields. Open lung biopsy documented lymphocytic interstitial pneumonitis which expressed Epstein-Barr virus genome in situ hybridization study. Following corticosteroid and cyclophosphamide therapy, clinical symptoms and radiologic infiltrations gradually remitted.
Case Report ; Cyclophosphamide/therapeutic use ; Female ; Glucocorticoids, Synthetic/therapeutic use ; Herpesviridae Infections/*complications ; *Herpesvirus 4, Human ; Human ; Immunosuppressive Agents/therapeutic use ; Lung Diseases, Interstitial/*complications/drug therapy ; Lupus Erythematosus, Systemic/*complications ; Middle Age ; Prednisone/therapeutic use ; Sjogren's Syndrome/*complications

A patient with Behcet's syndrome in whom multiple pulmonary artery aneurysms were completely resolved after a combined treatment with corticosteroid and chlorambucil is reported.
Aneurysm/*drug therapy ; Behcet Syndrome/*drug therapy ; Case Report ; Chlorambucil/*therapeutic use ; Drug Therapy, Combination ; Human ; Male ; Prednisone/*therapeutic use ; *Pulmonary Artery

A patient with Behcet's syndrome in whom multiple pulmonary artery aneurysms were completely resolved after a combined treatment with corticosteroid and chlorambucil is reported.
Aneurysm/*drug therapy ; Behcet Syndrome/*drug therapy ; Case Report ; Chlorambucil/*therapeutic use ; Drug Therapy, Combination ; Human ; Male ; Prednisone/*therapeutic use ; *Pulmonary Artery

Primary CNS lymphoma (PCNSL) is a very uncommon disease in children, and usually treated by chemotherapy, combined with focal or craniospinal radiotherapy (RT). However, adverse effects of RT are a concern. We evaluated the outcomes of childhood PCNSL, treated with systemic and intrathecal chemotherapy, but without RT. For fifteen years, six patients among 175 of non-Hodgkin lymphoma were diagnosed as PCNSL in Seoul National University Children's Hospital and we analyzed their medical records retrospectively. Their male:female ratio was 5:1, and median age was 10.1 yr. The primary sites were the sellar area in three patients, parietal area in one, cerebellum in one, and multiple areas in one. Their pathologic diagnoses were diffuse large B-cell lymphoma in three patients, Burkitt lymphoma in two, and undifferentiated B-cell lymphoma in one. Five were treated with the LMB96 treatment protocol, and one was treated with the CCG-106B protocol. None had RT as a first-line treatment. One patient had a local relapse and received RT and salvage chemotherapy, without success. No patient had treatment-related mortality. Their estimated 5-yr event-free and overall survival rates were both 83.3%. In conclusion, PCNSL is a rare disease in childhood, but successfully treated by chemotherapy without RT.
Adolescent ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Central Nervous System Neoplasms/diagnosis/*drug therapy ; Child ; Child, Preschool ; Cyclophosphamide/therapeutic use ; Cytarabine/therapeutic use ; Disease-Free Survival ; Doxorubicin/therapeutic use ; Etoposide/therapeutic use ; Female ; Humans ; Hydrocortisone/therapeutic use ; Infant ; Leucovorin/therapeutic use ; Lymphoma, Non-Hodgkin/diagnosis/*drug therapy ; Male ; Methotrexate/therapeutic use ; Prednisone/therapeutic use ; Recurrence ; Retrospective Studies ; Tomography, X-Ray Computed ; Treatment Outcome ; Vincristine/therapeutic use

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

A 69-year-old male was diagnosed in February 2004 with stage IV extranodal marginal zone B cell lymphoma involving the mediastinal nodes, lung parenchyma and bone marrow with high LDH. Shortness of breath developed following the 5th course of Rituximab-CHOP chemotherapy (cyclophosphamide, Vincristine, Doxorubicin, Prednisolone). Bronchoscopy guided transbronchial lung biopsy revealed interstitial thickening and type II pneumocyte activation, compatible with interstitial pneumonitis. After treatment with prednisolone a complete resolution of the dyspnea was observed. The patient was well on routine follow-up at the outpatient clinic, with no progression of lymphoma or interstitial pneumonitis.
Aged ; Antibodies, Monoclonal/*adverse effects/*therapeutic use ; Antineoplastic Combined Chemotherapy Protocols/*adverse effects/*therapeutic use ; Biopsy ; Cyclophosphamide/adverse effects/therapeutic use ; Doxorubicin/adverse effects/therapeutic use ; Humans ; Lung Diseases, Interstitial/*chemically induced/*pathology/radiography/surgery ; Lymphoma, B-Cell, Marginal Zone/*drug therapy ; Male ; Prednisone/adverse effects/therapeutic use ; Tomography, X-Ray Computed ; Vincristine/adverse effects/therapeutic use