April 2026 to July 2026
Author: Giovanni Fulvio
Paraproteinemias in Sjogren disease: cryoglobulinemia and serum
monoclonal gammopathy identify distinct clinical phenotypes and
differential lymphoma susceptibility
Longhino et al. evaluated the clinical and lymphoproliferative significance of cryoglobulinemia and serum monoclonal gammopathy in 1,202 patients with Sjögren disease (SjD) from the multicentre Italian GRISS registry. Cryoglobulinemia was associated with a predominantly vasculitic phenotype, whereas coexistence of cryoglobulinemia and monoclonal gammopathy identified a distinct lymphoproliferative phenotype and was strongly associated with lymphoma (OR 6.30, 95% CI 2.66–14.92; p<0.001); neither abnormality alone was associated with lymphoma.
Target trial emulation of biologic and targeted synthetic disease-modifying antirheumatic drugs for rheumatoid arthritis-associated interstitial lung disease
McDermott et al. used Medicare data (2006–2019) to emulate target trials comparing abatacept, Janus kinase inhibitors (JAKi), interleukin-6 inhibitors (IL-6i), and tumour necrosis factor inhibitors (TNFi) with rituximab in rheumatoid arthritis-associated interstitial lung disease (RA-ILD). No treatment significantly differed from rituximab for the composite of respiratory hospitalization, lung transplantation, or death. However, mortality was lower with abatacept (HR 0.84, 95% CI 0.71–0.98) and JAKi (HR 0.62, 95% CI 0.40–0.96).
Pulmonary manifestations of granulomatosis with polyangiitis and microscopic polyangiitis
Junek et al. characterized pulmonary involvement in antineutrophil cytoplasmic antibody-associated vasculitis (AAV) using a multicentre longitudinal cohort of 1,026 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Pulmonary manifestations occurred in 81.3% overall, including 62.7% at disease onset, and were present in 41.4% of relapse episodes. Nodules/cavities predominated in GPA and diffuse alveolar haemorrhage in MPA; 17.4% of affected patients developed permanent pulmonary damage.
Increased incidence of sudden cardiac death in systemic sclerosis: data from a nationwide cohort study
Fairley et al. nvestigated the incidence and risk factors of sudden cardiac death (SCD) in 1,708 patients with systemic sclerosis (SSc). Over 10,650 person-years, 32 SCD events occurred (1.9%; 300/100,000 person-years), 3–9 times higher than published community estimates. Coronary artery disease (HR 2.28, 95% CI 1.04–5.00), reduced left ventricular ejection fraction (HR 2.89, 95% CI 1.06–7.90), and smoking history (HR 2.80, 95% CI 1.21–6.49) were independently associated with SCD.
VEXAS syndrome and cancer: Insights about a possible “Tip of the Iceberg”. Ambidirectional data from the international AIDA network registries
Gavioli et al. evaluated cancer risk in VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome using international registry data, comparing patients with VEXAS with controls with other autoinflammatory diseases. Among participants aged >60 years (90 VEXAS, 174 controls), VEXAS was associated with nearly doubled cancer risk (RR 1.93, 95% CI 1.03–3.60; p=0.036). Cancer was also associated with relapsing polychondritis and the UBA1 p.Met41Thr variant.
Giovanni Fulvio, MD PhD
Country: Italy
Giovanni is a rheumatologist and researcher at the University of Pisa, where he completed a PhD in Clinical and Translational Sciences.
His research is focused on salivary gland ultrasonography and the role of imaging techniques in rheumatology, particularly in the diagnosis and management of Sjögren’s syndrome. He completed his fellowship at the Rheumatology Unit, University of Pisa, where he also serves as a tutor in salivary gland ultrasonography.
Giovanni is actively involved in European and national professional networks. He is a member of the Italian Society of Rheumatology, ERN ReCONNET, including the Sjögren’s disease group and the Transversal Working Group on Pregnancy and Family Planning, and EMEUNET. He has also served on the EMEUNET Newsletter Sub-Committee since 2024.