Don't neglect the rare adverse event with intravenous immunoglobulin: Hemolytic anemia.

Intravenous immunoglobulin (IVIG) associated hemolytic anemia is an under-recognized complication of IVIG therapy. The incidence of this adverse event is not clear. Patients at high risk for IVIG-associated hemolytic anemia include non-O blood group recipients and those undergoing high-dose administration for inflammatory or autoimmune disorders. Here, two different cases of IVIG-associated hemolytic anemia are demonstrated. The first patient, a 66 year-old male with Guillain-Barré syndrome, had a severe attack for which erythrocyte replacement was required. Mild hemolysis was detected during IVIG administration in the second patient, a 57 year-old female with chronic immune thrombocytopenic purpura. Following IVIG termination, the hemolysis diminished gradually. Although it is rare and often manageable, clinicians should be aware of and monitor patients for hemolytic anemia following IVIG therapy.

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 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.

Sexual dysfunction in patients with systemic sclerosis

Background/aim: The aim of the study was to investigate the effect of systemic sclerosis (SSc) on quality of life and sexual function in female patients. Materials and methods: The study included 30 sexually active female patients with SSc and 30 healthy control subjects. For all participants in both the patient and control groups, the female sexual index and SF-36 forms were completed and a detailed medical and sexual history was taken. Results: The mean age was 45.03 ± 9.22 years in the SSc group and 44.6 ± 11.52 years in the control group (P = 0.87). The SF-36 scores in the patient group were significantly lower than those in the control group. Sexual dysfunction was found in 26/30 (86.6%) of the SSc patients and in 6/30 (20%) of the control group (P = 0.0001). Significant differences were determined between the groups with respect to sexual desire, arousal, lubrication, orgasm, sexual satisfaction, and pain. There was no significant relationship between the subgroups of SSc patients, duration of disease, lung involvement, and FSFI scores. Conclusion: Sexual dysfunction and lower health quality are common problems in female patients with systemic sclerosis.