Systemic lupus erythematosus(SLE) is a prototypic autoimmune disease affecting various organ systems. Hypothermia is a rare manifestation of SLE. We experienced a case of SLE combined with hypothermia. A 36-year-old woman, who had been diagnosed as SLE 3 days before admission, admitted complaining of mental confusion. After admission, her body temperature, initially 36.1 degree C, became 32.6 degree C. Her core body temperature was less than 35.0 degree C. Despite of warming with heating lamp and blankets, her core temperature did not reach 35.0 degree C during 18 hours. Ten days later, her temperature exceeded 36.0 degree C.
Adrenal Cortex Hormones/administration & dosage ; Adult ; Atrial Fibrillation/diagnosis ; Atrial Fibrillation/complications* ; Body Temperature/physiology ; Case Report ; Cyclophosphamide/administration & dosage ; Drug Therapy, Combination ; Electrocardiography ; Female ; Follow-Up Studies ; Human ; Hypothermia/therapy ; Hypothermia/diagnosis ; Hypothermia/complications* ; Lupus Erythematosus, Systemic/drug therapy ; Lupus Erythematosus, Systemic/diagnosis ; Lupus Erythematosus, Systemic/complications*

Both systemic lupus erythematosus and Sjogren's syndrome are autoimmune diseases. Almost all organs can be involved but the gall bladder is an unusual site. We report a 39-year-old woman with systemic lupus erythematosus and Sjogren's syndrome presenting with acute acalculous cholecystitis. It is a very rare complication and, in the literature review, surgical interventions are mostly applied for treatment. In our case, high dose corticosteroid was tried and clinical manifestations and radiologic findings were improved.
Acute Disease ; Adult ; Case Report ; Cholecystitis/*complications/diagnosis/drug therapy ; Female ; Human ; Lupus Erythematosus, Systemic/*complications/diagnosis ; Methylprednisolone/therapeutic use ; Sjogren's Syndrome/*complications/diagnosis ; Tomography, X-Ray Computed

Antiphospholipid syndrome is characterized by recurrent episodes of arterial and venous thrombosis, spontaneous fetal losses, thrombocytopenia and persistently elevated levels of antiphospholipid antibodies. We experienced a case of Budd-Chiari syndrome in a 32-year old female lupus patient who was presented with left leg edema, ascites and esophageal varix. The clinical and laboratory findings were compatible with the cirteria for systemic lupus erythematosus (SLE) and she was found to have anticardiolipin antibody, thrombocytopenia and prolonged partial thromboplastin time. Initially, she was treated with intravenous heparin and uroki nase and she was followed up with warfarin, baby aspirin and steroids.
Adult ; Angiography ; Animal ; Antibodies, Antiphospholipid/blood* ; Case Report ; Drug Therapy, Combination ; Female ; Hepatic Vein Thrombosis/complications ; Hepatic Vein Thrombosis/diagnosis* ; Hepatic Vein Thrombosis/drug therapy ; Human ; Lupus Erythematosus, Systemic/complications ; Lupus Erythematosus, Systemic/diagnosis* ; Lupus Erythematosus, Systemic/drug therapy ; Tomography, X-Ray Computed

Lupus enteritis is a rare, severe complication of systemic lupus erythematosus (SLE), needing prompt diagnosis and proper management. However, SLE rarely presents as lupus enteritis at the time of initial diagnosis. Thus, delayed diagnosis and misdiagnosis are common. We report a case of a 25-year-old woman with lupus panenteritis. The patient had multiple hospitalizations for abdominal pain, nausea, and diarrhea, initially without any other symptoms suggestive of SLE, but was later observed to have malar rash and oral ulcers. Laboratory investigations were compatible with SLE, including positive antinuclear antibody (1:320) with speckled pattern. CT revealed diffuse hypodense submucosal thickening of the stomach, the entire small bowel, colon, appendix, and rectum. Treatment with high-dose corticosteroids followed by maintenance therapy with mycophenolate mofetil, hydroxychloroquine, and azathioprine resulted in clinical improvement. Diagnosis of lupus enteritis requires a high index of suspicion given the low incidence and nonspecific clinical findings.
Abdominal Pain/complications ; Adrenal Cortex Hormones/therapeutic use ; Adult ; Brain/diagnostic imaging ; Diagnosis, Differential ; Diarrhea/complications ; Endoscopy, Gastrointestinal ; Enteritis/pathology ; Female ; Humans ; Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy ; Magnetic Resonance Imaging ; Nausea/complications ; Tomography, X-Ray Computed

A 21-year-old woman presented with bilateral optic neuritis, combined with central retinal vein occlusion. General physical examination and neurologic consultation revealed no other findings. Laboratory investigation yielded an elevated erythrocyte sedimentation rate, positive LE preparation, elevated ANA titer, and elevated blood urea nitrogen and creatinine levels. Diagnosis of systemic lupus erythematosus (SLE) was made. Renal failure developed quickly and she was treated with hemodialysis, transfusion and subsequently systemic corticosteroid. Anti-phospholipid antibody was positive to lupus anti-coagulant and the titer was normalized after 2-month steroid therapy at which time the visual outcome differed between the eyes. The right eye showed improvement in visual acuity and visual field, but the left eye was not improved and retained a central scotoma. SLE needs to be considered in young women with optic neuritis when other causes of optic neuritis have been excluded, and serologic tests including anti-phospholipid antibody should be conducted.
Adult ; Case Report ; Female ; Fluorescein Angiography ; Human ; Lupus Erythematosus, Systemic/complications/*diagnosis/drug therapy ; Optic Neuritis/*diagnosis/drug therapy/etiology ; Perimetry ; Prednisolone/*therapeutic use ; Treatment Outcome ; Visual Acuity ; Visual Fields

Transverse myelitis (TM) extending from midbrain to the entire spinal cord accompanied by internuclear ophthalmoplegia is extremely rare but cause serious central nervous system complications in patients with systemic lupus erythematosus. We report a case of a 28-yr-old woman with TM extending from the midbrain to the conus medullaris longitudinally and internuclear ophthalmoplegia associated with systemic lupus erythematosus. Her neurological symptoms had an abrupt catastrophic onset and rapidly progressed to respiratory failure within 24 hr. Bilateral internuclear ophthalmoplegia was also followed by TM. Brain MR images showed definite brainstem lesions, which were deeply associated with internuclear ophthalmoplegia, and diffuse signal changes in the whole spinal cord, medulla, pons and midbrain. Clinical improvement of her ophthalmoplegia and of neurological dysfunction of the upper extremities was noted after prompt and aggressive treatment with intravenous pulsed methylprednisolone and cyclophosphamide. However, the neurological dysfunction of the lower limbs and bladder and colon paralysis were almost unchanged until six months passed.
Adult ; Cyclophosphamide/therapeutic use ; Female ; Humans ; Lupus Erythematosus, Systemic/*complications ; Magnetic Resonance Imaging ; Myelitis, Transverse/diagnosis/drug therapy/*etiology ; Ocular Motility Disorders/diagnosis/drug therapy/*etiology ; Prednisolone/therapeutic use

BACKGROUND/AIMS: Protein-losing enteropathy (PLE), characterized by severe hypoalbuminemia and peripheral edema, is a rare manifestation of systemic lupus erythematosus. This present study aimed to identify the distinctive features of lupus-related PLE and evaluate the factors related to the treatment response. METHODS: From March 1998 to March 2014, the clinical data of 14 patients with lupus PLE and seven patients with idiopathic PLE from a tertiary center were reviewed. PLE was defined as a demonstration of protein leakage from the gastrointestinal tract by either technetium 99m-labelled human albumin scanning or fecal alpha1-antitrypsin clearance. A positive steroid response was defined as a return of serum albumin to > or = 3.0 g/dL within 4 weeks after initial steroid monotherapy, and remission as maintenance of serum albumin > or = 3.0 g/dL for at least 3 months. A high serum total cholesterol level was defined as a level of > or = 240 mg/dL. RESULTS: The mean age of the lupus-related PLE patients was 37.0 years, and the mean follow-up duration was 55.8 months. Significantly higher erythrocyte sedimentation rate and serum total cholesterol levels were found for lupus PLE than for idiopathic PLE. Among the 14 patients with lupus PLE, eight experienced a positive steroid response, and the serum total cholesterol level was significantly higher in the positive steroid response group. A positive steroid response was associated with an initial high serum total cholesterol level and achievement of remission within 6 months. CONCLUSIONS: In lupus-related PLE, a high serum total cholesterol level could be a predictive factor for the initial steroid response, indicating a good response to steroid therapy alone.
Adult ; Aged ; Biomarkers/blood ; Cholesterol/blood ; Drug Therapy, Combination ; Edema/diagnosis/drug therapy/*etiology ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hypoalbuminemia/diagnosis/drug therapy/*etiology ; Immunosuppressive Agents/therapeutic use ; Lupus Erythematosus, Systemic/*complications/diagnosis/drug therapy ; Male ; Middle Aged ; Protein-Losing Enteropathies/diagnosis/drug therapy/*etiology ; Remission Induction ; Risk Factors ; Serum Albumin/metabolism ; Tertiary Care Centers ; Time Factors ; Treatment Outcome

No abstract available.
Antirheumatic Agents/therapeutic use ; Arthritis, Rheumatoid/blood/*complications/diagnosis/drug therapy/physiopathology ; Biological Markers/blood ; Drug Therapy, Combination ; Facial Dermatoses/complications/diagnosis ; Female ; Hand Joints/physiopathology/radiography ; Humans ; Immunosuppressive Agents/therapeutic use ; Inflammation Mediators/blood ; Knee Joint/physiopathology/radiography ; Lupus Erythematosus, Systemic/blood/*complications/diagnosis/drug therapy ; Middle Aged ; Syndrome ; Treatment Outcome

BACKGROUND: We assessed the efficacy of serial interferon-gamma release assays (IGRAs) for the diagnosis of latent tuberculosis infection (LTBI) in patients receiving immunosuppressive agents for treatment of rheumatic diseases in Korea. METHODS: Of 276 patients who underwent consecutive screening with one of two IGRAs [QuantiFERON-TB Gold or QuantiFERON-TB Gold In-Tube], 66 patients were evaluated by the serial IGRA for detection of LTBI during therapy with immunosuppressive agents. Information on clinical diagnosis, medication, previous TB, blood cell count, tuberculin skin test, and interferon-gamma (IFN-gamma) level measured by IGRA was collected. RESULTS: Of the 66 patients, the initial IGRA was positive in 24.2%, negative in 65.2%, and indeterminate in 10.6%. Forty-six patients (69.7%) showed consistent IGRA results during follow-up, and 13 patients (19.7%) had consistently positive results. IGRA conversion rate was 12.1% (8/66) and reversion rate was 4.5% (3/66). Conversion of IGRA results was only observed in ankylosing spondylitis patients, and the median interval between the two tests in patients with conversion was 8.5 months. The mean IFN-gamma level in the group of patients with consistently positive IGRA results was higher than that in the group with inconsistently positive results, although this trend was not statistically significant (P=0.293). Indeterminate results were observed most frequently in patients with systemic lupus erythematosus. CONCLUSIONS: In patients receiving immunosuppressive agents, both IGRA conversions and reversions were observed. Serial IGRA testing may not be needed in patients with a positive initial IGRA result showing high IFN-gamma levels, because of high consistency in the test results.
Adult ; Blood Cell Count ; Female ; Follow-Up Studies ; Humans ; Immunosuppressive Agents/*therapeutic use ; Interferon-gamma/*analysis ; *Interferon-gamma Release Tests ; Latent Tuberculosis/complications/*diagnosis/metabolism ; Lupus Erythematosus, Systemic/complications/diagnosis/metabolism ; Male ; Middle Aged ; Rheumatic Diseases/complications/diagnosis/drug therapy ; Spondylitis, Ankylosing/complications/diagnosis/metabolism ; Tuberculin Test

OBJECTIVEAntiphospholipid antibody (APL) is a particularly important laboratory diagnostic criterion for antiphospholipid syndrome (APS). The significances of positive APL in childhood are seldom reported nor fully understood. The purpose of this study was to analyze 13 cases with positive APL seen in our hospital and to study the relationship between the positive rates of APL and various clinical diseases especially systemic lupus erythematosus (SLE) in order to improve the clinical diagnoses and treatment level of APS in children.

METHODSThe clinical data collected from 2000 to 2002 of 13 hospitalized children with positive APL were retrospectively evaluated. Enzyme linked immunosorbent assay (ELISA) and indirect immunofluorescence technique were used respectively to detect APL and antineutrophil cytoplasmic autoantibodies (ANCA) of sera from those children. Other various indexes were also detected according to different characteristics of different diseases.

RESULTSEight cases had SLE; 2 had acute post-streptococcal infections. The other 3 cases did not show any evidences of primary diseases; they probably had primary APS. SLE was the most common primary diseases to cause development of APL and the cases with SLE showed more severe cutaneous vasculitis than SLE patients who were negative for APL. There was no significant relationship between the positive rates of APL and that of ANCA. Eight APL positive cases complicated with thrombocytopenia and bleeding were treated with high dosage of immunoglobulin [400 mg/(kg.d), for 3 - 5 d] intravenously; the clinical conditions of these cases were ameliorated soon. While the 5 cases who had thrombotic vasculitis and thromboembolism were treated with anticoagulant and antithrombotic therapy with low molecular weight heparin [50 - 100 U/(kg.d)], which led to good clinical effects.

CONCLUSIONSThe clinical manifestations of children positive for APL were somehow different from those of adults. Positive APL itself may be nonspecific, it can occur from different causes of diseases. APL detection may be useful to suggest anticoagulant and/or antithrombosis therapy. Treatments for APS should be variable according to different causes and severity of diseases, in the cases of thrombocytopenia and bleeding, high dose intravenous immunoglobulin should be given as soon as possible, while in the cases of thrombotic vasculitis and thromboembolism, anticoagulant and antithrombotic therapy should be given soon.

Adult ; Antibodies, Antineutrophil Cytoplasmic ; blood ; Antibodies, Antiphospholipid ; blood ; immunology ; Anticoagulants ; therapeutic use ; Antiphospholipid Syndrome ; blood ; complications ; diagnosis ; therapy ; Child ; Fibrinolytic Agents ; therapeutic use ; Hemorrhage ; etiology ; therapy ; Humans ; Immunoglobulins, Intravenous ; therapeutic use ; Lupus Erythematosus, Systemic ; immunology ; Streptococcal Infections ; immunology ; Thrombocytopenia ; etiology ; therapy ; Thromboembolism ; drug therapy ; etiology ; Thrombosis ; drug therapy ; etiology ; Vasculitis ; drug therapy ; etiology