Lupus nephritis presenting with massive ascites and pleural effusion (pseudo-pseudo Meigs' syndrome).

The triad of ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in the absence of ovarian malignancy in systemic lupus erythematosus patients is specifically named pseudo-pseudo Meigs' syndrome (PPMS) or Tjalma syndrome. In this case we reported a 33 years female patient with pleural effusion lasting for 3 years and new onset progressive massive ascites and increased level of CA-125. After she was evaluated for an underlying benign and malign ovarian tumor or any other malignancies, serologic tests were requested with respect to progressive renal dysfunction, proteinuria, lymphopenia, anemia, and effusion. She was diagnosed with systemic lupus erythamatosus (SLE) and renal biopsy showed class-V lupus nephritis. Immunosuppressive treatment led to improvement in both SLE activity and components of PPMS, including massive ascites and pleural effusion and without the need of diuretics. Co-existence of unexplained CA-125 increase, pleural effusion, and ascites might be related to PPMS and detailed examination to exclude malignancy and early and effective treatment of SLE are the mainstay of management.

Successful treatment of a rheumatoid arthritis patient with severe synovial hypertrophy and impaired shoulder function with microwave ablation technique.

Rheumatoid arthritis (RA) is a chronic, inflammatory systemic disorder of synovial joints and results in polyarthritis, chronical degeneration, and finally deformities and ankylosis in severe cases. Synovitis and pannus formation are results of inflammatory changes and lead into restriction in joint movement. Shoulders are among the later affected and larger joints and formation of synovitis in early active stages and pannus in later stages might be concluded with frozen shoulder and severe impairment in functionality. These late-term changes cannot be controlled with systemic or local anti-inflammatory agents and synovectomy is chosen in some cases. However, the results are not satisfactory and recurrence is common. In this case report, we presented a case of RA with severe shoulder pain, restricted movement due to synovial hypertrophy, and pannus formation which are resistant to local and systemic interventions and not suitable for surgical or chemical synovectomy. Microwave ablation (MWA) was performed successfully without any complication and she well responded in terms of DAS-28, functional, and pain scores. Range of motion and funcitonal restriction were recovered. This case report describes the use and promising results of MWA in RA with severe synovial hypertrophy and pannus formation even in the absence of active arthritis and effusion. MWA is a safe and minimally invasive technique that can be easily performed in coordinance of rheumatologists and interventional radiologists in proper cases.

A case of Behçet syndrome presenting with acute coronary syndrome.

Cardiac involvement (CI) is rare in Behçet syndrome (BS), but the important point is that CI may be the first manifestation of the disease. The presence of CI worsens the prognosis of BS, so early diagnosis and early initiation of immunosuppressive treatment (IST) are vital. Coronary aneurysm may develop spontaneously in these patients, or any vascular intervention may cause aneurysm with a pathergy-like reaction. The risk of restenosis is high after percutaneous coronary intervention or coronary artery bypass surgery applied without IST. Therefore, it should be kept in mind that IST constitutes the main step of treatment. Herein, we present a young male diagnosed with BS after acute coronary syndrome caused by coronary artery aneurysms and thrombosis.

Behcet's Syndrome Resembling Takayasu's Arteritis with the Distribution of Arterial Involvement: A Case Report and Literature Review.

Behcet's syndrome is a variable vessel vasculitis characterized by recurrent oral and genital ulcers with concomitant skin, ocular, neurologic, gastrointestinal, and joint involvement. Herein, we present a patient who was diagnosed with Behcet's syndrome, which with magnetic resonance angiography showed occlusion of the right subclavian artery at the level of the thoracic outlet and reverse flow in the right vertebral artery consistent with subclavian steal syndrome. In addition, partial narrowing was noted in the left renal artery. The distribution of arterial involvement resembled Takayasu's arteritis, although the presence of mucocutaneous lesions, male gender, history of deep vein thrombosis, and HLA-B51 positivity favored a diagnosis of vasculo-Behçet's syndrome. We treated the patient with methylprednisolone and cyclophosphamide. After the regression of vascular inflammation with immunosuppressive therapy, stenting was performed in the left renal artery.

Isolated inferior thyroidal artery vasculitis: A rare cause of neck pain.

Vasculitis is the inflammatory changes in vessels of any size that usually have a systemic involvement with a quite variable clinical presentation affecting various organs. Although systemic presentation is more common, in some cases localized inflammation of vasculature of a single organ or limited branches of aorta are reported. Here we present, an isolated vasculitis of bilateral inferior thyroidal arteries in a female patient aged 49 years, who presented with neck pain and was diagnosed with ultrasonography and computed tomographic angiography. The clinical and imaging findings were managed successfully with glucocorticoid induction and addition of methotrexate to the treatment. Localized forms of vasculitis are rarer and the limited size of the affected area makes diagnostic investigations and management more complicated. Non-invasive imaging modalities rather than conventional angiography provide useful information in a safer and easier way. Isolated vasculitis of thyroidal arteries is an extremely uncommon site and should be excluded in case of unexplained neck pain, even in the presence of normal laboratory examinations, probably because of the size of the involved vessels.

VEXAS syndrome with severe multisystem involvement: Rapid recovery after splenectomy.

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined disorder in which treatment is still unclear. Herein, a patient with VEXAS syndrome who had atypical findings and an interesting treatment course is presented as a case report. He had fatigue, recurrent fever, pulmonary infiltrates, proteinuria, anemia, leucopenia, transient skin rush and increased acute phase reactants. The patient, who could not tolerate corticosteroid tapering, recovered rapidly after diagnostic splenectomy and the pathological examination of the spleen revealed significant findings.

Visceral leishmaniasis in a patient with systemic lupus erythematosus: Dilemma in diagnosis and management.

Patients with systemic lupus erythemasus (SLE) have an increased risk of bacterial, viral, fungal or parasitic infections, especially if they are receiving immunosuppressive therapy. Leishmaniasis is a group of diseases caused by intracellular flagellate protozoan parasites belonging to the genus Leishmania. We present a 48-year-old female patient, diagnosed with SLE many years ago, who presented with high fever and pancytopenia. We thought that the patient's hematologic findings were related to SLE hematologic involvement. However, we investigated other possible causes when there was no response to drugs for the treatment of SLE. A second bone marrow biopsy showed Leishmania amastigotes and the patient was diagnosed with leishmaniasis. The patient was treated with liposomal amphotericin-B (treatment completed at 40 days). She showed rapid clinical improvement and showed no signs of disease after 4 months.

A Case of Protracted Febrile Myalgia Syndrome with Atypical Course and Severe Asymmetric Loss of Muscle Strength.

Protracted febrile myalgia syndrome (PFMS) is a rare form of familial Mediterranean fever (FMF) characterised by prolonged myalgia. The duration of PFMS is much longer than a typical 2-5-day attack familial Mediterranean fever and lasts for 2-6 weeks until they treated with corticosteroids. Colchicine is not effective for control of PFMS's attacks. The attacks typically resolve with corticosteroid and/or IL-1 receptor blockers. Herein, we present a young adult without typical familial Mediterranean fever clinical features but presenting with atypical course and severe asymmetric muscle strength loss. Thigh magnetic resonance imaging confirmed inflammation and oedema and muscle biopsy showed no pathological findings. Electromyography revealed myopathic findings during attack-period, despite normal results in attack-free study. The patient was treated successfully with anakinra and remarkable rapid recovery in both muscular findings and acute phase reactants were observed. PFMS should be considered even in the absence of apparent FMF attack pattern and in the presence of unexpected severe muscle weakness, especially in areas endemic for FMF and long-lasting myalgia attacks.

A case of palmoplantar pustular psoriasis induced by hydroxychloroquine in a patient with systemic lupus erythematosus.

Palmoplantar pustular psoriasis (PPP) is a rare clinical form of psoriasis. It is usually seen on the palms and soles, and affects patients' quality of life. In most cases, topical or systemic treatments are not sufficiently effective, so management of PPP is generally difficult. Hydroxychloroquine (HQ) is an antimalarial drug that is widely used in many autoimmune rheumatic diseases, mainly in systemic lupus erythematosus (SLE). Several reports describe the induction and exacerbation of psoriasis by HQ. Within this report, we aimed to put emphasis on considering possible drug effects by presenting a case of PPP, induced by HQ.

Prognostic significance of EGFR, MUC1 and PD-L1 expressions in cases with triple negative breast cancer.

PURPOSE: Twenty percent of the breast cancers are triple negative (TNBC). Despite the impressive progression in the biology of this subgroup, data is limited as compared to hormone and/or HER2 positive cases. Thus, the aim of this study was to detect the expression levels and to identify the prognostic values of MUC1, EGFR and PD-L1 in TNBC. METHODS: MUC1, EGFR and PD-L1 expressions were detected by immunohistochemistry in 97 cases with TNBC. Associations between clinical and histopathological parameters with overall survival (OS) and progression-free survival (PFS) were analyzed using the Kaplan-Meier method and compared by the log-rank test. Prognostic effects were analyzed by Cox proportional hazard models. RESULTS: During a median follow-up of 93 months (0.6-168.7) the mean PFS was 110.1 and OS was 121.8 months. Tumor diameter (T), involved lymph node status (N) and TNM were found to be prognostic for PFS and OS. PD-L1 in microenvironment (PD-L1 ME) and EGFR expression were found to be associated with longer PFS and OS, but MUC1 and tumor PD-L1 (PD-L1 TM) expressions were not. All combined analyses showed that in the subgroups of MUC1, PD-L1 TM or ME positive, EGFR expression was correlated with longer PFS and OS than those who were not. Older age (≥70 years), T and N status and also EGFR expression were found to be independent prognostic factors for OS in Cox regression analysis. CONCLUSION: EGFR expression was found to be one of the most important prognostic factors in addition to T and N status in cases with TNBC.

Assessment of potential predictive value of peripheral blood inflammatory indexes in 26 cases with soft tissue sarcoma treated by pazopanib: a retrospective study.

PURPOSE: The aim of this study was to evaluate the prognostic and predictive value of neutrophil-to-lymphocyte ratio (NLR), derived neutrophil-to-lymphocyte ratio (DNLR), lymphocyte-to-monocyte ratio (LMR), and platelet-to-lymphocyte ratio (PLR) in soft tissue sarcoma (STS) cases treated with pazopanib. MATERIALS AND METHODS: The study population included 26 STS cases treated with pazopanib for at least 3 months. NLR, DNLR, LMR, and PLR were evaluated at baseline, and at third month of therapy and also compared with response to pazopanib. Median measurements were taken as cutoff for NLR (4.8), DNLR (3.1), LMR (3.6), and PLR (195). The associations between these cutoff values and survival times (progression-free survival [PFS] and overall survival [OS]) were assessed by Kaplan-Meier curves and Cox proportional models. RESULTS: Patients with low pretreatment NLR and DNLR had longer OS (P=0.022, P=0.018), but low PLR was found to be associated only with longer OS. Additionally, decrease in NLR and DNLR after 3 months of therapy as compared with pretreatment measurements was found to be associated with an advantage for OS (P=0.021, P=0.010, respectively) and PFS (P=0.005, P=0.001, respectively). Response to pazopanib; changes in NLR, DNLR, LMR, and PLR; and >3 metastatic sites were found to be independent risk factors in univariate analysis, but NLR was the only independent risk factor in multivariate analysis. CONCLUSION: Low pretreatment and decrease in NLR and DNLR values, and regression/stable disease after 3 months of pazopanib are predictive factors for longer OS and PFS.

Prognostic significance of neutrophil/lymphocyte ratio (NLR) and correlation with PET-CT metabolic parameters in small cell lung cancer (SCLC).

PURPOSE: The aim of this study is to detect the prognostic significance of neutrophil/lymphocyte ratio (NLR) in SCLC and to evaluate the relation with 18F-FDG PET-CT metabolic parameters (PET-CT MPs). METHODS: Demographic parameters, laboratory values including NLR and other clinical variables were analyzed in 112 patients with small cell lung cancer (SCLC) and 54 of these patients had results of metabolic parameters detected with 18 FDG PET-CT [including SUVmax, SUVmean, metabolic tumor volume (MTV), whole body MTV (WBMTV), TLG (total lesion glycolysis), whole body TLG (WBTLG)] were evaluated for survival analyses. RESULTS: Mean and median overall survival (OS) and progression-free survival (PFS) were found to be significantly longer in cases with NLR < 4 compared with NLR > 4 in totally. Also stage, performance status, response to first-line therapy, LDH, and lymphocyte count were found to be prognostic for OS and PFS. MTV, WBMTV and WBTLG were found to be prognostic for both OS and PFS, while SUVmax found to be significant for OS. Patients with NLR ≥ 4, MTV ≥ 60.1, WBMTV ≥ 120 and WBTLG ≥ 1000 points had lower OS and PFS. A moderate positive correlation was found between NLR and SUVmean (r: 0.36), SUVmax (r: 0.34), TLG (r: 0.39), MTV (r: 0.51), WBMTV (r: 0.40), and WBTLG (r: 0.46). CONCLUSION: There is relationship between PET-CT metabolic parameters and NLR in SCLC. Highest correlation was found with NLR and MTV, WBMTV, and WBTLG, and evaluation of NLR together with these parameters predicts survival times and tumor biology more clearly in SCLC.

Primary small bowel lymphoma presenting as invagination.

One of the causes of invagination in adults is primary small bowel lymphoma. Primary small bowel lymphomas are rare, present themselves with complications due to diagnostic difficulties, and are diagnosed only after surgical intervention. In case of invagination, one of the complications of these tumors, namely primary small bowel lymphoma, should also be considered as a cause in the diagnosis. In this paper, the diagnosis and therapy of a rare case of primary polypoid-type small intestinal lymphoma demonstrating findings of obstruction due to invagination have been presented and discussed in the light of the literature.