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Systemic vasculitides comprise a collection of rare and heterogeneous disorders capable of impacting any organ and system, posing a considerable burden of mortality and comorbidity. As with previous annual reviews of this series, this review will offer a critical overview of the latest literature on pathogenesis, biomarkers, and treatment options in both small- and large-vessel vasculitis.

Systemic vasculitides are heterogeneous disabling diseases characterised by chronic inflammation of the blood vessels potentially leading to tissue destruction and organ failure. The recent COVID-19 pandemic has had a significant impact on the epidemiology and management of patients with systemic vasculitis. In parallel, new insights have been provided on systemic vasculitis pathogenetic mechanisms, possible new therapeutic targets, and newer glucocorticoid-sparing treatments with better safety profiles. As in the previous annual reviews of this series, in this review we will provide a critical digest of the most recent literature regarding pathophysiology, clinical manifestations, diagnostic tools and treatment options in small- and large-vessel vasculitis focusing on precision medicine in vasculitis.

Objectives: To investigate the clinical and laboratory patterns of HCV-unrelated cryoglobulinaemic vasculitis (CV), and the factors influencing its outcome., Methods: Prospective study of all anti-HCV and HCV-RNA negative patients with CV who have been observed since January 2004 in 17 centres participating in the Italian Group for the Study of Cryoglobulinaemias (GISC)., Results: 175 enrolled were followed up for 677 person-years. The associated conditions were primary Sjögren's syndrome (21.1%), SLE (10.9%), other autoimmune disorders (10.9%), lymphoproliferative diseases (6.8%), solid tumours (2.3%) and HBsAg positivity (8.6%), whereas 69 patients (39.4%) had essential CV. There were significant differences in age (p<0.001), gender (p=0.002), the presence of purpura (p=0.005), arthralgia (p=0.009), liver abnormalities (p<0.001), sicca syndrome (p<0.001), lymphadenopathy (p=0.003), splenomegaly (p=0.002), and rheumatoid factor titres (p<0.001) among these groups. Type II mixed cryoglobulins were present in 96 cases (54.9%) and were independently associated with purpura and fatigue (odds ratio [OR]4.3; 95% confidence interval [CI] 1.8-10.2; p=0.001; and OR2.8; 95%CI 1.3-6.3; p=0.012). Thirty-one patients died during follow-up, a mortality rate of 46/1000 person-years. Older age (for each additional year, adjusted hazard ratio [aHR] 1.13; 95%CI 1.06-1.20; p<0.001), male gender (aHR 3.45; 95%CI 1.27-9.40; p=0.015), type II MCG (aHR 3.31; 95%CI 0.09-1.38; p=0.047) and HBsAg positivity (aHR 7.84; 95%CI 1.20-36.04; p=0.008) were independently associated with greater mortality., Conclusions: HCV-unrelated CV is a multifaceted and often disabling disorder. The associated conditions influence its clinical severity, giving rise to significantly different clinical and laboratory profiles and outcomes.

Objectives: To study the incidence and prevalence of primary systemic vasculitides (PSV) in the Costa del Sol region (southern Spain) and to compare the major epidemiological studies in PSV with the results obtained in our area., Methods: Retrospective study including permanent residents ≥14 years (or older) diagnosed with PSV at the Hospital Costa del Sol (Marbella, Spain) between 1994 and 2010. Epidemiological data were collected and the annual incidence rate during the study period and the prevalence in 2010 were calculated per million population, except for GCA, which was estimated per 100,000 population >50 years., Results: Seventy-four adult patients were diagnosed with PSV, representing an annual incidence of 15.8 (95%CI 12.2-19.4) patients/million population. These diagnoses included 29 (39.1%) giant cell arteritis (GCA), 5 (6.7%) Takayasu's arteritis (TKA), 3 (4%) poly-arteritis nodosa (PAN), 29 (39.1%) antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) [10 (13.5%) granulomatosis with polyangiitis (GPA) (Wegener), 16 (21.6%) microscopic polyangiitis (MPA) and 3 (4%) eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss)], 7 (9.4%) IgA vasculitis (Henoch-Schönlein) (IgAV) and one (1.3%) cryobulinaemic vasculitis (CV). The annual incidence and 2010 prevalence for each of the PSV, respectively, were: GCA: 2.2/12.2; TKA: 1.1/10.5; PAN: 0.6/2.6; AAV: 6.2/44.8 (GPA: 2.1/15.8; MPA: 3.4/23.8; EGPA: 0.6/5.3); IgAV: 1.5/7.9; and CV: 0.2/0., Conclusions: The first epidemiological study of PSV in southern Spain corroborates their infrequency, with GCA and AAV as the PSV most often diagnosed. In southern Spain, the incidence and prevalence of PSV are lower than in northern Spain and in countries in the Northern Hemisphere.

Herewith we provide our annual digest of the recent literature on systemic vasculitis in which we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis, and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular, we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinaemia.

Herewith we provide a critical digest of the recent literature on systemic vasculitis. In this manuscript, we reviewed all the articles published during the last 12 months on large-, medium- and small-vessel vasculitis and selected the most relevant studies regarding the epidemiology, pathogenesis and management of systemic vasculitis. In particular we focused the attention on giant cell arteritis, ANCA-associated vasculitis and cryoglobulinemia.