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Background: To investigate the risk factors for the development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE)., Methods: The literature related to risk factors for the development of PAH in SLE patients was searched by the computer on China national knowledge infrastructure (CNKI), PubMed, and Embase, and the literature search was limited to the period of library construction to October 2022. Two researchers independently performed literature screening and literature information extracting, including first author, publication time, case collection time, sample size, and study factors, and used the Newcastle-Ottawa Scale (NOS) to evaluate the quality of the literature. The relationship between each clinical manifestation and laboratory index and the occurrence of PAH in SLE patients was evaluated based on the ratio (OR value) and its 95% CI., Results: A total of 24 publications were included, including 23 case-control studies and 1 cohort study with NOS ≥ 6, and the overall quality of the literature was high. The risk of PAH was higher in SLE patients who developed Raynaud phenomenon than in those who did not [OR = 2.39, 95% CI (1.91, 2.99), P < .05]; the risk of PAH was higher in SLE patients who were positive for anti-RNP antibodies than in those who were negative for anti-RNP antibodies [OR = 1.77, 95% CI (1.17, 3.2.65), P < .05]; the risk of PAH was higher in SLE patients with interstitial lung lesions than in those without combined interstitial lung lesions [OR = 3.28, 95% CI (2.37, 4.53), P < .05]; the risk of PAH was higher in SLE patients with combined serositis than in those without serositis [OR = 2.28, 95% CI (1.83, 2.84), P < .05]. The risk of PAH was higher in SLE patients with combined pericardial effusion than in those without pericardial effusion [OR = 2.97, 95% CI (2.37, 3.72), P < .05]; the risk of PAH was higher in SLE patients with combined vasculitis than in those without vasculitis [OR = 1.50, 95% CI (1.08, 2.07), P < .05]; rheumatoid factor-positive SLE patients had a higher risk of PAH than those with rheumatoid factor-negative [OR = 1.66, 95% CI (1.24, 2.24), P < .05]., Conclusion: Raynaud phenomenon, vasculitis, anti-RNP antibodies, serositis, interstitial lung lesions, rheumatoid factor, and pericardial effusion are risk factors for the development of PAH in patients with SLE., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objective: Endothelial dysfunction (ED) has an important role in the pathogenesis of systemic lupus erythematosus (SLE). Studies on other inflammatory diseases show that salusin-β with various mechanisms may play a role in the promotion of ED and inflammation. The aim of this study was to measure serum salusin-β levels in SLE patients and evaluate it as a potential biomarker in assessing SLE activity and predicting organ involvement., Methods: In a cross-sectional study, 60 patients diagnosed with SLE and 30 age- and sex-matched healthy controls were enrolled. Disease activity of SLE patients was assessed by the systemic lupus erythematosus disease activity index 2000 (SLEDAI-2 K). Serum levels of salusin-ß were measured using a human salusin-ß enzyme-linked immunosorbent assay kit., Results: Serum salusin-β levels in SLE and control groups were 474.2 ± 117.1 pg/ml and 157.7 ± 88.7 pg/ml, respectively. The difference was significant (P = 0.001). There was no significant correlation between serum salusin-β levels with age (r =  - 0.06, P = 0.632) and SLEDAI (r =  - 0.185, P = 0.158). In patients with nephritis and thrombosis, serum salusin-β was significantly higher. In addition, in patients with serositis, serum salusin-β was significantly lower. Multiple linear regression analysis showed that serum salusin-β levels retained a significant association with nephritis and thrombosis after model adjustment for serositis, nephritis, and thrombosis., Conclusions: Our findings showed that salusin-β might have a possible role in the pathogenesis of SLE. Salusin-β may be a potential biomarker for nephritis and thrombosis in SLE. Key Points • Serum salusin-β levels were significantly higher in SLE patients than the control group. • There was no significant correlation between serum salusin-β levels with age and SLEDAI. • Serum salusin-β levels retained a significant association with nephritis and thrombosis., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Purpose: To investigate the clinical and pathological characteristics of lupus nephritis (LN) patients with serositis and analyse the relationship between serositis and hyperuricemia (HUA) in LN patients in northeast China., Methods: The data of patients with LN diagnosed by renal biopsy in our hospital from April 2013 to May 2020 were retrospectively analyzed. The differences between the non-serositis and serositis groups were compared by t tests and Chi-square test. Factors with P < 0.05 in the univariate analyses were investigated further using binary logistic regression analysis to investigate the independent risk factors of serositis in patients with LN., Results: LN patients with serositis were more likely to have fever, hypertension, neuropsychiatric and hematological involvement than those without serositis (P < 0.05). Compared with the non-serositis group, LN patients with serositis were prone to have HUA, high D-dimer, high triglycerides, and had significant differences in the levels of plasma albumin (Alb), estimated glomerular filtration rate (eGFR), erythrocyte sedimentation rate, C-reactive protein, complement C3, 24-h urinary protein, pathological types, pathological score and SLEDAI score. Logistic regression analysis showed that HUA was one of risk factors for serositis in LN patients. The rate of complete remission in LN patients with serositis was significantly lower (P < 0.05) and the rate of no remission and mortality were significantly higher (P < 0.05) than LN patients without serositis., Conclusion: LN patients with serositis had more severe clinical and pathological manifestations, higher systemic lupus erythematosus (SLE) activity and worse prognosis. Hyperuricemia is associated with serositis in LN patients., (© 2021. The Author(s), under exclusive licence to Springer Nature B.V.)

Chronic myelomonocytic leukemia (CMML) is a rare clonal stem cell disorder associated with clinical and pathologic of myelodysplasia and myeloproliferation. Systemic autoimmune/inflammatory disorders (SAID) and polyserositis have been associated with CMML. These manifestations can be observed concomitantly, shortly before diagnosis or anytime along the course of illness. We report a case of myeloproliferative CMML who presented with polyserositis and positive serology for rheumatoid arthritis. Retrospective studies of myelodysplasia/CMML have reported 15% to 25% incidence of SAID. The most commonly observed disorders include systemic vasculitis, connective tissue diseases, polychondritis, seronegative arthritis, and immune thrombocytopenia. SAID does not confer adverse prognosis in retrospective studies. Polyserositis is less common; this may result from leukemic infiltrate or result from autoimmunity. Treatment of serositis includes steroids and cytoreductive agents. Serositis may confer poor prognosis and hypomethylating therapy may improve the outcome.

Objective: Artery calcification, as subclinical atherosclerosis, is attracting attention. The aim of this study was to determine the prevalence and risk factors of artery calcification in patients with systemic lupus erythematosus., Methods: 641 patients with systemic lupus erythematosus were enrolled in the study. Demographic, clinical, and laboratory characteristics were collected. Calcification score was quantified from the multi-detector computed tomography scan image using the Agatston Score method., Results: The total incidence of artery calcification was 25.9% (166/641), of which the percentages of aorta calcium and coronary artery calcification were 23.1% (148/641) and 8.4% (54/641), respectively. In multivariate models, systemic lupus erythematosus patients with artery calcification had longer disease duration than patients without artery calcification ( p < 0.05). Presence of serositis (OR 2.559, 95%CI 1.414-4.632), pneumonia (OR 2.022, 95%CI 1.102-3.711) and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score (OR 1.049, 95%CI 1.004-1.095) were independently associated with increased risk of aorta calcium, while the duration of corticosteroids use (OR 1.039, 95%CI 1.002-1.078) and cyclophosphamide therapy (OR 8.251, 95%CI 2.496-27.279) were independently associated with increased risk of coronary artery calcification in systemic lupus erythematosus patients. In systemic lupus erythematosus patients, aorta calcium was prone to occur at a younger age compared to coronary artery calcification, and aorta calcium score was positively correlated with age., Conclusions: Systemic lupus erythematosus patients had a much earlier onset and higher incidences of aorta calcium than coronary artery calcification. Presence of serositis, pneumonia, and higher SLEDAI score may predict increased risk of aorta calcium.

This study aimed to identify the long-term clinical outcomes and prognostic factors of patients with systemic lupus erythematosus (SLE)-associated pulmonary arterial hypertension (PAH) confirmed by right heart catheterisation.A multicentre prospective cohort of SLE-associated PAH was established. Baseline and follow-up records were collected. The primary end-point was death. The secondary exploratory end-point was treatment goal achievement (TGA), defined as an integrated outcome.In total, 310 patients were enrolled from 14 PAH centres. The 1-, 3- and 5-year survival rates were 92.1%, 84.8% and 72.9%, respectively. The 1-, 3- and 5-year TGA rates were 31.5%, 53.6% and 62.7%, respectively. Baseline serositis, 6-min walking distance >380 m and cardiac index ≥2.5 L·min -1 ·m -2 were identified as independent prognostic factors of TGA. Patients with baseline serositis were more likely to reach TGA after intensive immunosuppressive therapy. TGA was identified as a positive predictor of survival in patients with SLE-associated PAH.TGA was associated with long-term survival, which supports the treat-to-target strategy in SLE-associated PAH. Baseline heart function predicted both survival and treatment goal achievement in patients with SLE-associated PAH. Patients with serositis at baseline tended to benefit from intensive immunosuppressive therapy and have a better clinical outcome., Competing Interests: Conflict of interest: J. Qian has nothing to disclose. Conflict of interest: M. Li has nothing to disclose. Conflict of interest: X. Zhang has nothing to disclose. Conflict of interest: Q. Wang has nothing to disclose. Conflict of interest: J. Zhao has nothing to disclose. Conflict of interest: Z. Tian has nothing to disclose. Conflict of interest: W. Wei has nothing to disclose. Conflict of interest: X. Zuo has nothing to disclose. Conflict of interest: M. Zhang has nothing to disclose. Conflict of interest: P. Zhu has nothing to disclose. Conflict of interest: S. Ye has nothing to disclose. Conflict of interest: W. Zhang has nothing to disclose. Conflict of interest: Y. Zheng has nothing to disclose. Conflict of interest: W. Qi has nothing to disclose. Conflict of interest: Y. Li has nothing to disclose. Conflict of interest: Z. Zhang has nothing to disclose. Conflict of interest: F. Ding has nothing to disclose. Conflict of interest: J. Gu has nothing to disclose. Conflict of interest: Y. Liu has nothing to disclose. Conflict of interest: Y. Wang has nothing to disclose. Conflict of interest: X. Zeng has nothing to disclose., (Copyright ©ERS 2019.)

Objective: Nontraumatic osteonecrosis (ON) is a well-recognized complication causing disability and affecting quality of life in patients with systemic lupus erythematosus (SLE). The aim of this study was to identify the risk factors for ON, and to identify the minimal investigation(s) needed to optimally monitor the risk of ON in patients with SLE., Methods: A systematic review was conducted using MEDLINE and EMBASE. These databases were searched up to January 2016 using the Medical Subject Heading (MeSH) terms "Osteonecrosis," "Systemic lupus erythematosus," and synonymous text words. Randomized controlled trials, case control, cohort, and cross-sectional studies were included. Risk factors for ON in patients with SLE were compiled. The quality of each study was assessed using the Newcastle-Ottawa scale for nonrandomized studies. The quality of evidence of each risk factor was assessed using the Grading of Recommendations, Assessment, Development, and Evaluation method., Results: Of the 545 references yielded, 50 met inclusion criteria. Corticosteroid (CS) use may be strongly associated with ON in patients with SLE. Other clinical variables were moderately associated, including hypertension, serositis, renal disease, vasculitis, arthritis, and central nervous system disease. However, the evidence was low to very low in quality., Conclusion: Based on the best evidence available, CS use may be strongly associated with ON in patients with SLE. Results of this review were considered in the development of recommendations for the diagnosis and monitoring of patients with SLE in Canada and will guide clinicians in their assessment of these patients.

Rationale: Castleman disease (CD) is a nonclonal lymphoproliferative disorder sometimes manifested systemic inflammatory symptoms. Systemic lupus erythematosus (SLE) is an autoimmune disease characterized with multi-system involvement as well as broad spectrum of serum autoantibodies. When these two conditions happened to have similar clinical spectrum features, the confusion with each other occurred., Patient Concerns: A 46-year-old man suffered from chronic fever, nephrotic syndrome, acute kidney injury, anemia, thrombocytopenia and serositis, as well as hypocomplementemia and negative anti-nuclear antibody., Diagnoses: Meeting the classification criteria for SLE, the patient was diagnosed as SLE at first. The renal biopsy showed that he had endocapillary proliferative glomerulonephritis with negative immunofluorescence. Finally, he was diagnosed with CD after lymph nodes biopsy., Interventions: The patient was treated with oral prednisone (50 mg daily) but got poor response. After being proved to have CD, he was treated with CHOP chemotherapy., Outcomes: His condition was controlled by CHOP chemotherapy. After six course of chemotherapy, the proteinuria disappeared., Lessons: If patients, even qualified by classification criteria of SLE, had negative autoantibody or unsatisfied response to the standard treatment, the original diagnosis should be suspected. The biopsy may be help to identify the final criminals, such as CD.

Episcleritis and scleritis are relatively rare ocular diseases, which are commonly associated with rheumatic diseases including systemic lupus erythematosus (SLE). To investigate clinical and laboratory features of SLE-associated episcleritis and scleritis, we now report 4 cases of inpatients who were diagnosed with episcleritis or scleritis secondary to SLE from September 2005 to July 2016 in the Department of Rheumatology and Immunology in Peking University People's Hospital. Demographic, clinical and laboratory characteristics were summarized together with the treatment regimen and the prognosis; the literature was reviewed. There were 3 female and 1 male patients. The average age was (49.0±23.8) years and the mean duration of SLE at the onset of episcleritis or scleritis was (2.1±1.4) years. In addition to the eye involvement, the patients had mucocutaneous manifestations, serositis, lupus nephritis and interstitial pneumonia simultaneously; in the past, 1 patient experienced arthritis, 2 presented Raynaud's phenomenon, and 2 had hematologic involvement. All the patients had antinuclear antibody (ANA) of high titer. The anti double-stranded DNA (ds-DNA) antibody titers were increased in 2 patients. Three patients had positive anti-nucleosome antibody (ANuA) while the other 1 patient did not test it. The complement levels were decreased in 3 patients. The systemic lupus erythematosus disease activity index (SLEDAI) scores were more than 4 points in all the patients (ranging from 7-16), suggesting active disease. Ocular symptoms included pain, redness of the eye and tears. Ophthalmic examinations revealed 3 cases of episcleritis and 1 case of scleritis. Among the 4 patients, 2 patients experienced ocular complications including decrease in vision and uveitis. All the patients were treated with systemic corticosteroids combined with hydroxycloroquine; 3 patients were treated with immunosuppressants (cyclophosphamide in 2 patients and leflunomide in 1 patient). All of the 4 patients received topical steroid and 1 patient received periocular injection of triamcinolone acetonide; 1 patient received topical nonsteroidal anti-inflammatory drug (NSAID).No recurrence of episcleritis or scleritis was observed during the follow-ups. As a conclusion, scleritis and episcleritis, although uncommon, may occur in patients with autoimmune rheumatic diseases including SLE. The occurrence of episcleritis and scleritis may suggest active disease of SLE. Ocular complications need to be aware of in the patients. Prompt diagnosis and treatment was associated with good visual outcomes in the follow-ups.

The objective of this article is to investigate the clinical features of intestinal pseudo-obstruction (IPO) and/or ureterohydronephrosis in systemic lupus erythematosus (SLE). Sixty-one SLE patients with IPO and/or ureterohydronephrosis were analyzed retrospectively. A total of 183 cases were randomly selected as controls from 3840 SLE inpatients without IPO and ureterohydronephrosis during the same period. Patients were assigned to 1 of the 3 groups (SLE with IPO and ureterohydronephrosis, SLE with IPO, and SLE with ureterohydronephrosis). The clinical characteristics, treatments, and prognosis were compared between the 3 groups. There were 57 females and 4 males, with a mean age of 32.0 years. IPO was the initial manifestation of SLE in 49.1% of the cases, whereas ureterohydronephrosis in 32.5%. All patients were initially treated with a high-dose steroid. Thirty-one of these patients (50.8%) also received intravenous methylprednisolone pulse therapy. Two patients died of bowel perforation and lupus encephalopathy, and the other 59 patients (96.7%) achieved remission after treatment. The incidences of fever, glomerulonephritis, nervous system involvement, serositis, erythrocyte sedimentation rate elevation, hypoalbuminemia, hypocomplementemia, and anti-SSA antibody positivity were significantly higher in patients with IPO and/or ureterohydronephrosis than in the control group (without IPO and ureterohydronephrosis). Also, patients with IPO and/or ureterohydronephrosis had higher SLE Disease Activity Index scores than control patients. Compared with SLE patients with IPO, the patients with IPO and ureterohydronephrosis had a significantly higher incidence of gallbladder wall thickening, biliary tract dilatation, and serositis, whereas the patients with ureterohydronephrosis had less mucocutaneous involvement and serositis. Eight of the 47 IPO patients who initially responded well to immunotherapy relapsed; however, all responded well to retreatment with adequate immunotherapy. Of these 8 patients, 4 relapsed following poor compliance and self-discontinuation of steroid or immunosuppressant therapy. The rate of poor compliance with immunotherapy and the number of organ systems involved in patients in the recurrent IPO group were significantly higher than those in the nonrecurrent IPO group. IPO and ureterohydronephrosis are severe complications of SLE. As patients usually respond readily to early optimal steroid treatment, early diagnosis and timely initiation of glucocorticoid are important to relieve symptoms, prevent complications, and improve prognosis.

Background: Diagnosis of tuberculous serositis remains a challenge. The aim of this study was to evaluate the diagnostic efficiency of T-SPOT.TB on serous effusion mononuclear cells (SEMC) for diagnosing tuberculous serositis in a high TB burden area., Methods: The present prospective study enrolled patients with suspected tuberculous serositis in a tertiary referral hospital in Beijing, China, to investigate the diagnostic sensitivity, specificity, predictive value (PV), and likelihood ratio(LR) of these tests. Clinical assessment, T-SPOT.TB on SEMC, and T-SPOT.TB on PBMC were performed. Test results were compared with the final confirmed diagnosis., Results: Of the 187 participants, 74 (39.6%) were microbiologically or clinically diagnosed as tuberculous serositis and 93(49.7%) were ruled out. The remaining 20 (10.7%) patients were clinically indeterminate and excluded from the final analysis. Compared to that on PBMC, T-SPOT.TB on SEMC showed higher sensitivity (91.9%vs73.0%, P = 0.002), specificity (87.1%vs.73.1%, P = 0.017), PPV (85.0%vs.68.4%, P = 0.013), NPV (93.1%vs.77.3%, P = 0.003), LR+ (7.12vs.2.72) and LR- (0.09vs.0.37), respectively. The frequencies of spot forming cells (SFCs) for T-SPOT.TB on SEMC were 636 per million SEMC (IQR, 143-3443) in patients with tuberculous serositis, which were 4.6-fold (IQR, 1.3-14.3) higher than those of PBMC. By ROC curve analysis, a cut-off value of 56 SFCs per million SEMC for T-SPOT.TB on SEMC showed a sensitivity of 90.5% and specificity of 89.2% for the diagnosis of tuberculous serositis., Conclusions: T-SPOT.TB on SEMC could be an accurate diagnostic method for tuberculous serositis in TB endemic settings. And 56 SFCs per million SEMC might be the optimal cut-off value to diagnose tuberculous serositis.

Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease affecting millions of people worldwide. It can affect any organ systems of the body. However, all systems may not be involved initially rather than they may be affected gradually, sometimes over years. Diagnosis depends on characteristic clinical features and laboratory test results. Some features such as skin rash, joint symptoms and oral ulcers are common in SLE. But initial presentation of many patients is unusual because either they do not have these common features of the disease or the presentation mimics other illnesses. As a result, delayed diagnosis and misdiagnosis are common. Therefore, high index of initial suspicion of SLE is critical. In clinical practice, SLE should be suspected in any patient presenting with an unexplained disease process involving two or more organ systems. To make a diagnosis in an unusual presentation, thorough clinical evaluation with details history of both present and past illnesses as well as laboratory tests for SLE should be performed. Usually primary-care physicians first evaluate SLE patients; but there is no single article, where all the information on when to suspect SLE in an unusual presentation, is available in an integrated form. In this article, a list of conditions, when SLE should be suspected in an unusual presentation, has been given and some relatively common areas with diagnostic challenges of SLE have been briefly described. To prepare this manuscript, most articles have been identified through 'Pubmed' search using keywords-atypical/ unusual presentation of SLE, case reports on SLE, gastrointestinal manifestations of SLE, neuropsychiatric SLE, diagnostic challenges with SLE, etc. Selected most articles are from currently medline-indexed journals.

We herein report a case of Hashimoto's thyroiditis (HT) with sequential autoimmune hepatitis (AIH), chorea and polyserositis. The patient was a 24-year-old man who underwent subtotal thyroidectomy due to compression symptoms caused by goiter and was diagnosed with HT postoperatively based on pathological examinations two years previously. He had exhibited liver dysfunction and intermittent chorea since 2008. His liver function and polyserositis improved remarkably following the administration of ursodeoxycholic acid (UDCA) and methylprednisolone. This is a very rare case that can be classified as autoimmune polyglandular syndrome (APS) type 3. Early and adequate UDCA and glucocorticoid treatment may lead to a favorable prognosis.

Background and Aims: Inflammatory bowel disease is associated with increased risk of cholelithiasis. However, the histologic patterns in gallbladders have not been extensively studied. This study is designed to characterize the histopathologic features of cholecystectomy specimens in inflammatory bowel disease patients, compared to a control group., Methods: Cholecystectomy specimens in 78 Crohn's disease patients and 50 ulcerative colitis patients were reviewed. These were compared with 93 cholecystomies from noninflammatory bowel disease patients of approximate age and sex. The pattern and extent of inflammation was noted., Results: Marked chronic cholecystitis was present in 12% of ulcerative colitis patients (P<0.05) and 10.3% of Crohn's disease patients (P>0.05), compared to 4.3% of the noninflammatory bowel disease control group. Eight percent of ulcerative colitis patients (P<0.05) and 2.6% of Crohn's disease patients (P>0.05) had acute serositis, compared to 0% of the noninflammatory bowel disease control. The third inflammatory pattern, nodular lymphoid aggregates, was significantly increased in Crohn's disease patients after adjusting for the effect of cholelithiasis. Nodular lymphoid aggregates were found in 21.2% of Crohn's disease patients and 9.7% of ulcerative colitis patients without cholelithiasis, compared to 5% of noninflammatory bowel disease controls without cholelithiasis, a statistically significant difference between the Crohn's disease and control groups (P<0.05)., Conclusions: Inflammatory bowel disease patients show similar inflammatory patterns in cholecystectomy specimens compared to the general population. However, two inflammatory patterns that occur more often in ulcerative colitis patients are marked chronic cholecystitis and acute serositis, while nodular lymphoid aggregates are more common in Crohn's disease patients., (Copyright © 2012 European Crohn's and Colitis Organisation. Published by Elsevier B.V. All rights reserved.)

Evidence points to an association of prolactin to autoimmune diseases. We examined the correlation between hyperprolactinemia and disease manifestations and activity in a large patient cohort. Age- and sex-adjusted prolactin concentration was assessed in 256 serum samples from lupus patients utilizing the LIASON prolactin automated immunoassay method (DiaSorin S.p.A, Saluggia, Italy). Disease activity was defined as present if European Consensus Lupus Activity Measurement (ECLAM) > 2 or Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) > 4. Lupus manifestations were grouped by organ involvement, laboratory data, and prescribed medications. Hyperprolactinemia was presented in 46/256 (18%) of the cohort. Hyperprolactinemic patients had significantly more serositis (40% vs. 32.4%, p = 0.03) specifically, pleuritis (33% vs. 17%, p = 0.02), pericarditis (30% vs. 12%, p = 0.002), and peritonitis (15% vs. 0.8%, p = 0.003). Hyperprolactinemic subjects exhibited significantly more anemia (42% vs. 26%, p = 0.02) and marginally more proteinuria (65.5% vs. 46%, p = 0.06). Elevated levels of prolactin were not significantly associated with other clinical manifestations, serology, or therapy. Disease activity scores were not associated with hyperprolactinemia. Hyperprolactinemia in lupus patients is associated with all types of serositis and anemia but not with other clinical, serological therapeutic measures or with disease activity. These results suggest that dopamine agonists may be an optional therapy for lupus patients with hyperprolactinemia.

Objectives: Some studies have reported that serum CA125 level is elevated in SLE patients, and elevated CA125 level may be associated with kidney involvement and disease activity in SLE. However, none of the previous studies controlled confounding variables and the results remained controversial. The present study was aimed to investigate whether elevated serum CA125 level is independently associated with clinical and laboratory features of SLE by excluding various confounders in Chinese patients., Methods: A total of 156 SLE patients, consisting of 139 women and 17 men, were included in the study. Some clinical and laboratory characteristics of the patients were obtained by medical record review. Serum CA125 levels were measured by electrochemiluminescence immunoassays., Results: Compared with patients with normal CA125, those with elevated CA125 had significantly more serositis (37.5% vs. 1.9%, p<0.001) and lung involvement (37.5% vs. 12%, p=<0.001), higher SLEDAI scores (p<0.007). Furthermore, disease duration was significantly longer in those with elevated CA125. Univariate logistic regression analysis showed that elevated serum CA125 level was closely associated with disease duration (OR, 95%CI:1.005, 1.001-1.010; p=0.014), serositis (OR, 95%CI: 32.258, 6.993-142.857; p<0.001), renal involvement (OR, 95%CI: 2.283, 1.114-4.673; p=0.024), lung involvement (OR, 95%CI: 4.386, 1.927-10.000; p<0.001) and SLEDAI scores (OR, 95%CI: 1.098, 1.027-1.174; p=0.006). After controlling for various confounding variables, serositis and disease duration were the only two clinical variables significantly associated with elevation of serum CA125 level. The best cut-off value for CA125 using the ROC curve was 38 kU/L (sensitivity 85%, specificity 75%) and the area under the ROC curve was 0.777 with 95%CI of 0.685-0.868 (p<0.001). Furthermore, the serum CA125 levels can fall into the normal range again with the improvement of serositis., Conclusions: Of various clinical and laboratory variables of SLE, only serositis is independently associated with serum CA125 elevation.

Shrinking lungs syndrome (SLS) is a rare entity associated with autoimmune diseases and its underlying pathogenesis is still unclear. We describe a series of seven consecutive cases of SLS in systemic lupus erythematosus, all of them with serositis and six (85.7%) with anti-Ro/SSA antibodies. Our findings reinforce the hypothesis that SLS may be, in some cases, a consequence of diaphragmatic restriction due to pleuritic pain, and we suggest anti-Ro/SSA as a marker of this subgroup of SLS.

Constrictive polyserositis (pleuritis, pericarditis) is a syndrome within the underlying disease (tuberculosis, periodic disease, rheumatoid arthritis, systemic lupus erythematosus, asbestos, silicosis, uremia, some genetic diseases), a complication due to chest surgery or radiation or drug therapy, is occasionally idiopathic (fibrosing mediastinitis). There are frequently great difficulties in making its nosological diagnosis. The paper describes a patient in whom the onset of disease was exudative pleurisy with the signs of constriction, arthralgias; pleural punctures provided serous exudates with 80% lymphocytes. A year later there was ascitis and shin and foot edemas, which concurrent with hepatomegaly and cholestasis was regarded as cryptogenic liver cirrhosis. The signs of constrictive pericarditis were further revealed. The disease was complicated by the development of pulmonary artery thromboembolism (PATE) (which required the use of warfarin) and hemorrhagic vasculitis. Therapy with metipred in combination with isoniazid yielded a slight effect. The diagnoses of tuberculosis, liver cirrhosis, and autoimmune hepatitis, systemic vasculitis were consecutively rejected; the diagnosis of rheumatoid polyarthritis with systemic manifestations was made, by taking into account persistent arthalgias with the minimum signs of arthritis, noticeably increased C-reactive protein, rheumatoid factor, and cyclic citrullinated peptide antibodies (CCPA); plasmapheresis, therapy with metipred and methotrexate, and subtotal pericardectomy were performed. Constrictive polyserositis concurrent with PATE, hemorrhagic vasculitis (probably, drug-induced one), and hepatic lesion has been first described in a CCPA-positive patient with rheumatoid arthritis in the presence of moderate true arthritis (during steroid therapy).

Tyrosine kinase inhibitors (TKIs) have dramatically changed the treatment of chronic myeloid leukemia (CML) and are increasingly used in other malignancies. Despite the apparent selectivity of these agents significant side effects can occur mainly due to off target kinase inhibition. Clinical consequences of serosal inflammation, including pleural and pericardial effusions, have emerged as a frequent adverse event associated with dasatinib while occurring much less frequently during imatinib and nilotinib therapy. The pathogenesis is uncertain but may involve inhibition of platelet derived growth factor or expansion of cytotoxic T and natural killer cells. The development of serosal inflammation with dasatinib poses a significant challenge to physicians, as it cannot be predicted, the time of onset is variable, and management frequently requires repeat invasive procedures.

Pelvic inflammatory disease (PID) is generally a disease of young, sexually active patients. However, there have been few reports of computed tomography (CT) and the histopathologic findings of periappendicitis with PID in children. We present a case of PID with periappendicitis in a 12-year-old girl. Her CT findings are described, and the histopathologic findings of periappendicitis are discussed.

The aims of the present study are to evaluate the difference of the levels of soluble Fas (sFas) antigen between patients with systemic lupus erythematosus (SLE) and healthy controls and to explore whether sFas has a role in either the disease activity or the organ damage in SLE. Serum levels of sFas were measured in 40 Chinese patients with SLE and 15 age-, gender-, and race-matched healthy controls using double antibody ELISA. SLEDAI scores for disease activity were determined. Data of organ and tissue damage was obtained from clinical records. Serum sFas levels were significantly increased in both more active (mean=8043.8 pg/ml, P<0.001) and less active SLE patients (mean=4820.2 pg/ml, P<0.001) comparing to the healthy controls (mean=3253.4 pg/ml). There was also a significant difference in serum sFas levels between the more active SLE patients and less active SLE patients (P=0.04). But, the levels of sFas didn't correlate with SLEDAI. There was a significant difference in the serum sFas levels between patients with and without CNS disease (mean=9582.6, 6634.5 pg/ml; P=0.007). The same was true when patients with and without renal disease (mean=10972.7, 6520.1 pg/ml; P=0.019), as well as serositis (mean=10385.3, 6709.1 pg/ml; P=0.005) were analyzed. sFas is elevated in sera of SLE patients, especially in patients with active SLE. The elevated levels of sFas in the sera of patients with SLE may be closely associated with damage to the kidneys, central nervous system and serosa. Serum sFas may serve as a predictor of some organ and tissue damage in SLE.

Myelodysplastic syndrome (MDS) is a heterogenous group of stem cell disorders usually characterized by progressive refractory cytopenias, which could progress to acute myeloid leukemia. MDS may be associated with a wide spectrum of skin lesions, including neoplastic cell infiltration, Sweet's syndrome, pyoderma gangrenosum, erythema elevatum diutinum, vasculitis, and panniculitis. However, erythema nodosum is rarely associated with MDS. Unusual rheumatologic manifestations in patients with MDS also have been reported, which range from asymptomatic serological abnormalities to classic connective tissue disorders such as Sjögren's syndrome, relapsing polychondritis, systemic lupus erythematosus, rheumatoid arthritis and mixed connective tissue disease. However, concurrent erythema nodosum and serositis has rarely been reported. We describe a case of MDS with erythema nodosum and immune-mediated pericardial effusion in a 34-year-old woman.

A case report describes an adolescent girl with a treatment-resistant bipolar disorder, who developed pericarditis and polyserositis while being treated with clozapine. The sparse literature about this rare, severe side effect of clozapine is discussed. Clinical recommendations with regard to monitoring are given. If myocarditis/polyserositis occurs, clozapine has to be discontinued immediately.

The SLICC group believed that the definition of pleuritis should be expanded to include new pleural thickening and pericarditis to include characteristic history. Furthermore, SLICC suggested the addition of abdominal serositis to the current serositis criterion, manifested as either diffuse abdominal pain, with rebound or guarding, and/or ascites or bowel wall edema in the absence of other causes. Abdominal serositis can be secondary to either acute or chronic lupus peritonitis, with the former usually presenting as acute, generalized pain, and the latter as painless ascites.

Nasal-type T/natural killer (NK)-cell lymphoma, a distinct clinicopathological entity is highly associated with Epstein-Barr virus which shows an aggressive course. We present a CD56+ nasal-type T/(NK)-cell lymphoma case with systemic manifestations of rhabdomyolysis and polyserositis who died of multiorgan failure shortly after his admission to hospital in spite of adequate chemotherapy and supportive care.

A retrospective analysis of the case records of 494 systemic lupus erythematosus (SLE) patients under follow-up at University Hospital, Kuala Lumpur during 1976-1990 was performed. Overall mortality was 20.2% (100 patients). The causes of death were infection (30%), renal (15%), respiratory (14%), neurological (5%), cardiovascular (7%), other causes (2%) and unknown (27%). Active SLE was a contributing factor in 19% of the deaths. The patients who died had significantly more renal disease, neurological disease, serositis or thrombocytopenia by the end of the first year of disease compared to the survivors. As in other series, infection and active SLE remain important causes of death.

Gastrointestinal symptoms are common in patients with systemic lupus erythematosus (SLE) and can be due to primary gastrointestinal disorders, complications of therapy or SLE itself. In this case report, we describe three different presentations and causes of gastrointestinal complaints in patients with SLE. Diagnostic and management problems are discussed.

A 57-year-old woman consulted for a severe xanthelasma followed by a severe and complex systemic disease and died ten years later with xanthomas extended to the face and trunk. Her lipid and cholesterol levels were normal but HDL was below normal values. Diabetes as well as amyloidosis, paraproteinemia and Bence Jones protein in urine were not found. Three years after her initial consultation monocytosis appeared in most but not all of her hemograms. Soon after her spleen was palpable and became very large. She had three bone marrow biopsies showing progressive white cell hypercellularity that was considered as possibly reactive. During her long illness she had recurrent conjunctivitis and systemic complications such as arthritis, pleural and pericardial effusions, vasculitis, sudden deafness with Ménière-like vertigo, erythema nodosum and myositis that responded, at least partially, to steroid treatments though she remained corticoid dependent in order to control her more severe symptoms. Those complications were considered to be probably due to vasculitis or immune phenomena. After a long follow up it was clear that her disease was better classified as diffuse plane xanthoma (DPX) related to a myeloproliferative syndrome and vasculitis. Her final hospitalization was due to a severe esophageal moniliasis and she died a few days later. An autopsy was performed and a chronic myelomonocytic leukemia was the final diagnosis. The ultimate cause of death was a perforated acute duodenal ulcer with peritonitis. Possible relations between DPX, immunologic reactions and myelomonocytic leukemia are discussed.

Objective: To investigate the prevalence of juvenile chronic arthritis (JCA), familial Mediterranean fever (FMF), and Behçet's disease in Turkish children through a field survey., Methods: The field survey was based on cluster centering with 2 level strata. A total of 46,813 children were screened. For the diagnosis of chronic arthritis and Behçet's previously suggested criteria were used. We have developed criteria for the diagnosis of probable FMF. Children previously diagnosed to have these diseases were also defined and included., Results: JCA was found in 6.4/10,000. 2.8/10,000 children were previously diagnosed as FMF (minimum phenotype frequency). Together with the probable diagnosis of FMF, the prevalence increased to 9.3/10,000. The findings were also compared with those of our center. None of the 46,813 children had Behçet's disease., Conclusion: The prevalence of chronic arthritis is similar to the other childhood populations reported. However, FMF has a very high prevalence.

Background: The nosologic position of subacute cutaneous lupus erythematosus (SCLE) is controversial. More than four American Rheumatism Association (ARA) criteria for systemic lupus erythematosus (SLE) are found in a proportion of patients diagnosed as having SCLE; thus such cases could be classified as SLE., Objective: Our purpose was to determine whether ARA criteria for SLE are helpful in differentiating SCLE from SLE and whether cutaneous and visceral changes, immunologic findings, and photosensitivity provide a basis for diagnosis of SCLE., Methods: A cohort of 143 patients (79 with SCLE, 58 with SLE, and six with overlapping features of SCLE and SLE) was studied clinically, histologically, and immunologically as well as by phototesting. The patients were observed for up to 10 years, and the course of the disease and response to therapy were evaluated in each group., Results: SCLE differed from SLE by cutaneous changes, significantly less frequent kidney involvement, serositis and arthritis, and the rare presence of double-stranded DNA, U1RNP, and Sm antibodies characteristic of SLE. Ro(SS-A) and La(SS-B) antibodies were detected with similar frequency, and photosensitivity was not related to the presence of Ro antibody. In contrast, photoreproduction (appearance of LE lesion in irradiated area) was significantly more frequent in patients with SCLE. The course of SCLE in older patients was less severe than in younger patients, and aggressive therapy was usually not required., Conclusion: Patients with SCLE (although the majority fulfill more than four ARA criteria for SLE) show significant differences from those with SLE in terms of cutaneous and visceral involvement, immunologic findings, photosensitivity, course of the disease, and the requirement for therapy. Therefore SCLE should be recognized as a separate subset. However, cases of overlapping SLE and SCLE suggest a close relation.

A severe case of juvenile paracoccidioidomycosis (PCM), manifested as cholestatic jaundice, lymph node enlargement and an unusual form of polyserositis, associated with portal hypertension secondary to schistosomiasis, as well as bacteremias caused by E. coli and S. aureus and post-transfusional hepatitis C is reported. Temporary unresponsiveness of in vivo and in vitro cellular immune responses to P. brasiliensis were registered. The authors discuss the possible interference of either agent in the host immune response, thus explaining the severity of PCM in the present case.

We report about a 25-year-old female patient, who met all the criteria of adult-onset Still's disease except for the typical nonpruric rash. The disease exacerbated during pregnancy. Polyserositis was the predominant clinical manifestation. The histological examination of the bone marrow biopsy revealed changes similar to those seen in a myelodysplastic syndrome. The clinical condition improved after therapy with high-dose steroids and cyclophosphamide.

Fifteen (11 percent) of 140 patients with systemic lupus erythematosus (SLE) and five (31 percent) of 16 patients with polyarteritis nodosa (PA) developed disease-related signs and symptoms of an acute surgical abdomen. Peripheral vasculitis (p less than 0.025), nervous system involvement (p less than 0.05), ischemic necrosis of bone (p less than 0.05), thrombocytopenia (p less than 0.01), and circulating rheumatoid factor (p less than 0.01) are all significantly more prevalent in patients with SLE and abdominal involvement compared with those without an abdominal syndrome. Similarly, except for ischemic necrosis of bone, these same features are more prevalent in patients with PA and acute abdomens. Eleven (73 percent) of 15 patients with SLE had exploratory laparotomy for acute abdominal events. Nine were found to have evidence of intra-abdominal arteritis and only two had polyserositis without gross evidence of vasculitis. Eight (53 percent) of the 15 patients with SLE and acute abdomens died as a result of their abdominal crises. Six of the seven surviving patients represent those most recently seen and aggressively treated. All five patients with PA and acute abdomens required operative intervention and all had gross evidence of mesenteric arteritis with large ischemic segments resulting in infarction and perforation. All died in contrast to only two (18 percent) of the 11 PA patients without abdominal involvement.

Serious clinical sequelae of lupus serositis are uncommon and rarely a cause of morbidity. We describe two patients, one with chronic adhesive pericarditis and one with extensive small-bowel adhesions due to lupus peritonitis. In both, delayed institution of adequate prednisone therapy may have played a contributing role.