Non-criteria autoantibodies in antiphospholipid syndrome may be associated with underlying disease activity.

BACKGROUND: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by persistent antiphospholipid antibodies (aPLs) with arterial and venous thrombosis and/or pregnancy morbidity. In recent years, several studies have highlighted the potential role of non-criteria aPL in diagnosing APS patients. AIM: This study aimed to determine the association of the presence of non-criteria aPL antibodies to the clinical and laboratory features of patients with a diagnosis of APS. METHODS: Eighty patients diagnosed with APS and under observation in the rheumatology clinic of Ankara City Hospital were assessed. Patient demographic and clinical features were meticulously recorded. Non-criteria antibodies tested in our center included antiphosphatidylserine IgA, antiphosphatidylserine IgM, beta 2 glycoprotein IgA, anti-cardiolipin IgA, antiphospholipid antibody IgG, and antiphospholipid antibody IgM. Antibodies from patients who were tested for at least one non-criteria antibody were documented. RESULTS: Out of 80 patients, 55 (68.8%) were tested for at least one non-criteria antibody, and 29 of those patients (52.7%) tested positive for at least one non-criteria antibody. The antiphospholipid antibody IgM and the beta 2 glycoprotein IgA were the most commonly tested non-criteria antibodies. Patients with non-criteria antibody positivity had a higher frequency of Ds DNA positivity and low complement (62.0% vs. 35.0%, p = 0.042; 69.0% vs. 38.0%, p = 0.023), respectively. In addition, positivity for anti-cardiolipin IgG and b2 glycoprotein IgG was significantly higher in the group positive for non-criteria antibodies (79% vs. 31%, p ≤ 0.001; 72.0% vs. 19%, p ≤ 0.001). There was no significant difference between the clinical features of patients with at least one positivity for non-criteria antibodies and those without. CONCLUSION: Systemic lupus erythematosus (SLE) is the most commonly associated disease with APS, being present in approximately 35% of cases [1]. Since the majority of the patient group in our study had APS that was secondary to SLE, non-criteria antibody positivity may be linked to the immunological activity of SLE. Large multicenter studies are necessary to investigate the clinical significance of isolated/combined positivity for criterion/non-criteria aPLs.

A single-center rheumatology experience of sarcoidosis: observations from 70 patients.

BACKGROUND: The aim of this study is to determine the demographic, clinical and laboratory characteristics of the patients who followed up with the diagnosis of sarcoidosis, to investigate the distribution frequency of rheumatological findings and to examine the disease management from the perspective of rheumatology. METHODS: Patients who were followed up with the diagnosis of sarcoidosis in the rheumatology clinic of Ankara City Hospital between November 2019 and November 2022 were evaluated. Demographic, clinical, radiological, serological, laboratory, and histopathological findings, and rheumatological, systemic, and locomotor system examination findings of the patients were obtained from the medical data registered in the hospital. RESULTS: A total of seventy sarcoidosis patients (48.98 ± 11.78 years, %75 female) were included in the study. Joint involvement was observed in 64.3% of cases, skin involvement in 48.6% of cases, and ocular involvement in 25.7% of cases. The ankle was the most frequently involved joint, followed by the knee and small joints in the foot. Corticosteroids were the most used therapeutic agent, and pulmonary and joint findings were the most common reasons for starting treatment. CONCLUSIONS: Sarcoidosis is a disease that mimics many diseases, misdiagnosis and treatment should be avoided with a good and fast differential diagnosis. Clinicians, especially rheumatologists, should remember sarcoidosis more frequently and keep it in mind in the differential diagnosis.

Rheumatology experience with FDG PET / CT in inflammation of unknown origin: a single - centre report for determining factors associated with diagnostic precision.

BACKGROUND / AIM: The use of PET / CT is becoming more common in the elucidation of inflammatory processes in which the underlying cause cannot be determined by conventional examinations. Although PET / CT is an effective method for detecting inflammatory foci, the precise diagnosis may not be obtained in all cases. In addition, considering factors such as radiation exposure and cost, it becomes important to identify patients who can get results with PET / CT. In this study, it was aimed to examine the factors that can predict the differential diagnostic value of PET / CT by retrospectively scanning patients who underwent PET / CT for inflammation of unknown origin (IUO) in rheumatology practice. METHODS: Demographic, clinical and laboratory information of the patients followed up in our clinic and who underwent PET / CT for differential diagnosis were enrolled. Whether they were diagnosed after PET / CT and during the follow - up period, and their diagnoses were examined. RESULTS: A total of 132 patients were included in the study. A previous diagnosis of rheumatic disease was present in 28.8 % of the patients, and a history of malignancy was present in 2.3 % . The patients were divided into three groups: group 1 patients with increased FDG uptake in PET / CT and diagnosis confirmed by PET / CT, group 2 patients with increased FDG uptake in PET / CT but diagnosis was not confirmed, and group 3 patients without increased FDG uptake in PET / CT. Increased FDG uptake in PET / CT was detected in 73 % of the patients. While PET / CT helped the diagnosis in 47 (35.6 %) patients (group 1), it did not help the diagnosis in 85 (64.4 %) (groups 2 and 3). Thirty - one (65.9 %) of the diagnosed patients were diagnosed with a rheumatologic disease. When the 3 groups were compared, male gender, advanced age, CRP levels, presence of constitutional symptoms, SUVmax values and number of different organs with increased FDG uptake were higher in Group 1. Sixty - six percent and 74 % of the patients in groups 2 and 3 were not diagnosed during the follow - up period. No patient in group 3 was diagnosed with malignancy during follow - up. CONCLUSION: PET / CT has high diagnostic value when combined with clinical and laboratory data in the diagnosis of IUO. Our study revealed that various factors can affect the diagnostic value of PET / CT. Similar to the literature, the statistically significant difference in CRP levels shows that patients with high CRP levels are more likely to be diagnosed with an aetiology in PET / CT. Although detection of involvement in PET / CT is not always diagnostic, there was an important finding that no malignancy was detected in the follow - up in any patient without PET / CT involvement. Key points • PET / CT is an effective method for detecting inflammatory foci. • PET / CT has proven to be effective in the diagnosis of rheumatological diseases, the extent of disease and the evaluation of response to treatment. • Indications for the use of PET / CT in the field of rheumatology and the associated factors and clinical features supporting the diagnosis with PET / CT are still to be fully clarified. • In routine practice, with PET / CT, both delays in diagnosis and examinations performed during diagnosis and the cost can be reduced.

COVID-19 disease frequency, risk factors, and re-infection rates in patients with autoimmune rheumatic disease receiving rituximab.

INTRODUCTION: Rituximab, which is used in autoimmune rheumatic diseases (ARD), can cause both an increased risk of development of COVID-19 disease and re-infection due to its potent and long-acting immunosuppression. So, we aimed to evaluate the frequency, risk factors and re-infection rates of COVID-19 in ARD patients receiving rituximab. METHODS: A single-center retrospective study was performed with patients receiving rituximab for ARD in 12 months before the onset of COVID-19 in Turkey. The data regarding severe acute respiratory syndrome-coronavirus 2 reverse transcription polymerized chain reaction (RT-PCR) test, clinical, laboratory, and mortality data of all patients were collected from medical records. Logistic regression analysis was used for predictors of COVID-19 disease. COVID-19 re-infection was defined as RT-PCR positivity and recurrence of acute COVID-19 symptoms after at least 1 negative RT-PCR in patients with clinical improvement. RESULTS: Ninety-eight ARD patients with rituximab were evaluated and 23 (23%) of them had COVID-19. The presence of hypogammaglobulinemia increased the risk of COVID-19 disease 8-fold. COVID-19 pneumonia occurred in 13 (57%) and these patients' age was higher than those without pneumonia (59.6 ± 11.8 vs 44.9 ± 14.2 years, P = 0.013). Mortality due to COVID-19 was 13% and COVID-19 re-infection was seen in 20% of survivors. CONCLUSION: Regardless of the underlying rheumatic disease and organ involvements, hypogammaglobulinemia in ARD could be a risk factor for COVID-19 development, and advanced age could be for COVID-19 severity. Moreover, COVID-19 re-infection rates are high.

Effects of sulfasalazine in axial spondyloarthritis on COVID-19 outcomes: real-life data from a single center.

INTRODUCTION: Compared to biological agents, little is known about the impact of sulfasalazine therapy on COVID-19 outcomes in patients with Axial Spondyloarthritis (AxSpA). Therefore, we aimed to evaluate the COVID-19 severity in AxSpAs receiving sulfasalazine and biologic-agent. MATERIALS AND METHODS: A total of 219 SARS-CoV-2 positive AxSpA patients were retrospectively analyzed. COVID-19 pneumonia, hospitalization rate, and length of stay were used to determine COVID-19 severity. AxSpA patients were mainly grouped and compared as sulfasalazine and non-sulfasalazine. Afterward, we excluded no-treatment patients to reveal the drug's effects more clearly and regrouped AxSpA patients as sulfasalazine-monotherapy (34.3%), biologic-monotherapy (33.7%), and sulfasalazine + biologic (7.3%). RESULTS: Fifty-nine percent of the patients were male and the mean age was 45.0 years. Peripheral arthritis was 35% and uveitis 15%. In total, 41.5% of them have received sulfasalazine and 41.0% biologic agents, and the remaining patients with no AxSpA-specific treatment. In the first comparison, the sulfasalazine group had a higher age, more frequent COVID-19 pneumonia, hospitalization, and longer hospitalization than a non-sulfasalazine group. In the pairwise comparison of 3 treatment groups, the demographic and clinical features, the hospitalization rate and the length of hospital stay were similar but the sulfasalazine-monotherapy group had a higher frequency of COVID-19 pneumonia than the biologic-monotherapy group (23% vs. 7%, p = 0.008). CONCLUSION: Our results imply sulfasalazine may be related to more severe COVID-19 in AxSpA patients. These patients should be followed more carefully in the presence of COVID-19, regardless of reasons such as age, comorbidity, and extra-axial disease, and consideration of discontinuing sulfasalazine maybe even thought.

A single-center COVID-19 vaccine experience with CoronaVac and BNT162b2 in familial Mediterranean fever patients.

AIM: To determine frequency of adverse events and attacks related to vaccination in recipients of CoronaVac and BNT162b2 in familial Mediterranean fever (FMF) patients, and to search whether history of prior COVID-19 or a booster dose increases occurrence of adverse events/attacks. METHODS: FMF patients were surveyed for administration of any COVID-19 vaccine and vaccine-related adverse events or FMF attacks. Demographic, clinical, vaccine-related data, history of COVID-19 infection before or after vaccination, adherence to FMF treatment during vaccination were collected. RESULTS: A total of 161 vaccinated FMF patients were included. Ninety-three patients out of 161 had reported suffering from an adverse event/attack after a vaccine dose. There were 54.7% of BNT162b2 recipients who reported any adverse event after any vaccine dose in comparison to 29.9% of CoronaVac recipients (P < .001). There were 22.2% of BNT162b2 recipients who reported suffering from a FMF attack within 1 month after vaccination in comparison to 19.4% of CoronaVac recipients (P = .653). When patients with or without adverse event/attack were compared, no significant differences were observed in means of demographics, comorbid diseases, disease duration, total vaccine doses, or treatments adhered to for FMF. Rates of adverse events/attacks were similar between patients with and without prior COVID-19. In booster recipients, adverse events/attacks were most frequent after the booster dose. CONCLUSIONS: A considerable number of FMF patients suffered from vaccine-related adverse events/attacks, particularly with BNT162b2. No serious events or mortalities due to vaccination were detected. Demographics, clinical characteristics and prior history of vaccination did not significantly affect these results. We observed an increased rate of adverse events/attacks with booster dose administration.

Performance of the systemic lupus erythematosus risk probability index in a cohort of undifferentiated connective tissue disease.

OBJECTIVES: We sought to evaluate the performance of the SLE Risk Probability Index (SLERPI) for identification of SLE in a large cohort of patients with UCTD. METHODS: The SLERPI was applied in a cohort of patients who met classification criteria for UCTD and did not fulfil any classification criteria for other defined CTD including SLE. Patients with a SLERPI score of >7 were 'diagnosed' as SLE. Patients diagnosed with SLE and those not were compared in terms of disease characteristics and index parameters. RESULTS: A total of 422 patients with UCTD were included in the study. Median (interquartile range) SLERPI was 4.25 (2.5) points, while 39 (9.2%) patients had a SLERPI score >7 and were diagnosed as SLE. Patients with younger age (P = 0.026) and presence of malar rash (P < 0.0001), mucosal ulcer (P < 0.0001), alopecia (P < 0.0001), ANA positivity (P < 0.0001), low C3 and C4 (P = 0.002), proteinuria >500 mg/24 h (P = 0.001), thrombocytopenia (P = 0.009) or autoimmune haemolytic anaemia (P < 0.0001) were more likely to fulfil criteria for SLE by the SLERPI. CONCLUSION: SLERPI enabled a significant proportion of patients to be identified as SLE in our UCTD cohort. This new probability index may be useful for early identification of SLE among patients with signs of CTD without fulfilling any definite criteria set.

First Reported Case of Echinococcal Disease on a Renal Graft Successfully Treated With Albendazole.

Echinococcal disease is an endemic disease for eastern Mediterranean countries. Various types of kidney involvement have been reported. Here, we report the first case of echinococcal disease on a transplanted kidney in a patient who was successfully treated with albendazole alone. The patient (a 38-year-old female) was evaluated for elevated creatinine levels 7 months after receiving a living-donor allograft. Standard immunosuppression therapy protocols were applied. Tacrolimus level was normal, and the patient was compliant with treatment. Creatinine level was 1.91 mg/dL (baseline: 1.2 mg/dL); proteinuria level was 1300 mg/day. The graft was found to be normal, as evaluated with standard sonographic methods. A kidney biopsy was performed, which showed that part of the cortical parenchyme was infiltrated by echinococcal protoscolices with hooklets. Because there were no cysts present on the graft, we concluded that disease was at an early stage. The patient was given albendazole for 3 months. After therapy, all echinococcal structures disappeared. Her creatinine level dropped to baseline, and proteinuria resolved. Echinococcal disease can affect transplanted kidneys. Albendazole is a valuable treatment option for patients who are not candidates for surgical resection.