Purpose To examine the prevalence of isolated IgA anti-β2Glycoprotein I (anti-β2GPI) positivity and the association of these antibodies, and a subgroup that bind specifically to domain IV/V of β2GPI, with clinical manifestations of the Antiphospholipid Syndrome (APS) in three patients groups. The pathogenicity of IgA anti-β2GPI was also evaluated in a mouse model of thrombosis. Methods Patients with systemic lupus erythematosus (SLE) from a multiethnic, multicenter cohort (LUpus in MInorities, NAture versus nurture [LUMINA]) (n=558), patients with SLE from the Hopkins Lupus Cohort (n=215), and serum samples referred to the Antiphospholipid Standardization Laboratory (APLS) (n=5,098) were evaluated. IgA anti-β2GPI titers and binding to domain IV/V of β2GPI were examined by enzyme-linked immunosorbent assay (ELISA). CD1 mice were inoculated with purified IgA anti- β2GPI antibodies, and surgical procedures and ELISAs were performed to evaluate thrombus development and tissue factor (TF) activity. Results A total of 198 patients were found to be positive for IgA anti-β2GPI isotype, and 57 patients were positive exclusively for IgA anti-β2GPI antibodies. Of these, 13 of 23 patients (56.5%) in the LUMINA cohort, 17 of 17 patients (100%) in the Hopkins cohort, and 10 of 17 patients (58.9%) referred to APLS had at least one APS-related clinical manifestation. Fifty-four percent of all the IgA anti-β2GPI positive serum samples reacted with domain IV/V of anti-β2GPI, and 77% of those had clinical features of APS. Isolated IgA anti-β2GPI positivity was associated with an increased risk for arterial thrombosis (p<0.001), venous thrombosis (p=0.015) and all thrombosis (p<0.001). The association between isolated IgA anti-β2GPI and arterial thrombosis (p=0.0003) and all thrombosis (p=0.0003) remained significant after adjusting for other risk factors for thrombosis. In vivo mouse studies demonstrated that IgA anti-β2GPI antibodies induced significantly larger thrombi and higher TF levels compared to controls. Conclusion Isolated IgA anti-β2GPI positive titers may identify additional patients with clinical features of APS. Testing for these antibodies when other antiphospholipid (aPL) tests are negative and APS is suspected is recommended. IgA anti-β2GPI antibodies directed to domain IV/V of β2GPI represent an important subgroup of clinically relevant antiphospholipids.

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ObjectiveVenous thromboembolism (VTE) is a major cause of mortality and morbidity in hospitalized patients, particularly those with autoimmune disorders. The Nationwide Inpatient Sample (NIS) database was analyzed to determine trends in the rate of hospitalization, mortality from VTE, epidemiology, and outcomes in hospitalized patients with systemic lupus erythematosus (SLE) to assess its impact.MethodsThe 2003‐2011 NIS database of the Healthcare Cost and Utilization Project was queried to identify all adults (age 18 years and older) hospitalized with SLE and VTE. Demographic characteristics and in‐hospital outcomes of this population were compared with those of patients with SLE without a VTE diagnosis. A multivariate logistic regression analysis was used to obtain the adjusted odds ratio (OR).ResultsThe total number of hospitalized patients with SLE was 299 595, of whom 9175 (3.06%) had VTE. After adjusting for potential confounders, compared with those without VTE, patients with SLE and VTE had significantly higher inpatient mortality (5% vs. 2.0%; OR 2.35 [95% confidence interval (CI) 2.10‐2.62]; P < 0.001), greater disability at discharge (34% vs. 26%; OR 1.53 [95% CI 1.46‐1.62]; P < 0.001), a longer length of stay (LOS) by 3.57 days, and higher cost of hospitalization by $25 400. In this database, patients with SLE and VTE were younger and of male sex. Also, African American race and a higher number of comorbidities were associated with an increased risk of VTE in patients with SLE.Conclusion VTE in hospitalized patients with SLE is associated with significantly higher inpatient mortality, greater disability at discharge, an increased LOS, and higher cost of hospitalization. This cross‐sectional study helps with quantifying the risk of VTE in hospitalized patients with SLE and provides information on the immense human and material cost this complication leads to. These data can be very useful in the development and implementation of appropriate prophylactic strategies in the high‐risk population with SLE.

Tumid lupus erythematosus (TLE) is a rare variant of cutaneous lupus erythematosus. Clinically, it lacks typical changes found in discoid lupus and antinuclear antibodies (ANA) levels are elevated in only 10% of the patients. Coexistent systemic lupus erythematosus (SLE) has been reported to be rare, and literature shows only a few case reports. We present a case of coexistent tumid lupus and SLE. We present a case of a 48-year-old Caucasian female who presented with chronic facial rash, photosensitivity, intermittent oral ulcers, joint pain with morning stiffness, and unintentional weight loss. Laboratory studies showed positive ANA at 1:640, elevated erythrocyte sedimentation rate, positive anticardiolipin immunoglobulin (Ig) G, anticardiolipin IgM, and anti-beta-2 glycoprotein IgM. Skin biopsy of the rash showed a superficial and deep dense lymphocytic infiltrate with mucin deposition, histopathology favoring tumid lupus. The patient was diagnosed with TLE with SLE and was started on hydroxychloroquine with improvement in her rash. Ultraviolet light and certain medications have been proven to play a role in the pathogenesis of tumid lupus. It usually responds to photoprotection, topical treatment, or oral antimalarial therapy.

A 65-year-old woman with a medical history of well-controlled scalp psoriasis, chronic kidney disease stage 3, hypothyroidism, and chronic obstructive pulmonary disease was referred to rheumatology for increasing joint pains. The patient had a history of small cell lung cancer (SCLC) with lymph node involvement for 3 years. She had tried and failed carboplatin with etoposide, paclitaxel, topotecan, and chest and cranial radiation therapy. Nivolumab was started for SCLC at the dose of 240 mg every 2 weeks, and after 12 weeks, she started noticing joint pains in her hands, feet, and knees. She presented to the rheumatology office after using From the