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*

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

BACKGROUND/AIMS: To compare the clinical characteristics and outcomes of systemic lupus erythematosus between male and female Korean patients. METHODS: A retrospective analysis was performed at a single tertiary hospital from August 1994 to May 2010. Male patients were matched with two to three female patients based on age and disease duration. Organ damage was assessed using the Systemic Lupus International Collaborating Clinics/American College of Rheumatology damage index (SLICC/ACR DI). RESULTS: Fifty-three male patients were compared with 150 female patients. Renal disorders were found more frequently in male patients at disease onset (p < 0.001); the adjusted odds ratio (OR) demonstrated a significant sex preponderance for renal manifestations (OR, 3.26; 95% confidence interval [CI], 1.62 to 6.57). Diffuse proliferative lupus nephritis and end-stage renal disease requiring dialysis during the disease course were more prevalent in male patients (p = 0.025 and p < 0.001, respectively). The risk for requiring long-term dialysis was significantly higher in male than in female patients (OR, 4.02; 95% CI, 1.07 to 15.06), as was the mean SLICC/ACR DI (1.55 +/- 1.35 vs. 1.02 +/- 1.57, respectively; p = 0.028). CONCLUSIONS: Our data demonstrate that Korean patients with lupus have characteristics similar to those of cohorts reported previously. Male patients had significantly higher incidences of renal manifestations and organ damage.
Adult ; *Asian Continental Ancestry Group ; Female ; Humans ; Immunosuppressive Agents/therapeutic use ; Incidence ; Kaplan-Meier Estimate ; Kidney Failure, Chronic/diagnosis/ethnology/therapy ; Lupus Erythematosus, Systemic/diagnosis/*ethnology/mortality/therapy ; Lupus Nephritis/diagnosis/ethnology/therapy ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prevalence ; Prognosis ; Renal Dialysis ; Republic of Korea/epidemiology ; Retrospective Studies ; Risk Factors ; Sex Distribution ; Sex Factors ; Tertiary Care Centers ; Time Factors ; Young Adult

BACKGROUND/AIMS: We investigated whether transthoracic echocardiography-suspected pulmonary hypertension (PH) affects survival in systemic lupus erythematosus (SLE) patients and examined factors associated with PH occurrence and survival. METHODS: This retrospective single-center study included 154 Korean SLE patients fulfilling the American College of Rheumatology criteria (January 1995 to June 2013). Student t test, Mann-Whitney U test, Kaplan-Meier curves, and log-rank tests were used for comparisons. RESULTS: A total of 35 SLE patients with PH (SLE/PH+) and 119 without PH (SLE/PH-) were analyzed. Higher percentages of interstitial lung disease, Raynaud's phenomenon (RP), World Health Organization functional classification III/IV, and cardiomegaly were found in SLE/PH+ compared to SLE/PH-. Furthermore, the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index was significantly higher in SLE/PH+ (2.46 +/- 1.245 vs. 1.00 +/- 1.235), whereas survival rates were significantly higher in SLE/PH- in log-rank tests (p = 0.001). In multivariate analysis, the adjusted mortality hazard ratio (HR) for SLE/PH+ patients was 3.10. Subgroup analysis demonstrated a higher percentage of lupus nephritis in the SLE/PH+ patients who died (p = 0.039) and low complement-3 levels (p = 0.007). In univariate analysis, the mortality HR for SLE/PH+ patients with lupus nephritis was 4.62, whereas the presence of RP decreased the mortality risk in multivariate analysis; adjusted HR, 0.10. CONCLUSIONS: PH is an independent factor predicting survival in SLE patients. The presence of lupus nephritis resulted in an increased trend for mortality, whereas coexistence of RP was associated with a better survival prognosis in SLE/PH+ patients.
Adolescent ; Adult ; Cardiomegaly/diagnosis/epidemiology ; Chi-Square Distribution ; Female ; Humans ; Hypertension, Pulmonary/diagnosis/*mortality ; Kaplan-Meier Estimate ; Lung Diseases, Interstitial/diagnosis/mortality ; Lupus Erythematosus, Systemic/diagnosis/*mortality ; Lupus Nephritis/diagnosis/mortality ; Male ; Multivariate Analysis ; Prognosis ; Proportional Hazards Models ; Raynaud Disease/diagnosis/epidemiology ; Republic of Korea ; Retrospective Studies ; Risk Factors ; Young Adult

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 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*

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

BACKGROUND: Pulmonary embolism is a relatively common disease but may also be manifestated as a lethal disease. Most previous studies on pulmonary embolism included hemodynamically stable patients who were able to tolerate a confirmative diagnostic workup, including ventilation-perfusion lung scan or pulmonary angiography. However, in most cases of acute massive pulmonary embolism, patients are unstable to tolerate a confirmative diagnostic workup. Studies of only stable patients with pulmonary embolism may have a bias on evaluating the clinical course and prognosis of pulmonary embolism. Therefore, we designed a study to observe the clinical manifestations, diagnostic methods, treatment modality, and to investigate the prognostic factors of patients with acute pulmonary embolism who present with overt or impending right heart failure using the diagnostic criteria suggested by MAPPET study. METHODS: Among 103 patients diagnosed as pulmonary embolism from 1990 to 1997, 63 patients(male/female : 21/42, mean age : 56 15) were enrolled as acute major pulmonary embolism by MAPPET's diagnostic criteria. Patients were included in the study if they showed clinical, echocardiographic and cardiac catheterization findings signifying acute right heart failure or pulmonary hypertension due to pulmonary embolism, together with: 1) a diagnostic pulmonary angiogram, or 2) a lung scan indicating high probability of pulmonary embolism, or 3) at least 3 of the followings: 1) syncope; 2) tachycardia (heart rate > 100 beats /min); 3) dyspnea or tachypnea (> 24 breaths/min or need for mechanical ventilation); 4) arterial hypoxemia (partial arterial pressure of oxygen < 70mmHg while breathing room air) in the absence of pulmonary infiltrates on chest x-ray; 5) ECG signs of right heart strain. RESULTS: Among the 63 patients, 15 patients(23.8%) did not have an underlying disease. Eleven patients(17.5%) had malignancy, 8 patients had an operation in the recent 20 days, 6 patients had chronic pulmonary disease, 5 patients had a history of congestive heart failure and cerebrovascular accident respectively, 4 patients had a previous history of pulmonary embolism, 3 patients had vasculitis such as Behcets' disease and systemic lupus erythematosus and a history of venous thrombosis, respectively. The main clinical manifestation on the time of diagnosis was dypnea in 55 patients(87.3%), which was the most frequent, and chest pain in 18 patients(28.6%), syncope in 10 patients(15.9%), and tachycardia in 2 patients(3.2%). The diagnostic methods were echocardiography(43 patients, 68.3%), lung perfusion scan(39 patients, 61.9%), chest computed tomography(16 patients, 26.4%), pulmonary angiography(4 patients, 6.3%) and right heart catherization (2 patients, 3.2%). In order to examine deep vein thrombosis, lower extremity Duplex ultrusonography and venography were performed in 11 patients(17.5%) and 7 patients(11.1%) respectively. The overall in-hospital mortality was 38.1%(24 patients). The factors influencing in-hospital mortality were associated malignancy(p< 0.01) and unstable vital sign(systolic blood pressure of less than 90mmHg)(p< 0.05). CONCLUSION: Acute pulmonary embolism with overt or impending right heart failure is a significant lethal disease with a high in-hospital mortality. The predictors of mortality were associated malignancy and unstable vital sign.
Angiography ; Anoxia ; Arterial Pressure ; Bias (Epidemiology) ; Blood Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Chest Pain ; Diagnosis ; Dyspnea ; Echocardiography ; Electrocardiography ; Heart ; Heart Failure ; Hospital Mortality* ; Humans ; Hypertension, Pulmonary ; Lower Extremity ; Lung ; Lung Diseases ; Lupus Erythematosus, Systemic ; Mortality ; Oxygen ; Perfusion ; Phlebography ; Prognosis ; Pulmonary Embolism* ; Respiration ; Stroke ; Syncope ; Tachycardia ; Tachypnea ; Thorax ; Vasculitis ; Venous Thrombosis ; Vital Signs

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

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*