A 22-year-old woman presented with fever and pancytopenia. One year ago, she was diagnosed as Salmonella group D bacteremia and myelofibrosis associated with SLE at another hospital. She was placed on high dose steroid, however, there was no improvement. Two months ago, she was diagnosed as recurrent Salmonella group D bacteremia. After admission to our hospital, she was placed on intravenous antibiotics and high dose intravenous immunoglobulin. A significant improvement in laboratory and clinical condition occurred and bone marrow biopsy showed complete resolution of fibrosis. We report a case of SLE with myelofibrosis and recurrent Salmonella group D bacteremia.
Anti-Bacterial Agents ; Bacteremia* ; Biopsy ; Bone Marrow ; Female ; Fever ; Fibrosis ; Humans ; Immunoglobulins* ; Lupus Erythematosus, Systemic* ; Pancytopenia ; Primary Myelofibrosis* ; Salmonella Infections ; Salmonella* ; Young Adult

Low dose methotrexate (MTX) is widely used for treatment of rheumatoid arthritis (RA) due to prompt clinical response, relatively lack of serious side effects, tolerability and simplicity of medication. However, several serious adverse effects have been reported with the use of MTX. The prevalence of hematologic toxicity, including leukopenia, thrombocytopenia, megaloblastic anemia, and pancytopenia, is estimated to be 3% in MTX-treated RA patients. Pancytopenia, which occurs unpredictably, is one of the most serious adverse effects and the prevalence is estimated to be 1.4% and fatal pancytopenia reported to occur in 17% of these patients. Old age, impaired renal function, concurrent infection, hypoalbuminemia, increased mean corpuscular volume of red blood cell, concomitant medication such as trimethoprim-sulfamethoxazole and nonsteroidal antiinflammatory drug, are the risk factors of MTX induced pancytopenia. We experienced a case of fatal MTX induced early aplastic anemia in RA patients who medicated 3 times MTX (7.5mg/weekly, 22.5mg of cumulative dose) refractory to treatment with steroid pulse, recombinant human granulocyte macrophage colony stimulating factor (rhGM-CSF) and immunoglobulin.
Anemia, Aplastic* ; Anemia, Megaloblastic ; Arthritis, Rheumatoid* ; Erythrocyte Indices ; Erythrocytes ; Granulocyte-Macrophage Colony-Stimulating Factor ; Humans ; Hypoalbuminemia ; Immunoglobulins ; Leukopenia ; Methotrexate* ; Pancytopenia ; Prevalence ; Risk Factors ; Thrombocytopenia ; Trimethoprim, Sulfamethoxazole Drug Combination

Antineutrophil cytoplasmic antibodies (ANCAs) are now regarded as a serologic marker for pauci-immune crescentic necrotizing glomerulonephritis either in renal-limited form or in association with systemic vasculitis, such as Wegener? granulomatosis, microscopic polyarteritis, and Churg-Strauss syndrome. Two major ANCA antigens have been indentified: proteinase3, which produces a cytoplasmic staining pattern termed C-ANCA, and myeloperoxidase, which produces a perinuclear pattern termed P-ANCA on ethanol-fixed neutrophils by indirect immunofluorescence. In ANCA- associated diseases, eosinphilia in excess of 1.5X109/L has been proposed to be characteristic of Churg-Strauss syndrome and is rare in other forms of ANCA-associated systemic vasculitis and crescentic necrotizing glomerulonephritis. Recently, there were two cases of P-ANCA positive crescentic necrotizing glomerulonephritis with peripheral blood eosinophilia and extrarenal microscopic vasculitis without asthma or granulomas. We experienced a patient with P-ANCA positive pauci-immune necrotizing glomerulonephritis with few eosinophilic infiltration and eosinophilia. He improved with oral prednisolone along with combination of intravenous cyclophosphamide. So we report this case with the review of literature.
Antibodies, Antineutrophil Cytoplasmic* ; Asthma ; Churg-Strauss Syndrome ; Cyclophosphamide ; Cytoplasm ; Eosinophilia* ; Eosinophils ; Fluorescent Antibody Technique, Indirect ; Glomerulonephritis* ; Granuloma ; Humans ; Neutrophils ; Peroxidase ; Prednisolone ; Systemic Vasculitis ; Vasculitis

Systemic lupus erythematosus (SLE) is a disease of unknown etiology in which tissues and cells are damaged by pathogenic autoantibodies and immune complexes. Skin manifestations of SLE include malar rash, discoid rash, photosensitivity, oral ulcer, panniculitis, urticaria, bullae, erythema multiforme and lichen planus-like lesions. It has long been recognized that dystrophic soft tissue calcification may occur in association with certain connective tissue disorders such as scleroderma or dermatomyositis. Soft tissue calcification in a patient with SLE has been rarely reported. We have experienced a patient with SLE who presented with diffuse subcutaneous tissue calcification on face, chest, abdomen and all extremities.
Abdomen ; Antigen-Antibody Complex ; Autoantibodies ; Connective Tissue ; Dermatomyositis ; Erythema Multiforme ; Exanthema ; Extremities ; Humans ; Lichens ; Lupus Erythematosus, Systemic* ; Oral Ulcer ; Panniculitis ; Skin Manifestations ; Subcutaneous Tissue* ; Thorax ; Urticaria

Giant cell arteritis is an inflammatory disease that mainly involves the cranial branches of the arteries originating from the aortic arch. It affects the white populations over the age of 50 almost exclusively who live in Northern Europe and in the United states. There is close relationship between giant cell arteritis and polymyalgia rheumatica. So far, there have been a few reports of temporal arteritis in Korea. However, giant cell arteritis with typical pathological findings in temporal artery has not been reported. We describe a case who showed typical pathological findings of giant cell arteritis in superficial temporal artery. She had been diagnosed as polymyalgia rheumatica in several months ago.
Aorta, Thoracic ; Arteries ; Europe ; Giant Cell Arteritis* ; Giant Cells* ; Korea ; Polymyalgia Rheumatica* ; Temporal Arteries ; United States

Stevens-Johnson syndrome is an acute devastating condition which is related to certain drugs or infections. This syndrome involves multiple organs such as mucocutaneous, respiratory, occular and gastrointestinal systems. Patients who have underlying immunologic diseases are thought to be more susceptible. In lots of case reports and studies, more than 100 drugs have been implicated as causes of Stevens-Johnson syndrome or toxic epidermal necrolysis, but not so much as in Chinese Herb medications. Recently, we have experienced a patient with Stevens-Johnson syndrome after taking herb medicine and diagnosed as SLE during evaluation and treatment. We report this patient with a brief review of literatures.
Asian Continental Ancestry Group ; Humans ; Immune System Diseases ; Lupus Erythematosus, Systemic* ; Stevens-Johnson Syndrome*

No abstract available.
Arthritis, Rheumatoid* ; Humans

No abstract available.

Plasmacytomas are tumors composed of plasma cells of variable maturity, which are histologically identical to those seen in multiple myeloma. Ankylosing spondylitis is a chronic inflammatory disease, probably resulting from the interaction of a genetic predisposition involving HLA-B27 with an environmental event such as enteric bacterial infection. Multiple myeloma has been intermittently reported in patients with ankylosing spondylitis. It has been proposed that the protracted stimulation of immunocytes by inflammatory lesions on the mucosal surfaces of the gastrointestinal, respiratory tracts may be implicated in the pathogenesis of multiple myeloma in some patients. We observed a 23 year old male patient with a history of plasmacytoma who subsequently developed ankylosing spondylitis. He was diagnosed as plasmacytoma 4 years ago and took a radiation therapy. There was no previous report of ankylosing spondylitis following plasmacytoma. The relationship between two diseases is uncertain until now and further study should be needed.
Bacterial Infections ; Genetic Predisposition to Disease ; HLA-B27 Antigen ; Humans ; Male ; Multiple Myeloma ; Plasma Cells ; Plasmacytoma* ; Respiratory System ; Spondylitis, Ankylosing* ; Young Adult

Adult onset Still's disease (AOSD) is a multi-systemic inflammatory disorder characterized by several distinguished manifestations including high spiking fever, evanescent salmon-colored skin rash, arthralgia/arthritis, hepato-splenomegaly, lymphadenopathy, sore throat, serositis, and leukocytosis. The frequently noticed cardiopulmonary manifestation is pleuritis, pneumonitis, and pericarditis. Diffuse myocardial dysfunction is uncommon in AOSD, but it may be the cause of life-threatening heart failure. We have experienced a case of AOSD with acute heart failure in 20-year-old female complained of high fever and skin rash. On echocardiogram, the wall motion of left ventricle was globally decreased with a marked diminished ejection fraction (<25%). Two weeks after treatment with high dose steroid and intravenous immunoglobulin, her symptoms and cardiac function on echocardiogram was completely resolved. To our knowledge, this is the first case of AOSD with acute heart failure reported in Korea.
Estrogens, Conjugated (USP)* ; Exanthema ; Female ; Fever ; Heart Failure* ; Heart Ventricles ; Humans ; Immunoglobulins ; Korea ; Leukocytosis ; Lymphatic Diseases ; Pericarditis ; Pharyngitis ; Pleurisy ; Pneumonia ; Serositis ; Still's Disease, Adult-Onset* ; Young Adult