Neuropathic pain in axial spondyloarthropathy is underdiagnosed and a confounding factor in biologic drug-switching decision: a cross-sectional study.

OBJECTIVES: The aim of this study was to evaluate the relationship between the presence of neuropathic pain (NeP), disease activity scores and biologic drug-switching decisions in the subjects with axial spondyloarthritis (axSpA) receiving biologic treatment. METHODS: PainDETECT Questionnaire was used to evaluate the presence of NeP in the patients with axSpA aged ≥18 years who had been receiving biologic treatment for at least 6 months. The relationships between disease activity scores, inflammatory markers, life quality index, biologic drug-switching decisions and the presence of NeP were analyzed. RESULTS: A total number of 175 patients with axSpA [ankylosing spondylitis (AS) (n:150) and non-radiographic axSpA (nr-axSpA) (n:25)] were enrolled in the study. NeP was detected in 41.7% of the patients and it was more common in females than in males (p:0.009). PainDETECT scores were positively correlated with disease activity scores, but they were not correlated with inflammatory marker levels. NeP was found to be significantly more common in whom the biologics had been switched 3 or more times (p:0.007). PainDETECT scores were higher and NeP was more prevalent (p:0.028) in the patients for whom drug-switching decisions had been made due to primary or secondary unresponsiveness. CONCLUSION: NeP is more common than estimated in the patients with axSpA and current disease activity scores are insufficient to make a distinction between NeP and inflammatory pain. NeP is a confounding factor in the evaluation of treatment response to biologic agents. In the subjects with AS and nr-axSpA with primary or secondary treatment unresponsiveness, the presence of NeP must be considered before biologic drug-switching decisions. Key Points • Neuropathic pain (NeP) is common in subjects with AxSpA treated with multiple biologic agents. • Current disease activity scores for AxSpA are insufficient to make a differentiation between NeP and inflammatory pain. • NeP is a confounding factor in the evaluation of treatment response to biologic agents. • Patients with AxSpA should be re-evaluated in terms of the presence of neuropathic pain before making biologic drug-switching decisions.

Pneumomediastinum, pneumatosis intestinalis and pneumoperitoneum in a patient with polymyositis: case-based review.

Pneumomediastinum (PnM), pneumatosis intestinalis (PI), and pneumoperitoneum (PP) are rare complications of inflammatory myositis. We present a 59-year-old polymyositis (PM) patient who experienced all three complications simultaneously. The patient who presented with proximal muscle weakness, dysphagia, and weight loss was diagnosed with PM due to elevated muscle enzymes and consistent electromyography and muscle biopsy with inflammatory myopathy. On the 45th day of her immunosuppressive treatment, PnM, PI, and PP were detected incidentally in 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/computed tomography (CT) scan performed for severe weight loss and treatment-resistant severe disease. Since the patient had no symptoms or signs of PnM and PP, no additional intervention was applied to the current treatment, and spontaneous regression was observed in the follow-up. In addition to this case, we reviewed patients with PM who developed PBM, PP, and PI in the literature.

C allele in transforming growth factor-ß1 rs1800471 gene polymorphisms might indicate a protective feature in encapsulating peritoneal sclerosis development.

INTRODUCTION: Peritoneal fibrosis may progress in peritoneal dialysis (PD) patients to a fatal clinical condition called encapsulating peritoneal sclerosis (EPS). Transforming growth factor (TGF)-ß plays a pivotal role in the pathogenesis of peritoneal fibrosis. We aimed to investigate the association among polymorphisms in the gene encoding TGF-ß1, which were -509C/T (rs1800469), +869T/C (rs1982073), and +915G/C (rs1800471) in EPS patients. METHODS: A total of 16 PD patients who were clinically and radiologically diagnosed with EPS were enrolled and 22 age- and gender-matched PD patients were selected as the non-EPS group. RESULTS: G allele frequency at the rs1800471 gene polymorphism was significantly higher in the EPS group than non-EPS group (p = 0.005). Interestingly, the non-EPS group patients had CC or CG polymorphisms. CONCLUSION: C allele in TGF-ß1 rs1800471 gene polymorphisms might indicate a protective feature in EPS development. Knowing the presence of polymorphism may be effective in selecting renal replacement therapy in patients.

The risk of cancer in patients with primary Sjögren syndrome: a single-center study from Turkey.

BACKGROUND: The aim of this study is to determine the risk of cancer in patients with primary Sjögren syndrome (pSS) from a single center in Turkey. METHODS: Clinical data of the subjects with pSS were retrospectively analyzed. The incidence of cancer for general population was obtained from GLOBOCAN 2018. Age- and sex-specific standardized incidence ratios (SIR) of solid and hematological cancers were calculated compared with the general population. RESULTS: Four hundred thirty patients with pSS were included in the study. The majority of the patients were female (n = 396, 92.1%), and the mean age was 58.6 ± 12.0 years. Thirty-four patients (7.9 %) were diagnosed with cancer (26 solid and 8 hematological) during follow-up. The SIR for all cancers was 2.45 (95% CI, 1.625-3.275). The SIR was 2.42 (95% CI, 1.542-3.298) for solid cancers and 8.42 (95% CI, 2.394 - 14.446) for hematological cancers. The most diagnosed malignancies were breast cancer (n = 6), ovarian cancer (n = 6), and non-Hodgkin lymphoma (NHL) (n = 4). There was an increased risk for ovarian cancer (SIR 12.76, 95% CI, 2.545-22.975). The SIR values were 2.08 (95% CI, 0.419-3.741) and 10.81 (95% CI, 0.216-21.404) for breast cancer and NHL, respectively. DISCUSSION: The risk of hematological and solid cancers was higher in the patients with pSS when compared to general population. In our pSS cohort, the risk for ovarian cancer was found to be increased, which has not been previously reported in the literature.

The use of anti-interleukin-1 agents and tumor necrosis factor-alpha inhibitors in renal transplant recipients.

OBJECTIVES: The aim of this study was to investigate the efficacy and safety of anti-interleukin-1 (anti-IL-1) agents and tumor necrosis factor-alpha (TNF-α) inhibitors in renal transplant patients. PATIENTS AND METHODS: Between February 2014 and February 2020, data of 12 renal transplant recipients (9 males, 3 females; median age: 51 years; range, 19 to 70 years) who received anti-IL-1 agents or TNF-α inhibitors for inflammatory diseases in the post-transplant time period and were followed in a single transplant center (n=12) were retrospectively analyzed. A total of 46 cases were reported in the literature, before the data were collected. The overall outcomes of all cases were analyzed in this study. RESULTS: Thirty-seven patients received anti-IL-1 agents in the post-transplant period. The main indications for anti-IL-1 agents were familial Mediterranean fever (FMF) and amyloidosis (75.7%). The continuation rate of colchicine treatment in patients with FMF was 85.7%. Anti-IL-1 agents prevented attacks completely in 89.3% of FMF patients. The number of cases used TNF-α inhibitors among renal transplant patients was lower (n=21). The TNF-α inhibitors were used mainly for inflammatory bowel diseases (57.1%) and ankylosing spondylitis (33.3%) and suppressed the disease activity in most of the patients with inflammatory diseases (72.7%). Death (n=3) and malignancies (n=3) were reported in patients who received TNF-α inhibitors, but not in patients who received anti-IL-1. The renal outcomes and graft survival rates were satisfactory in patients who received both anti-IL-1 agents and TNF-α inhibitors. CONCLUSION: Our results support that anti-IL-1 agents can be used effectively and safely in renal transplant patients.

The risk of cancer in patients with primary Sjögren syndrome; A single-center study from Turkey.

BACKGROUND: The aim of this study is to determine the risk of cancer in the patients with primary Sjögren syndrome (pSS) from a single-center in Turkey. METHODS: Clinical data of the subjects with pSS were retrospectively analyzed. The incidence of cancer for general population was obtained from GLOBOCAN 2018. Age- and sex-specific Standardized Incidence Ratios (SIR) of solid and hematological cancers were calculated compared with the general population. RESULTS: Four hundred thirty patients with pSS were included in the study. The majority of the patients were female (n=396, 92.1%), and the mean age was 58.6 ±12.0 years. Thirty-four patients (7.9 %) were diagnosed with cancer (26 solid and 8 hematological) during follow-up. The SIR for all cancers was 2.45 (95% CI, 1.625- 3.275). The SIR was 2.42 (95% CI, 1.542-3.298) for solid cancers and 8.42 (95% CI, 2.394 - 14.446) for hematological cancers. The most diagnosed malignancies were breast cancer (n=6), ovarian cancer (n=6), and non-Hodgkin lymphoma (NHL) (n=4). There was an increased risk for ovarian cancer (SIR 12.76; 95% CI, 2.545-22.975). The SIR values were 2.08 (95% CI, 0.419-3.741) and 10.81 (95% CI, 0.216-21.404) for breast cancer and NHL, respectively. CONCLUSION: The risk of hematological and solid cancers was higher in the patients with pSS when compared to general population. In our pSS cohort, the risk for ovarian cancer was found to be increased, which has not been previously reported in the literature.

The effects of biological agents on vascular structural lesions in Takayasu's arteritis.

OBJECTIVES: The aim of the present study was to evaluate the effects of biological disease-modifying antirheumatic drugs (bDMARDs) administered to patients with Takayasu's arteritis (TAK) on disease activity and vascular damage. METHODS: This study included TAK patients who were receiving bDMARDs for at least six months. Disease activity (National Institutes of Health [NIH]), vascular lesions, and vascular damage (Combined Arteritis Damage Score [CARDS]) scores were determined. RESULTS: There were 21 TAK patients who received infliximab (INF) and/or tocilizumab (TCZ) (mean age = 38.6±11.8 years; female proportion = 20 [95.2%]). The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and NIH disease activity score were found to significantly decrease with bDMARD treatments. There were also significant decreases in the mean CARDS and the total number of vascular lesions after treatment (p<0.05). Unlike occlusions, an important decrease was observed in the occurrences of stenosis and aneurysms with bDMARD treatments. Regression was detected in the vascular lesions of 15 (71.4%) patients compared to the last image before bDMARD therapies. CONCLUSIONS: Our study results indicate that biological agents, such as INF and/or TCZ, that are used in the treatmentof TAK are capable of remedying certain vascular lesions and may provide additional benefits to patients with TAK who do not sufficiently respond to conventional synthetic disease-modifying antirheumatic drug (DMARD) treatment.

Clinical and serological characteristics of seronegative primary Sjögren's syndrome: a comparative study.

OBJECTIVES: This study compared the clinical and serological characteristics of seronegative and seropositive primary Sjögren syndrome (pSS) and examined whether current classification criteria for pSS cover seronegative pSS. METHODS: The study group comprised 375 patients (341 women and 34 men) diagnosed with pSS. A clinical diagnosis by an expert rheumatologist was considered the "gold standard" for the diagnosis of pSS. The clinical and serological characteristics of the patients were retrospectively collected from hospital medical files. RESULTS: Fifty-eight of the 375 pSS patients (15.5%) were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. There were no statistically significant differences in terms of patient age, age at diagnosis, sex distribution, clinical features, and laboratory findings between seronegative and seropositive pSS. The frequency of hypergammaglobulinemia was higher in seropositive pSS. The 2016 ACR/ULAR criteria best covered most seronegative pSS cases (84.5%). For seronegative pSS, the agreement between the 2002 AECG, 2012 ACR, and 2016 ACR/EULAR criteria was relatively low. CONCLUSIONS: The clinical features of seronegative pSS (i.e., a lack of four autoantibodies in serum) were similar to those of seropositive pSS. The current classification criteria for pSS should not be used in the diagnosis of seronegative pSS, as the agreement between the different sets of criteria was low, and some patients fell outside the classification. Further clinical and laboratory studies are needed to identify the features that distinguish seronegative pSS. Key Points • Approximately 15% of the pSS patients were seronegative for ANA, RF, anti-Ro, and anti-La autoantibodies. • Seronegative pSS was diagnosed based on lymphocytic infiltrations in lip biopsy samples. • The clinical features of seronegative pSS were similar to those of seropositive pSS. • The current classification criteria for pSS should not be used in the diagnosis of seronegative patients, as the agreement between the different sets of criteria was low, and some patients fell outside the classification.

Survival analysis of patients with Sjögren's syndrome in Turkey: a tertiary hospital-based study.

OBJECTIVES: This study was conducted to determine long-term survival rates and the factors associated with mortality in Turkish primary Sjögren syndrome (pSS) patients. METHODS: All patients diagnosed with pSS between 2004 and 2014 were included in this study. By January 2019, all subjects still living by the end of the study, as well as any death, were identified. Survival rates and standard mortality rates (SMRs) using general population mortality data were calculated. Mortality-related factors were determined by univariate and multivariate analysis. RESULTS: During follow-up, 33 cases of 372 pSS patients resulted in death (8.9%). Of those patients, they were typically older at disease onset, at recruitment, and had shorter follow-up times (p < 0.001 for all). The overall SMR of all pSS patients compared with the general population was 2.11 (95% confidence interval (CI) 1.39-2.83). Male pSS patients had a higher SMR than that of general male patients. Overall survival rates were 97.8% at five years, 90.2% at 10 years, and 87.1% at 15 years in patients with pSS. The survival rate of pSS patients was significantly lower than the general Turkish population (p = 0.011). Multivariate Cox regression analysis showed that older age at disease onset and the presence of interstitial lung disease (ILD) were independent risk factors for mortality. CONCLUSIONS: Based on these data, mortality rates of Turkish pSS patients are higher compared with the general population. Survival significantly decreased in the pSS patients with ILD, especially in older male patients at disease onset. Male gender and malignancy may also be associated with a worse prognosis in pSS patients.Key Point• Mortality in Sjögren's syndrome.