Vasculitis associated with immune checkpoint inhibitors—a systematic review

Clinical Rheumatology - Recent experimental and genetic studies have implicated the role of programmed cell death protein 1 (PD-1), programmed cell death protein-ligand 1 (PDL-1), and cytotoxic T...     

Prosthetic valve endocarditis presenting as loss of the metallic click sound

Prosthetic valve endocarditis is a significant infection. It is often serious, and may result in a complicated course leading to valvular malfunction. We present the case of a 50-year-old male with an aortic Medtronic Hall valve, who presented with loss of his normal metallic click. A transthoracic echocardiogram confirmed the diagnosis of endocarditis and of an aortic-root abscess. Blood cultures were positive for nutritionally deficient Streptococcus. He underwent successful surgery and later was discharged. Patients with mechanical heart valves are often bothered by the metallic sound. It can interfere with their daily life. However, the loss of the click may indicate valvular dysfunction, dehiscence of the prosthesis, and/or tissue infection with abscess formation.     

Patient-reported outcomes in ANCA-associated vasculitis. A comparison between Birmingham Vasculitis Activity Score and routine assessment of patient index data 3

The objective of this study was to determine health-related quality of life (HRQoL) in patients with ANCA-associated vasculitis (AAV) as measured by the “routine assessment of patient index data 3” (RAPID3) and whether RAPID3 is correlated with disease activity as determined by the Birmingham Vasculitis Activity Score (BVAS). Data from patients at an academic institution vasculitis clinic seen between Jan 2010 and Jan 2012 were collected using chart review. BVAS and RAPID3 scores were calculated at each patient visit. RAPID3 was compared between patients in remission (BVAS?=?0) and patients with active disease (BVAS?>?=1) at all visits for four consecutive visits, when data available, at least 3 months apart during the period mentioned. Robust generalized estimating equations (GEE) in linear regression models evaluated associations between the RAPID3 and BVAS over all available observations, adjusting for intra-subject correlations. Thirty-four patients were included in the study, 26 had granulomatosis with polyangiitis (GPA), five microscopic polyangiitis (MPA), and three eosinophilic granulomatosis with polyangiitis (EGPA). Patients at first visit had impaired HRQoL as measured by RAPID3 [6.8 (3.1–12.6)]. The median RAPID3 scores were higher in patients with active disease as compared to patients in remission (7.0 vs. 3.0, p?=?0.115; 8.8 vs. 1.0, p?=?0.011; 6.1 vs. 2.0, p?=?0.032; and 11.7 vs. 2.0, p?=?0.128 for visits 1, 2, 3, and 4, respectively). In longitudinal GEE models incorporating all observations there was a strong association between the RAPID3 (per 1 unit) and BVAS (per 1 unit) [β 0.21 (0.10, 0.32) p?<?0.001]. RAPID3 can be used to measure HRQoL in patients with AAV. RAPID3 correlated significantly with BVAS. RAPID3 can discriminate between disease states in AAV. This instrument may help document patient experience and add to clinical decisions.     

Thrombotic complications after intravenous immunoglobulin therapy in two patients

Although intravenous immunoglobulin (IVIg) generally is considered a safe treatment for various autoimmune and inflammatory disorders, rare cases of thrombosis may occur. We describe two patients who experienced thrombotic complications associated with IVIg therapy. A 54-year-old woman with idiopathic thrombocytopenia received IVIg 1 g/kg/day for 2 days. While receiving her infusion on day 2, she had an ischemic stroke with hemiparesis; 3 days later she developed deep vein thrombosis. A 33-year-old woman with Evans' syndrome received IVIg 400 mg/kg/day for 5 days and developed deep vein thrombosis 1 week after therapy was completed; she then received warfarin. Six months later, she received an additional course of IVIg for recurrent hemolytic anemia; 1 day later she died of pulmonary thromboembolism. We suggest that IVIg may promote thrombosis by increasing blood viscosity, activating platelets, or causing vasospasm and should be administered with caution.     

Toll-like receptors in systemic lupus erythematosus; prospects for therapeutic intervention

Recent experimental and clinical studies have placed new emphasis on the role of the innate immune system in SLE. Nucleic acid-containing immune complexes activate the innate response by engaging specific Toll-like receptors (TLRs) and promote the generation of autoantibodies. Pharmacologic modulation of TLR-directed pathways may offer new therapeutic approaches for the treatment of SLE.