Systemic lupus erythematosus (SLE) is a multisystem ic inflammatory disorder mediated by autoantibodies and immune complexes that manifests with a variety of symptoms. Lupus enteritis is a serious complication of SLE and carries a high mortality rate, however the diagnosis is not easy for there are no specific clinical manifestations and laboratory findings. Lupus enteritis usually involves the mesenteric arteries causing ischemic changes of the small and large bowels, and yet rarely involves the rectum. Here, we report a case of a 26-year-old female lupus enteritis patient who presented with atypical abdominal pain. Early diagnosis was made by abdominal pelvic computed tomography (CT) scan, which showed extensive involvement of lupus enteritis from proximal small bowel to rectum. She recovered after being treated with high dose steroid therapy.
Abdominal Pain ; Adult ; Antigen-Antibody Complex ; Autoantibodies ; Diagnosis ; Early Diagnosis ; Enteritis* ; Female ; Humans ; Lupus Erythematosus, Systemic ; Mesenteric Arteries ; Mortality ; Rectum*

Infection is a frequent problem in patients with systemic lupus erythematosus (SLE). Infections contribute greatly to the morbidity of patients and are one of the commonest causes of death. The high frequency and unusual spectrum of infections can be attributed to the multiple disturbances of immune function in SLE in combination with the effects of immunosuppressive therapy. There is increasing evidence to indicate that opportunistic infections including tuberculosis make a large contribution to the infectious mortality in SLE. Tuberculosis is a major cause of morbidity and mortality, particularly in our country where tuberculosis is still endemic. The indolent nature of tuberculous bone and joint disease often leads to delayed or missed diagnosis, sometimes with devastating consequences for the patient. We report a case of multifocal and complicated osteoarticular tuberculosis developing in the spines and knee joint due to delayed diagnosis, with review of literatures.
Cause of Death ; Delayed Diagnosis ; Diagnosis ; Humans ; Joint Diseases ; Knee Joint ; Lupus Erythematosus, Systemic* ; Mortality ; Opportunistic Infections ; Spine ; Tuberculosis ; Tuberculosis, Osteoarticular*

Cryptococcosis is an opportunistic infection that generally occurs in patients with cell-mediated immune dysfunction and involves the central nervous system. Infection is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE) patients because of its innate immune dysfunction along with the administration of steroids and immunosuppressants. However, central nervous system cryptococcosis has rarely been reported in SLE patients. A timely diagnosis is critical because of its significant mortality and morbidity. Most cases of cryptococcal meningitis in SLE patients have been reported in those treated with steroids or immunosuppressants. We report on a SLE patient not on medication, who was diagnosed with cryptococcal meningoencephalitis.
Central Nervous System ; Cryptococcosis ; Diagnosis ; Humans ; Immunosuppressive Agents* ; Lupus Erythematosus, Systemic* ; Meningitis, Cryptococcal ; Meningoencephalitis* ; Mortality ; Opportunistic Infections ; Steroids

Systemic lupus erythematosus(SLE) is a disease of unknown etiology in which multiple organs are damaged by pathogenic autoantibodies and immune complexes. Neuropsychiatric manifestations in SLE were first described by Kaposi in 1872. These are so diverse that they include psychosis, depression, stroke, seizure and cognitive dysfunction etc. These patients are frequently consulted for psychiatric evaluation. Neuropsychiatric manifestations in SLE are also among the leading causes of morbidity and mortality and associated with poor long-term outcome. So it is essential to recognize and intervene these symptoms early. But the clear diagnostic criteria for CNS involvement in SLE have not been formulated, and diversity and fluctuation of illness make it difficult to confirm it. The authors reported three cases of SLE with severe neuropsychiatric manifestations. These patients showed symptoms such as disorientation, auditory and visual hallucibation, delusion and mood instability. They became frequently impulsive and violent and had risks to injure themselves or others. Although CNS involvement in SLE is not well known, we reviewed the pathogenesis, classification, diagnosis, clinical manifestation and treatment of them.
Antigen-Antibody Complex ; Autoantibodies ; Classification ; Delusions ; Depression ; Diagnosis ; Humans ; Lupus Erythematosus, Systemic* ; Mortality ; Psychotic Disorders ; Seizures ; Stroke

Most rheumatic diseases are chronic inflammatory diseases. Kidney-related symptoms of rheumatic diseases are often present, which increase mortality and morbidity of patients with rheumatic diseases. When patients with rheumatic diseases show signs or symptoms of renal involvement, management for primary rheumatic diseases should be more aggressive. In general, the risk and severity of renal involvement in patients with rheumatic diseases depend on the type of primary rheumatic diseases. Rheumatic disease itself, chronic use of immunosuppressive agents and non-steroidal anti-inflammatory drugs, and comorbidities, such as diabetes, hypertension, and cardiovascular complications, are the main causes of renal involvement in patients with rheumatic diseases. Many studies have reported the predominant features of renal involvement in most rheumatic diseases. We have attempted to summarize the relationships between rheumatic diseases and renal diseases, and clinical or pathophysiological features of renal involvement resulting from primary rheumatic diseases except systemic lupus erythematosus. Review for renal involvement, particularly in relation to early diagnosis and management of renal involvement in rheumatic diseases, is clinically significant because renal involvement in rheumatic diseases generally implies a bad prognosis.
Comorbidity ; Early Diagnosis ; Humans ; Hypertension ; Immunosuppressive Agents ; Inflammation ; Kidney Diseases ; Lupus Erythematosus, Systemic ; Mortality ; Prognosis ; Rheumatic Diseases*

Systemic lupus erythematosus is a systemic disorder which has frequent involvement of gastrointestinal tract. Non specific symptoms such as anorexia, nausea, diarrhea and abdominal pain are well known symptoms when the gastrointestinal tract is involved. The most feared gastrointestinal complication of systemic lupus erythematosus is lupus enteritis. The pathological change in lupus enteritis is usually a result of mesenteric vasculitis. Major complications such as intestinal bleeding and perforation may occur and sometimes result in sugery. Because of high mortality rate in case of major complications, early diagnosis and appropriate treatment is very important. We experienced three patients with lupus enteritis who presented with severe abdominal pain and dirrhea. They were diagnosed by characteristic radiographical findings of small bowel series and barium study. All radiographical findings has been resolved completely with the steroid therapy. Conclusively we can induce complete remission by steroid therapy alone, if we diagnose lupus enteritis in the early period of disease course.
Abdominal Pain ; Anorexia ; Barium ; Diarrhea ; Early Diagnosis ; Enteritis* ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Lupus Erythematosus, Systemic ; Mortality ; Nausea ; Vasculitis

Systemic lupus erythematosus(SLE) is an autoimmune disease involving many organs. Mesenteric vasculitis is one of the serious organ involvement in this disease. Mesenteric vasculitis may produce mucosal ulceration, bowel edema with paralytic ileus, hemorrhagic ileitis, intussusception, perforation and mesenteric infarction. Because of high mortality rate, early diagnosis and treatment of this manifestation is very important. We experienced a case of SLE with extensive smell bowel infarction due to mesenteric vasculitis. She was 29 years old. She was suffered frem severe abdominal pain for three days. Abdominal computed tomography showed diffusse wall thickening of the small intestinal wall. After high dose intravenous steroid, her abdominal symptoms were improved. She was discharged with low dose oral steroid(15mg/day) treatment. Seven months later, she presented with sudden abdominal pain. Intravenous methylprednisolon(2mg/Kg) was started. Three days later, her abdominal symptoms were not subsided. On the plain radiograph of the abdomen, marked ileus was demonstrated. Mesenteric angiography showed diffuse decreased small bowel vascularity. A provisional diagnosis of small bowel infarction and obstruction, we performed exploratory laparotomy. She undergone small bowel segmental resection. The diagnosis was small bowel infaction due to mesenteric vasculitis.
Abdomen ; Abdominal Pain ; Adult ; Angiography ; Autoimmune Diseases ; Diagnosis ; Early Diagnosis ; Edema ; Humans ; Ileitis ; Ileus ; Infarction* ; Intestinal Pseudo-Obstruction ; Intussusception ; Laparotomy ; Lupus Erythematosus, Systemic ; Mortality ; Smell ; Ulcer ; Vasculitis*

BACKGROUND: Diffuse alveolar hemorrhage (DAH) is rare but often fatal. To determine the clinical manifestations of DAH, its etiology, clinical course and prognosis were studied. METHODS: A retrospective analysis was performed in 21 patients that were diagnosed as DAH. Diagnosis of DAH was based on the presence of the "classical triad" of hemoptysis, anemia, and rapidly progressive infiltrates on chest X-ray and a finding of bronchoalveloar lavage or lung biopsy. RESULTS: Thirteen patients (61.9%) had collagen vascular diseases (CVDs) as underlying disease and 10 patients had systemic lupus erythematosus. Females were more prevalent in CVD than in non-collagen vascular disease (NCVD). Otherwise, there were no significant differences between the two groups in terms of clinical manifestations. Dyspnea (95.2%), cough (76.2%), hemoptysis (61.9%), and fever (33.0%) were frequent symptoms. The initial creatinine level was higher in CVD than in NCVD (3.27±3.15 mg/dl vs. 1.19±0.94 md/dl, p=0.030). The corresponding drop in hemoglobin level was 2.69±1.26 g/dl. Maximal drop in hemoglobin preceded the progression of infiltrates on the chest radiograph by 1.38±4.22 days. The mortality rate was higher in the patients with NCVD than in those with CVD (50.0% vs. 23.1%). CONCLUSION: The DAH can occur not only in patients with CVD but also in those with NCVD. Higher creatinine level CVD in patients is associated with renal involvement in conjunction with DAH. The maximal drop in hemoglobin preceeding the progression of infiltrates on the chest radiograph suggests that the drop in hemoglobin is important for diagnosing DAH.
Anemia ; Biopsy ; Collagen ; Cough ; Creatinine ; Diagnosis ; Dyspnea ; Female ; Fever ; Hemoptysis ; Hemorrhage* ; Humans ; Lung ; Lupus Erythematosus, Systemic ; Mortality ; Prognosis ; Radiography, Thoracic ; Retrospective Studies* ; Therapeutic Irrigation ; Thorax ; Vascular Diseases

Systemic lupus erythematosus(SLE) is an autoimmune disease which may affect many different organs and disclose various clinical manifestations. Recently central nervous system(CNS) involvement has been recognized as an increasingly significant contributor to morbidity and mortality of SLE. The clinical manifestations of CNS-lupus are highly variable and range from mild cognitive dysfunction, movement disorder, headache, psychosis to life-threatening stroke and coma. Among the neuropsychiatric disorders encountered in patients with SLE, cerebrovascular disease has been a relatively rare complication. The diagnosis and management of CNS-lupus is difficult because of the lack of useful diagnostic methods. If, cerebrovascular involvement is suspected, then aggressive treatment such as high dose steroid, immunosuppressive therapy, plasma exchange, may be required to reduce high mortality rate. We experienced 2 cases cerebrovascular disease occurring in SLE patients which presented with various neuropsychiatric manifestations. They were diagnosed as CNS-lupus by neuropsychiatric symptoms, brain MRI, and EEG, and showed good response to high dose steroid pulse therapy.
Autoimmune Diseases ; Brain ; Coma ; Diagnosis ; Electroencephalography ; Headache ; Humans ; Lupus Erythematosus, Systemic* ; Magnetic Resonance Imaging ; Mortality ; Movement Disorders ; Plasma Exchange ; Psychotic Disorders ; Stroke

PURPOSE: Pneumocystis jirovecii pneumonia occurs in various immunocompromised patients. Despite the prophylaxis strategies in clinical practice, certain patients develop P. jirovecii pneumonia. This study was performed to investigate pediatric cases with P. jirovecii pneumonia in a single center. METHODS: We identified pediatric patients younger than 19 years with microbiologically confirmed P. jirovecii pneumonia from January 2000 to February 2014. A retrospective chart review was performed. RESULTS: Fifteen episodes of P. jirovecii pneumonia in 14 patients were identified with median age of 8.3 years (range, 0.4-18.6 years). Among these patients, 11 patients had hematology-oncology diseases, 2 had primary immunodeficiency disorders (one with severe combined immunodeficiency and the other with Wiskott Aldrich syndrome), 1 had systemic lupus erythematosus and 1 received kidney transplant. Four patients were transplant recipients; 1 allogeneic and 2 autologous hematopoietic cell transplant and 1 with kidney transplant. The median absolute lymphocyte count at the diagnosis of P. jirovecii pneumonia was 5,156 cells/mm³ (range, 20-5,111 cells/mm³). In 13 episodes (13 of 15, 86.7%), patients were not receiving prophylaxis at the onset of P. jirovecii pneumonia. For treatment, trimethoprim/sulfamethoxazole was given as a main therapeutic agent in all 15 episodes. Steroid was given in 9 episodes (60%). Median treatment duration was 15 days (range, 4-33 days). Overall mortality at 60 days was 35.7% (5 of 14). CONCLUSION: Majority of our patients developed P. jirovecii pneumonia while not on prophylaxis. Continuous efforts and more data are needed to identify high risk patients who may get benefit from P. jirovecii pneumonia prophylaxis.
Diagnosis ; Humans ; Immunocompromised Host ; Kidney ; Lupus Erythematosus, Systemic ; Lymphocyte Count ; Mortality ; Pediatrics ; Pneumocystis carinii ; Pneumocystis jirovecii* ; Pneumocystis* ; Pneumonia* ; Retrospective Studies ; Severe Combined Immunodeficiency ; Transplant Recipients ; Transplants