Clinical features of systemic lupus erythematosus patients with splenomegaly: focussed on the cytopenias.

This study aimed to investigate the clinical features of splenomegaly, mainly focussing on cytopenia, in patients with systemic lupus erythematosus (SLE). Cytopenia was commonly observed in 111 SLE patients with splenomegaly (n = 79, 71.2%). During the follow-up period, two patients developed haematologic malignancy after the diagnosis of SLE and splenomegaly, but no patients experienced severe complications (e.g. splenic rupture) related to splenomegaly.

Long-term outcomes and prognostic factors after aortic valve surgery in patients with Takayasu arteritis.

BACKGROUND: Aortic regurgitation (AR) is a common cardiovascular complication in patients with Takayasu arteritis (TAK), and complication after aortic valve surgery (AVS) is not rare. This study aimed to identify the long-term postoperative outcomes for significant AR in patients with TAK compared with those in patients without TAK. METHODS: We included 35 patients with TAK with moderate-to-severe AR who underwent AVS and compared their postoperative outcomes with those of 105 age- and operation period-matched patients with severe AR but without TAK. The risk factors for poor outcomes [all-cause death and major adverse cardiac and cerebrovascular events (MACCE)] in patients with TAK were analyzed using multivariate Cox regression. RESULTS: The 10-year overall survival rate was 70.5% in patients with TAK and 89.4% in those without TAK (p = 0.048). The MACCE and reoperation rates were significantly higher in patients with TAK (10-year freedom from MACCE, 58.2% vs. 86.4% [p < 0.001]; 10-year freedom from reoperation, 64.5% vs. 98.3% [p < 0.001]). Eighteen of the 35 patients with TAK (51.4%) had poor outcomes, and multivariate analysis revealed that significant coronary artery involvement [hazard ratio (HR), 4.178; 95% confidence interval (CI), 1.222-14.282; p = 0.023] and decreased estimated glomerular filtration rate (HR, 0.968; 95% CI 0.947-0.989; p = 0.003) were associated with poor outcomes. CONCLUSION: The long-term postoperative outcomes for AR were poorer in patients with TAK than in those without TAK. The poor outcomes in patients with TAK were associated with coronary artery involvement and decreased renal function.

Macrophage activation syndrome in rheumatic disease: Clinical characteristics and prognosis of 20 adult patients.

OBJECTIVES: Macrophage activation syndrome (MAS) is a hyperinflammatory condition that is known to be secondary hemophagocytic lymphohistiocytosis (HLH) in patients with rheumatic disease. The aim of study was to evaluate the clinical manifestations and outcomes in patients with MAS with rheumatic disease. MATERIALS AND METHODS: We performed a retrospective study of 20 adult patients who were diagnosed with MAS from 2012 to 2020. MAS was classified according to the HLH-2004 criteria. Patients' information, including clinical features, laboratory findings, and treatment regimens, was collected, and the overall survival rate was estimated by the Kaplan-Meier method. RESULTS: Twenty patients (18 women, 35.6 ± 18.3 years) who met the HLH-2004 criteria also fulfilled the 2016 EULAR/ACR/PRINTO classification criteria for MAS, and HScore was higher than 169 (mean, 241.1). Fourteen patients with systemic lupus erythematosus and 6 patients with adult-onset Still's disease were included. All patients were treated initially with corticosteroids, and 16 patients required additional immunosuppressants. The overall survival at 3 and 6 months was 75.2% and 64.3%. In survivors, renal impairment was less common (7.7% versus 71.4%, p = 0.007), the levels of AST (364.0 versus 81.0 IU/L, p = 0.019) and LDH (1346.0 versus 343.0IU/L, p = 0.014), and platelet count (90.0 versus 43.0 × 109/L, p = 0.02) were higher in compared to non-survivors. Nine patients had opportunistic infections, five of whom died during admission. CONCLUSION: The mortality of patients with MAS associated with rheumatic disease remains high. Renal impairment, levels of AST and LDH, and platelet count might be associated with prognosis.

Risk of New-Onset Diabetes Mellitus Associated with Antirheumatic Drugs in Patients with Rheumatoid Arthritis: A Nationwide Population Study.

Background: This study aimed to investigate the effect of disease-modifying antirheumatic drugs (DMARDs) on diabetes mellitus (DM) development in rheumatoid arthritis (RA). Methods: This nested case−control study with a cohort of 69,779 DM-naïve adult patients with RA was conducted from 2011 to 2019 in South Korea. Cases with incident DM were identified and individually matched to randomly selected controls (1:4). DMARDs use was measured for 1 year before the index date and stratified by exposure duration. The association of each DMARD use with DM risk was estimated using conditional logistic regression adjusted for comorbidities and concomitant drug use. Results: Of the patients, 5.4% were newly diagnosed with DM. The use of statins and a higher cumulative dose of corticosteroids were associated with an increased DM risk. In a multivariable-adjusted analysis, cumulative duration of exposure (CDE) >270 days/year, hydroxychloroquine (HCQ; adjusted odds ratio [aOR], 0.76) and methotrexate (MTX; aOR, 0.81) were associated with a significant decrease in DM risk, and tacrolimus (TAC; aOR, 1.27) was associated with an increased risk. Conclusions: Long-term use of HCQ and MTX (>270 days/year) was associated with a reduction in DM incidence as opposed to TAC.

Idiopathic Inflammatory Arthritis in the Auditory Canal in a Patient With Hearing Impairment: A Case Report and Literature Review.

Inflammatory arthritis can affect the auditory system during the disease course. Although most cases show asymptomatic hearing impairment, it can result in hearing loss. Here we describe the case of a 70-year-old female with hearing impairment associated with idiopathic inflammatory arthritis in her auditory system. She had suffered from hearing difficulties for decades; however, the causes of her hearing impairment had not been evaluated. Pure tone audiometry showed severe sensorineural hearing loss requiring a cochlear implant. The workup for the cochlear implant revealed erosive changes in the incudomalleolar and incudostapedial joints with soft tissue swelling on temporal bone computed tomography. Bone pathology revealed plasmacytic infiltration and granulomatous inflammation. Laboratory examinations showed elevated levels of inflammatory markers; otherwise, she had negative results for all autoantibodies. In patients with idiopathic hearing loss, inflammatory arthritis of the middle ear without peripheral arthritis can provide a clue regarding the cause of the hearing loss.

Anaphylaxis to tocilizumab in patients with rheumatic disease.

Background: Anaphylaxis to tocilizumab has been reported anecdotally. Therefore, we evaluated the incidence of anaphylaxis in patients starting tocilizumab. Materials & methods: This retrospective study included patients with rheumatic disease who were administered tocilizumab from 2013 to 2020. The incidence of anaphylaxis was examined during the first 6 months. Results: During follow-up, four of 171 patients developed anaphylaxis within the third course of infusions. The incidence of anaphylaxis to tocilizumab was higher in patients with adult-onset Still's disease (AOSD) than in those with other rheumatic disease (21.4% in AOSD vs 0.7% in rheumatoid arthritis vs 0% in Takayasu arteritis). Conclusions: When we consider tocilizumab treatment, especially in AOSD, we should keep in mind that intensive monitoring for anaphylaxis is necessary.

Lay abstract Anaphylaxis is a life-threatening allergic reaction that can be triggered by food, latex and drugs. Tocilizumab is widely used to treat rheumatic disease, including rheumatoid arthritis (RA), adult-onset Still's disease (AOSD) and Takayasu arteritis (TAK). As case reports of tocilizumab-induced anaphylaxis have previously been reported, we evaluated the incidence of anaphylaxis in patients starting tocilizumab treatment. Over the course of 6 months, four of 171 (21.4% in AOSD vs 0.7% in RA vs 0% in TAK) patients developed anaphylaxis within the third course of infusions. An AOSD patient with anaphylaxis, who presented with active disease status persistently, was successfully treated with tocilizumab again after desensitization. In conclusion, when considering tocilizumab treatment, especially in AOSD patients, we should keep in mind that intensive monitoring for anaphylaxis is necessary.

Risk of progression of idiopathic pulmonary fibrosis to connective tissue disease: a long-term observational study in 527 patients.

OBJECTIVE: Connective tissue disease (CTD) might occur during the course of idiopathic pulmonary fibrosis (IPF). Clinical factors associated with CTD development in IPF patients have still not been identified. We investigated which antibodies have a significant association with the development of CTD during the clinical course of IPF. METHODS: We retrospectively reviewed the records of 527 patients with a first diagnosis of IPF between January 2007 and March 2014 and investigated the time to CTD development after IPF diagnosis in these patients. RESULTS: CTD developed in 15 patients at a median of 2.1 years (range 1.2-4.8) after IPF diagnosis. All patients had anti-neutrophil cytoplasmic antibodies (ANCA) or autoantibodies that met the serology criteria for interstitial pneumonia with autoimmune features (IPAF). Survival duration for IPF patients with progression to CTD was 5.3 (3.8, 6.7) years, which was significantly longer than for IPF patients without progression to CTD [2.9 (1.7, 4.8), p = 0.001]. Independent risk factors for CTD development in IPF patients included female gender [adjusted hazard ratio (HR) 5.319, p = 0.0082], titer of rheumatoid factor (RF; adjusted HR, 1.006; p = 0.022), titer of anti-citrullinated protein antibody (ACPA; adjusted HR, 1.009; p = 0.0011), and titer of myeloperoxidase (MPO)-ANCA (adjusted HR, 1.02; p < 0.0001). CONCLUSION: Progression to CTD is uncommon in IPF patients. However, a significant number of IPF patients with high titers of RF, ACPA, or MPO-ANCA progressed to CTD. RF, ACPA, and MPO-ANCA might be significantly associated with CTD development in IPF patients. Key Points • A significant number of IPF patients with high titers of RF, ACPA, or MPO-ANCA progressed to CTD. • IPF/UIP with high titers of RF, ACPA, or MPO-ANCA might be the initial clinical manifestation of CTD. • RF, ACPA, and MPO-ANCA may be significantly associated with the development of pulmonary fibrosis in patients with CTD.

Early discontinuation of tofacitinib in patients with rheumatoid arthritis co-treated with rifampin for latent tuberculosis.

OBJECTIVE: Rifampin is known to influence the pharmacokinetics of tofacitinib owing to drug interactions. The aim of this study was to determine the efficacy of tofacitinib on co-administration with rifampin in rheumatoid arthritis (RA) patients. METHODS: Biologic-naïve RA patients treated with tofacitinib were selected, and electronic medical reports were reviewed retrospectively. All patients underwent screening for latent tuberculosis infection (LTBI) before starting tofacitinib, and patients with positive results were treated to prevent progression to active tuberculosis. To evaluate the efficacy of tofacitinib with or without rifampin, the discontinuation rates of tofacitinib were examined during the first 6 months. Kaplan-Meier analysis was used to construct cumulative discontinuation curves, and comparisons were performed using the log-rank test. RESULTS: Among 81 patients who starting tofacitinib, 21 were LTBI-positive and 18 were administered rifampin concomitantly with tofacitinib. Additionally, 14 of the 81 patients (17.3%) discontinued tofacitinib during the follow-up, and 7 patients discontinued tofacitinib because of uncontrolled RA activity. The discontinuation rates of tofacitinib within the first 6 months were significantly higher in patients treated with rifampin for LTBI than in those not treated with rifampin (lack of efficacy: 24.7% vs. 5.1%, P<0.01; all causes: 38.9% vs. 11.2%, P<0.01). CONCLUSIONS: Discontinuation rates were higher in RA patients who started tofacitinib during chemoprophylaxis involving rifampin than in those who did not receive rifampin. Physicians should be aware that the efficacy of tofacitinib could be decreased by chemoprophylactic regimens for tuberculosis.