Legionella Serositis: A Rare Presentation.

BACKGROUND: Legionella has a higher prevalence in India than in the world. Legionaries' disease most commonly involves the lungs but because of increased awareness, extrapulmonary manifestations are also being diagnosed more frequently. CASE DESCRIPTION: We present a case of a young female with acute onset of fever and chest pain. On initial investigation, an electrocardiogram (ECG) reported widespread pulse rate (PR) depression suggestive of pericarditis which was confirmed by ECG. High-resolution computed tomography (HRCT) thorax suggested mild bilateral pleural effusion with normal lung parenchyma. elevated erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) added to the diagnosis of serositis. Serological study for atypical organisms was remarkable for positive immunoglobulin M (IgM) for Legionella. She was treated with a high dose of steroids and azithromycin successfully. CONCLUSION: Isolated extrapulmonary presentation of legionaries disease is often overlooked and is common. So it should be always included in the diagnostic armamentarium as treatment is highly efficacious if started early.

Serositis and lymphopenia are common features of systemic lupus erythematosus following SARS-CoV-2 infection: A case report and literature review.

The coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can affect a number of human systems, including the respiratory, cardiovascular, neurological, gastrointestinal, and musculoskeletal systems. These symptoms persist long after the acute infection has healed and is called "long COVID". Interestingly, there have been a series of reports that SARS-CoV-2 infections trigger the development of various autoimmune diseases such as systemic lupus erythematosus (SLE), inflammatory arthritis, myositis, vasculitis. Here, we report a novel case of SLE characterized by persistent pleural effusion and lymphopenia following SARS-CoV-2 infection. This is the first case in the Western Pacific region to our knowledge. Furthermore, we reviewed 10 similar cases including our case. By looking at the characteristics of each case, we found that serositis and lymphopenia are common features of SLE following SARS-CoV-2 infection. Our finding suggests that patients with prolonged pleural effusion and/or lymphopenia after COVID-19 should be checked for autoantibodies.

Peritonitis as the first presentation of systemic lupus erythematous: a case report.

BACKGROUND: Systemic lupus erythematosus is an inflammatory disease affecting several organs. Serositis is one of the systemic lupus erythematosus presentations, but peritonitis is a relatively rare presentation. Particularly, it is extremely rare to observe peritonitis as the first presentation of systemic lupus erythematosus. CASE PRESENTATION: Here, we present a case of peritonitis without other symptoms of systemic lupus erythematosus, in a patient who was finally diagnosed with systemic lupus erythematosus. Our patient was a 27-year-old Persian/Caucasian male with fatigue, weakness, weight loss, abdominal distension, massive ascites, and normocytic hemolytic anemia. He did not mention any prior medical conditions and did not use any drugs. There were no signs of thyroid dysfunction, cardiac dysfunction, cancers, infectious diseases, hepatitis, kidney diseases, or other diseases. Low-gradient, high-protein ascites fluid, and positive antinuclear antibody and anti-double stranded DNA were in favor of systemic lupus erythematosus. Corticosteroid pulse therapy led to resolution of ascites, and the patient was discharged with prednisolone and hydroxychloroquine. CONCLUSION: Peritonitis is a rare presentation of systemic lupus erythematosus, particularly as the first presentation and in the absence of other signs and symptoms; however, systemic lupus erythematosus should be considered as one the differential diagnoses for peritonitis when other etiologies have been ruled out.

Post-Streptococcus mitis infection polyserositis.

We report a case of a 42-year-old man who presented with acute epigastric and retrosternal chest pain and exertional dyspnoea, and was subsequently diagnosed with polyserositis secondary to post-Streptococcal mitis infection. A CT scan showed a large pericardial effusion requiring pericardiocentesis, small bilateral pleural effusions and small amount of ascites. Several serological tests were done, which were all found to be normal. Pericardial and pleural fluid aspirates revealed an exudate. Culture of the pleural fluid yielded growth of S.  mitis and this was deemed the cause of the polyserositis, which is rare. The patient made a spontaneous recovery. He was started on colchicine by the cardiologists to help prevent pericardial fluid recurrence and this was continued for 3 months. A dental review confirmed the presence of dental caries, the possible source of infection. On follow-up, the patient remained well with no further relapses.

Klinefelter's syndrome with systemic lupus erythematosus and atrial fibrillation.

We describe a 65-year-old man who presented with arthralgia, reduced body hair and gynecomastia. He showed severe pancytopaenia. Laboratory examination revealed high follicle-stimulating hormone, low testosterone and oestradiol, elevated antinuclear antibodies, anti-dsDNA and ESR levels, as well as low complement levels. An electrocardiogram showed atrial fibrillation. Computed tomography and dual-energy x-ray absorptiometry showed pleural effusion and osteoporosis. Chromosome analysis revealed 47, XXY karyotype. The unifying diagnosis was therefore Klinefelter's syndrome (KS) with systemic lupus erythematosus (SLE), with manifestations of pancytopaenia, atrial fibrillation, serositis and osteoporosis. After immunosuppressive therapy, his physical condition and pancytopaenia improved. Sex hormones and gene escape from X chromosome inactivation may contribute to the pathogenesis of SLE. Clinicians should consider autoimmune processes when patients with KS present with pancytopaenia or additional features of a systemic autoimmune disorder.

A 60-Year-Old Woman With Acute Chest Pain and Bilateral Pleural and Pericardial Effusions.

CASE PRESENTATION: A 60-year-old woman presented with acute-onset, progressively worsening shortness of breath and pleuritic chest pain for 3 days. She also complained of a dry cough, but no fever or chills. There was no history of swelling of the feet; nor was there a history of nausea or diarrhea. She was a lifelong nonsmoker and had no history of recent travel or sick contacts. Her medical history included hypertension and ulcerative colitis. The ulcerative colitis was in remission and she had not been taking medications for this for over 7 years. Her home medications included alendronate, amlodipine, aspirin, atenolol, and vitamin D3 supplements. She had no allergies.

[A Case in Which Good Control of Carcinomatous Serositis Was Achieved Using Chemotherapy Including Albumin-Bound Paclitaxel(Nab-PTX)for Advanced-Stage Pulmonary Adenocarcinoma Exhibiting Carcinomatous Pleurisy and Carcinomatous Pericarditis].

The subject was a 63-year-old man. The patient was transported by ambulance to the hospital because of dyspnea caused by carcinomatous pleurisy and carcinomatous pericarditis, after which pericardial drainage was performed; however, Staphylococcus aureus bacteremia arose as a complication. Adequate control of carcinomatous serositis was achieved usingchemotherapy, includingalbumin -bound paclitaxel(nab-PTX), which is a nanoparticle formulation bindinghuman serum albumin and paclitaxel, in combination with 1 course of antibiotics. For cancerous serositis cases, platinum combination chemotherapy usingnab -PTX is believed to be 1 treatment option in which good disease control can be expected along with bevacizumab, whose efficacy has already been confirmed.

Multifocal osteolysis with chylous polyserositis and intrathoracic vein thrombosis: a diagnostic challenge for rheumatologists.

Vanishing bone disease with multisystemic involvement may mimic systemic autoimmune or autoinflammatory diseases. We present a 19-year-old man who was hospitalized due to chest pain following a progressive osteolysis of the bony thorax. The disease later expanded into the pleura, peritoneum and pericardium in a form of massive chylous polyserositis. The patient also developed thrombosis of multiple central veins, which in turn worsened the chylothorax by increasing the pressure in the thoracic duct. This is the first case of vanishing bone disease complicated by triple chylous effusions and central vein thrombosis.

Pulmonary manifestations in late versus early systemic lupus erythematosus: A systematic review and meta-analysis.

OBJECTIVES: Phenotypes differ between late- and early-onset systemic lupus erythematosus (SLE). Prior studies suggested that there may be more pulmonary disease among late-onset patients. Our objective was to perform a systematic review and meta-analysis to evaluate the differences in pulmonary manifestations in late- versus early-onset SLE. METHODS: We searched the literature using PubMed, CINAHL, Web of Science, Cochrane Library, and EMBASE. We excluded studies that did not include American College of Rheumatology SLE classification criteria, an early-onset SLE comparison group, or those that defined late-onset SLE as <50 years of age. We rated study quality using the Newcastle-Ottawa Quality Scale. We used Forest plots to compare odds ratios (95% confidence intervals) of pulmonary manifestations by age. Study heterogeneity was assessed using I2. RESULTS: Thirty-nine studies, representing 10,963 early-onset and 1656 late-onset patients with SLE, met eligibility criteria. The odds of developing several pulmonary manifestations were higher in the late-onset group. Interstitial lung disease (ILD) was nearly three times more common (OR = 2.56 (1.27, 5.16)). Pleuritis (OR = 1.53 (1.19, 1.96)) and serositis (OR = 1.31 (1.05, 1.65)) were also more common in the late-onset group. The mean Newcastle-Ottawa Quality Scale score for study quality was moderate (6.3 ± 0.7, scale 0-9). CONCLUSIONS: Pulmonary manifestations of SLE were more common in late-onset SLE patients compared to their younger peers, in particular ILD and serositis. Age-related changes of the immune system, tobacco exposure, race, and possible overlap with Sjögren's syndrome should be examined in future studies.

The peritoneum: healing, immunity, and diseases.

The peritoneum defines a confined microenvironment, which is stable under normal conditions, but is exposed to the damaging effect of infections, surgical injuries, and other neoplastic and non-neoplastic events. Its response to damage includes the recruitment, proliferation, and activation of a variety of haematopoietic and stromal cells. In physiological conditions, effective responses to injuries are organized; inflammatory triggers are eliminated; inflammation quickly abates; and the normal tissue architecture is restored. However, if inflammatory triggers are not cleared, fibrosis or scarring occurs and impaired tissue function ultimately leads to organ failure. Autoimmune serositis is characterized by the persistence of self-antigens and a relapsing clinical pattern. Peritoneal carcinomatosis and endometriosis are characterized by the persistence of cancer cells or ectopic endometrial cells in the peritoneal cavity. Some of the molecular signals orchestrating the recruitment of inflammatory cells in the peritoneum have been identified in the last few years. Alternative activation of peritoneal macrophages was shown to guide angiogenesis and fibrosis, and could represent a novel target for molecular intervention. This review summarizes current knowledge of the alterations to the immune response in the peritoneal environment, highlighting the ambiguous role played by persistently activated reparative macrophages in the pathogenesis of common human diseases. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

A 12-year retrospective review of bullous systemic lupus erythematosus in cutaneous and systemic lupus erythematosus patients.

Objective The aim of this study was to investigate the clinical features, laboratory findings, systemic manifestations, treatment and outcome of patients with bullous systemic lupus erythematosus in a tertiary care center in Thailand. Methods We performed a retrospective review from 2002 to 2014 of all patients who fulfilled the diagnostic criteria for bullous systemic lupus erythematosus to evaluate for the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence pattern, serological abnormalities, internal organ involvement, treatments and outcome. Results Among 5149 patients with cutaneous lupus erythematosus and/or systemic lupus erythematosus, 15 developed vesiculobullous lesions. Ten patients had validation of the diagnosis of bullous systemic lupus erythematosus, accounting for 0.19%. Bullous systemic lupus erythematosus occurred after the diagnosis of systemic lupus erythematosus in six patients with a median onset of 2.5 months (0-89). Four out of 10 patients developed bullous systemic lupus erythematosus simultaneously with systemic lupus erythematosus. Hematologic abnormalities and renal involvement were found in 100% and 90%, respectively. Polyarthritis (40%) and serositis (40%) were less frequently seen. Systemic corticosteroids, immunosuppressants, antimalarials and dapsone offered resolution of cutaneous lesions. Conclusion Bullous systemic lupus erythematosus is an uncommon presentation of systemic lupus erythematosus. Blistering can occur following or simultaneously with established systemic lupus erythematosus. We propose that clinicians should carefully search for systemic involvement, especially hematologic and renal impairment, in patients presenting with bullous systemic lupus erythematosus.

[Celiac disease in adult patients revealed by polyserositis: about a case]. / Maladie cœliaque de l'adulte révélée par une polysérite.

Celiac disease (CD) is an autoimmune disease affecting multiple organs. It often presents as gastrointestinal manifestations associated with malabsorption. However, serosa involvement uncommonly reveals this enteropathy, making the diagnosis difficult. We here report the case of JA, aged 63 years, admitted to hospital to detect the cause of malabsorption syndrome associated with polyserositis signs including pleurisy, pericarditis, ascites and hydrocephalus. The diagnosis of CD was based on endoscopic signs without serology tests. Patient's evolution was partially favorable, due to lack of compliance with a gluten-free diet. Our study reports the first case of CD revealed by polyserositis. CD should be suspected in patients with malabsorption syndrome, in the absence of evocative signs.

A case of drug-induced lupus erythematosus secondary to trimethoprim/sulfamethoxazole presenting with pleural effusions and pericardial tamponade.

We report a case of drug-induced lupus erythematosus (DILE) secondary to trimethoprim/sulfamethoxazole (TMP/SMX) in a patient with underlying inflammatory bowel disease (IBD). The initial presentation was with febrile pleural and pericardial effusions followed by cardiac tamponade. The patient was treated with a short course of corticosteroids with complete resolution of symptoms. To our knowledge this is the first reported case of TMP/SMX-induced DILE presenting with life-threatening serositis. When confronted with sterile exudative effusions, clinicians should strongly consider non-infectious etiologies.

Life-threatening Medical Complications Due to Ovarian Hyperstimulation Syndrome: A Hidden Etiology.

Ovarian hyperstimulation syndrome is usually an iatrogenic complication in women taking ovulation induction medications during assisted reproduction. We hereby report the case of a 25 years old female who presented with hypertension, polyserositis with tense ascites and large cystic ovaries. She developed sigmoid and transverse sinus thrombosis. She had undergone a clandestine ovulation induction therapy as a commercial ovum donor. She fitted in severe category of ovarian hyperstimulation syndrome.

The prevalence and risk factors for serositis in patients with systemic lupus erythematosus: a cross-sectional study.

This study aims to estimate the prevalence of serositis and identify risk factors for serositis in a large cohort of systemic lupus erythematosus (SLE) patients. A cross-sectional study was conducted based on the medical records of patients hospitalized with SLE at the First Affiliated Hospital of Anhui Medical University and Anhui Provincial Hospital. Patients were diagnosed with serositis when they presented with symptoms and signs of pleuritis or/and pericarditis. We explored factors associated with the generation and quantity of serositis by using binary and ordinal logistic regression analysis. Among the 1668 lupus patients, 298 have serositis. Active lupus disease, fever (≥38 °C) and high D-dimer were all significantly associated with the generation and quantity of serositis. Male gender was independent significant risk factor for pleuritis but not for pericarditis, while low complement C4 and high erythrocyte sedimentation rate (ESR) were risk factors for pericarditis rather than for pleuritis. The possible prevalence of serositis in patients with SLE was 17.9%. The significant associations of active lupus disease, fever (≥38 °C) and high D-dimer with serositis suggest that higher disease activity and hypercoagulability may both contribute to the generation and development of serositis in SLE. The risk factors for pleuritis and pericarditis in SLE are similar but not identical.

Both prolonged remission and Lupus Low Disease Activity State are associated with reduced damage accrual in systemic lupus erythematosus.

OBJECTIVES: To identify predictors of organ damage and specifically the relationship between prolonged disease remission or low disease activity and damage accrual in a longitudinal cohort of SLE patients. METHODS: Data were prospectively assessed including the occurrence of minor/major flares. Once a year remission and Lupus Low Disease Activity State (LLDAS) were determined retrospectively. A prediction model for damage accrual during follow-up was constructed with backward logistic regression analyses. Secondly, odds ratios (ORs) for damage accrual (SLICC damage index increase of ⩾ 1 during follow-up) were calculated for patients with or without prolonged remission during 5 years, and with or without LLDAS in ⩾ 50% of observations. RESULTS: Data from 183 patients with a median follow-up duration of 5.0 years were analysed. The most significant predictors for damage accrual were: occurrence of ⩾ 1 major flare, mean daily prednisone dose during follow-up and nephrological manifestations at baseline. Prolonged remission was present in 32.5% (38/117) and LLDAS in ⩾ 50% of observations in 64.5% (118/183) of patients. Both the presence of prolonged remission during 5 years and LLDAS in ⩾ 50% of observations were associated with a reduced risk of damage accrual (OR = 0.20, 95% CI: 0.07, 0.53, P = 0.001 and OR = 0.52, 95% CI: 0.28, 0.99, P = 0.046, respectively). CONCLUSION: This cohort study shows that prolonged remission and LLDAS were associated with an improved outcome, as determined by yearly assessments. In order to improve the outcome in SLE patients, future studies should investigate whether these targets can be reached actively with therapeutic strategies.