Mortality and causes of death across the systemic connective tissue diseases and the primary systemic vasculitides

Abstract

Objectives. Studies assessing relative mortality risks across the spectrum of systemic inflammatory rheumatic diseases are largely missing. In this study, we wanted to estimate standard mortality ratios (SMRs) and causes of death in an ethnically homogeneous cohort covering all major CTDs and primary systemic vasculitides (PSVs). Methods. We prospectively followed all incident CTD and PSV cases included in the Norwegian CTD and vasculitis registry (NOSVAR) between 1999 and 2015. Fifteen controls for each patient matched for sex and age were randomly drawn from the Norwegian National Population Registry. Causes of death were obtained from the National Cause of Death Register, death certificates and hospital charts.Results. The cohort included 2140 patients (1534 with CTD, 606 with PSV). During a mean follow-up time of 9 years, 279 of the patients (13%) died, compared with 2864 of 32 086 (9%) controls (P < 0.001). Ten years after diagnosis, the lowest survival was 60% in dcSSc, 73% in anti-synthetase syndrome (ASS) and 75% in lcSSc. In the CTD group, the highest SMRs were observed in dcSSc (SMR 5.8) and ASS (SMR 4.1). In the PSV group, Takayasu arteritis (SMR 2.5) and ANCAassociated vasculitis (SMR 1.5) had the highest SMRs. Major causes of death were cardiovascular disease (CTD 27%, PSV 28%), neoplasms (CTD 25%, PSV 27%), chronic respiratory disease (CTD 20%, PSV10%) and infections (CTD 9%, PSV 16%). Conclusion.We observed premature deaths across the spectrum of CTDs and PSVs, with highest SMRs in dcSSc and ASS. The overall mortality was highest in the CTD group.

The Importance of Demographic and Geographical Factors on the Incidence and Outcome of Systemic Small Vessel Vasculitis Associated with Anti-Neutrophil Cytoplasmic Antibodies

“…In a recent study based on the Norwegian systemic connective tissue disease and vasculitis registry (NOSVAR) based at the Rheumatology Department in Oslo, the SMR for AAV was found to be 1.5 (95% CI 1.0-2.1) [234]. The reason for the discrepancy between this result and the results in Paper III and the study from the Norwegian Kidney Biopsy Registry is probably that fewer of the patients in NOSVAR had severe renal involvement.…”

“…Development of LN is associated with worse outcome in terms of both morbidity and mortality. The standardized mortality ratio (SMR) in patients with SLE is 1.5-3.0 [195,[232][233][234], and in patients with LN it is 5.9-6.6 [235,236]. In a population-based Norwegian SLE cohort, the SMR was 3.8 in LN patients compared with 1.7 in non-LN patients.…”

Familial aggregation of longevity in giant cell arteritis and polymyalgia rheumatica

“…Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely related, chronic immune-mediated rheumatic diseases, limited to elderly patients. The long-term mortality is at least not increased in GCA and PMR [ 1 – 5 ] or even decreased compared to the general population [ 6 – 9 ]. It is surprising because these diseases share several risk factors of increased mortality with other immune-mediated rheumatic diseases.…”

“…The genetic background (high prevalence of these diseases in special populations, association with special HLA antigen) and immunological profiles (with strongly pronounced autoinflammatory component) of PMR/GCA patients’ are unique [ 6 , 14 , 15 ], therefore, we hypothesize that these might correlate with some survival advantage. Explanation of this potential advantage would imply a need for including some genetic or behavioural factors.…”

Takayasu Arteritis in Chronic Migraine

“…It is more prevalent in Central and South America, Africa, India, and the Far East [4]. In a recent study, the mortality rate of TA was estimated to be 2.5 × 100,000 inhabitants due to the association of this arteritis with premature deaths [5,6].…”