Chronic Myelomonocytic Leukemia Presenting With Polyserositis and Seropositivity for Rheumatoid Arthritis.

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.

A rare combination: chylous polyserositis and autoimmune myelofibrosis as a presentation of systemic lupus erythematosus.

Chylous polyserositis and autoimmune myelofibrosis occurring concomitantly inn a case of SLE are a rare phenomenon. We here report a case of a 38-year-old woman who was admitted with a history of cough and shortness of breath for 1½ months along with fever and abdominal distension for 1 month. She also had arthralgias, weight loss and pancytopenia. She was diagnosed as a case of SLE with Chylous polyserositis and autoimmune myelofibrosis. She was started on steroids and immunosuppressive therapy, to which she responded. To summarize, this is the first case report where chylous polyserositis and pancytopenia due to autoimmune myelofibrosis occurred which was responsive to steroids and immunosuppressive therapy.

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.

Anti-SmD1 antibodies are associated with renal disorder, seizures, and pulmonary arterial hypertension in Chinese patients with active SLE.

Detection of autoantibodies in systemic lupus erythematosus (SLE) plays an important role in timely diagnosis and earlier treatment of SLE. In this study, we used a SmD1 polypeptide-based ELISA to determine anti-SmD1 antibody in 269 SLE, including100 naïve (had not been treated with steroids or immunosuppressants at study inception) SLE patients and 169 non-naive SLE patients; 233 controls with other rheumatic diseases (RDC) (70 RA, 40 AS, 73SSc, and 50 SS), and 110 healthy controls (HC) group. The positive rate of anti-SmD1 among all SLE patients was 60.97%, higher than that in the RDC group (13.30%, P = 0.000) or the HC group (9.09%, P = 0.000). The positive rate of anti-SmD1 in non-naive SLE patients was higher than that for anti-dsDNA antibodies (44.97%, P = 0.03). Positivity for anti-SmD1 only was found in 14.00% of naive SLE patients and 16.00% of non-naive SLE patients. In naive SLE patients, the serum concentration of anti-SmD1 was lower after treatment than before treatment (P = 0.039). Active SLE patients positive for anti-SmD1 were more likely to have malar rash, rash, nonscarring alopecia, PAH and hypocomplementemia. High positivity for anti-SmD1 only in patients with SLE indicated the importance and necessity of detection of anti-SmD1 in patients with SLE.

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.

Chronic GvHD-associated serositis and pericarditis.

Serositis is a rare manifestation of chronic GvHD (cGvHD). No risk factors or laboratory changes associated with this syndrome have been recognized to date, and outcomes have not been described in a large series. We searched our institutional database for patients undergoing allogeneic hematopoietic cell transplant identified as having serositis or pericarditis. Laboratory studies from prior to diagnosis, at diagnosis and post diagnosis of serositis, as well as outcomes from invasive procedures were included. Twenty patients met criteria for cGvHD-associated serositis, and all but three patients had a prior diagnosis of cGvHD. Fifteen were male, and the complication occurred in the setting of immunosuppressant taper in 12 cases. Ten patients required invasive interventions, including pericardial window or stripping. A significant increase in blood monocytes and decrease in serum albumin were identified at diagnosis compared with pre-diagnosis. Out of 20 patients, 17 were treated with steroids, with 12 demonstrating a complete response. These data suggest that cGvHD-associated serositis occurs mainly in the setting of treated as opposed to de novo cGvHD and biomarkers associated with the syndrome include a decrease in albumin and an increase in absolute monocyte count. Outcome data from larger series are required to better understand the optimal management of this rare complication.

Xanthogranulomatous inflammation of the perimetrium with infiltration into the uterine myometrium in a postmenopausal woman: a case report.

BACKGROUND: Xanthogranulomatous inflammation is an uncommon form of chronic inflammation that is destructive to the normal tissue of affected organs. Although xanthogranulomatous endometritis and xanthogranulomatous salpingitis of the female genital tract has been described previously, to the best of our knowledge, this is the first report of xanthogranulomatous inflammation with infiltration into the uterine myometrium from the perimetrium without endometritis. CASE PRESENTATION: A 68-year-old Japanese woman with intermittent lower abdominal pain and low-grade fever who was initially treated with antibiotics underwent hysterectomy due to abscess formation in the posterior wall of the myometrium and perimetrium (the outer serosal layer of the uterus). Histopathological findings revealed that the abscess was caused by xanthogranulomatous inflammation with the granulation tissue and chronic inflammatory cells that consisted of focal and sheets of foam cells. The inflammation destroyed the perimetrial elastic lamina, and the myometrium was deeply infiltrated by the xanthoma cells. Neither endometritis nor salpingitis was coexistent with the xanthogranulomatous inflammation. CONCLUSION: The patient was diagnosed as xanthogranulomatous inflammation, most likely arising from the perimetrium. Our findings suggest that the perimetrium, as well as the endometrium and adnexae, is one of the origins of xanthogranulomatous inflammation in female genital tract.

Hashimoto's thyroiditis concomitant with sequential autoimmune hepatitis, chorea and polyserositis: a new entity of autoimmune polyendocrine syndrome?

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.

Tuberculosis mesenteric adenopathy and polyserositis.

A 41-year-old female patient was admitted into Surgery Clinic accusing abdominal pain, diarrhea, fever and chills. Based on clinical, biological and imaging data, it was established a diagnosis of pelviperitonitis and it was initiated an antibiotic and anti-inflammatory treatment. As fever and abdominal pain continued, it was decided to go on with surgery that revealed suppurated and perforated mesenteric adenopathy. Pus was sampled for bacteriological exam and also biopsy was performed for pathological exam. The result of pathological exam was suggestive for a specific granulomatous lesion (TB lesion). It was established diagnosis of TB mesenteric adenopathy and it was initiated specific anti-TB treatment according to WHO guidelines. After three, respectively five months of treatment, patient developed a right laterocervical adenopathy that fistulized in both cases, despite the correct treatment administered. No resistant TB strain and no atypical mycobacteria was discovered.

Familial Mediterranean fever presenting with pulmonary embolism.

Familial Mediterranean fever (FMF) is the autoinflammatory disease and hereditary periodic fever syndrome that most commonly affects people of Eastern Mediterranean origin. It is characterized by recurrent self-limited attacks of fever and serositis, with an increase in acute-phase reactant markers, and is transmitted in an autosomal recessive pattern. Inflammation shifts the hemostatic mechanisms favoring thrombosis. There are few reports of an increased risk of hypercoagulability in patients with FMF in the absence of amyloidosis and nephrotic syndrome. In this case report, we describe a 43-year-old Turkish patient who presented with right-sided pleuritic chest pain and pulmonary embolism. The patient described having prior similar attacks of serositis, but had never been diagnosed with FMF. Further workup revealed an increase in acute phase reactants, negative hypercoagulability studies and heterozygosity for the M694V mutation in the pyrin (MEFV) gene. We identified untreated FMF and chronic inflammation as his only risk factor for pulmonary embolism. With this case report, we support recent studies that have demonstrated that inflammation may lead to prothrombotic states in patients with FMF.

[Severe disseminated constrictive polyserositis in a patient with rheumatoid arthritis].

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).

Serosal inflammation (pleural and pericardial effusions) related to tyrosine kinase inhibitors.

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.

Young woman with polyserositis, ovarian cystic mass and increased level of CA-125. Case report of peritoneal and pleural tuberculosis.

A 21-year-old woman was addressed to our department for progressive abdominal swelling, fatigue and fever. The clinical examination, the ultrasound examination and the computed tomography showed the presence of polyserositis (ascites and pleural effusion) and revealed a cystic mass at the level of right ovary. The laboratory work-up showed an increased level of CA-125, suggesting a malignancy. The thoracoscopy with visualization of the pleura revealed disseminated small white spots. The laparoscopic exploration of the pelvis and of the peritoneum also showed the same disseminated lesions and a cystic-like mass at the level of the right ovary which was excised and diagnosed as a benign cyst. At the analysis of the frozen and paraffin sections, the diagnostic of pleural and peritoneal tuberculosis was made and the specific quadruple treatment was started with a good evolution at two months and with the normalization of the CA-125 level. This case report underlines the importance of tuberculosis in the differential diagnosis of patients with polyserositis and increased levels of CA-125.

Pelvic inflammatory disease with periappendicitis in a pediatric patient.

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.

Lupus erythematosus (LE) cells in ascites: initial diagnosis of systemic lupus erythematosus by cytological examination: a case report.

Systemic lupus erythematosus (SLE) is an autoimmune disease, involving multiple organs. Diverse manifestations may obscure the diagnosis and confuse our thinking process, especially when few clues are present at the beginning. Serositis is one of the various presentations, and the presence of lupus erythematosus (LE) cell in body fluid may be a hint for the final diagnosis of SLE. Herein, we present a young female patient diagnosed of SLE with initial presentation of lupus peritonitis. Finding of LE cell in ascites prompted us for immunologic survey. Diagnosis of SLE was confirmed with high titer of anti-nuclear antibody and antibody to double-stranded DNA. Cytologic examination of body fluid is mainly useful in detecting malignant cells, but high specificity of this marker aids in early diagnosis of SLE.

Computed tomographic features of appendiceal serositis in pelvic inflammatory disease: comparison with pathological findings.

OBJECTIVE: The purpose of this study was to describe the computed tomographic (CT) features of the appendiceal serositis in women with pelvic inflammatory disease and to compare these with the pathological findings. METHODS: Appendiceal serositis was pathologically evaluated in patients with pelvic inflammatory disease who underwent surgery within 3 days of computed tomography. On retrospective review of CT findings, each appendix was evaluated for the following characteristics: location relative to the cecum, maximal diameter, morphology of wall thickening, contrast enhancement, and presence of appendicolith and cecal wall thickening. The presence of fatty infiltration of the periappendiceal fat, mesentery, and omentum was evaluated. The presence of pelvic abscess or ascites, lymph nodes, and paralytic ileus was noted. RESULTS: On pathological review, 10 patients were shown to have appendiceal serositis: mild serositis in 3 patients, moderate in 4, and severe in 3. The maximal appendiceal diameter ranged from 5.4 to 8.9 mm (mean diameter, 7.1 +/- 0.9 mm). Diffuse wall thickening with collapsed lumen was detected in 6 patients. Focal wall thickening with intraluminal gas bubbles or an air-fluid level was detected in 4 cases. Peripheral rim enhancement of the appendix was detected in 3 patients with focal wall-thickened appendix. There was no association between the feature of appendiceal wall thickening and the pathological severity of serositis. Mesenteric fatty infiltration was detected in 5 patients and omental fatty infiltration in 3 patients. Fatty infiltration of the mesentery and omentum was more commonly presented in patients with severe serositis. Pelvic abscesses, including pyosalpinx, were detected in 7 patients; a small amount of free fluid was seen in 8 patients. CONCLUSIONS: The CT findings of appendiceal serositis are diffuse or focal wall thickening without severe distension, common association with mesenteric fatty infiltration, and pelvic abscesses.

Diagnosis of active tuberculous serositis by antigen-specific interferon-gamma response of cavity fluid cells.

BACKGROUND: To develop a more accurate methodology for diagnosing active tuberculous pleurisy, as well as peritonitis and pericardits of tuberculous origin, we established an antigen-specific interferon gamma (IFN-gamma)-based assay that uses cavity fluid specimens. METHODS: Over a 19-month period, 155 consecutive, nonselected patients with any cavity effusion were evaluated. Study subjects were 28 patients with bacteriologically confirmed active tuberculous serositis and 47 patients with definitive nontuberculous etiology. Culture was performed for 18 h with fluid mononuclear cells in the supernatant of the effusion together with saline or Mycobacterium tuberculosis-specific antigenic peptides, early secretory antigenic target 6 and culture filtrate protein 10. IFN-gamma concentrations in the culture supernatants were measured. RESULTS: In patients with active tuberculous serositis, antigen-specific IFN-gamma responses of cavity fluid samples were significantly higher than those of nontuberculous effusion samples. Area under the receiver operating characteristic (AUROC) curve was significantly greater for cavity fluid IFN-gamma response (AUROC curve, 0.996) than for cavity fluid adenosine deaminase and whole-blood IFN-gamma responses (AUROC curve, 0.882 and 0.719, respectively; P = .037 and P < .001, respectively). Although the AUROC curve was greater for cavity fluid IFN-gamma response than for background cavity fluid IFN-gamma level (AUROC curve, 0.975), the AUROC curves were not statistically significantly different (P = .74). However, multivariate logistic regression analysis revealed that cavity fluid IFN-gamma responses were significantly associated with the diagnosis, even after adjustment for background IFN-gamma level (adjusted odds ratio, 1.21; 95% confidence interval, 1.03-1.42; P < .001). CONCLUSIONS: The cavity fluid IFN-gamma assay could be a method for accurately and promptly diagnosing active tuberculous serositis.