Background Systemic vasculitis (SV) with or without chronic structural changes of the blood vessels is the basic pathological process in rheumatoid arthritis (RA) and in progressive systemic sclerosis (SSc), followed by consecutive ischemic (degenerative and/or necrotic) processes and fibrosis in various organs.
Objectives The aim of this study was to determine post mortem the mortality of SV in RA and in SSc, respectively.
Methods Twelve organs (heart, lung, liver, spleen, kidneys, pancreas, gastrointestinal tract, adrenal glands, skeletal muscle, peripheral nerve, skin and brain) of 36 RA and of 11 SSc in-patients with SV were studied.
RA and SSc were confirmed clinically according to the criteria of the ACR. SV was determined histologically. The basic disease, complication(s), and causes of death were determined on the basis of clinical data and autopsy findings, confirmed in a detailed review of extensive histological material (50-100 tissue blocks from each patient).
Results In RA patients the number of organs involved by vasculitis varied; not all investigated organs were involved and the prevalence (incidence and severity) of vasculitis in each organ was different. In SSc patients SV was present in each of the investigated organs, with different incidence and severity.
SV led to death in 19 (52.8%) of 36 RA patients: in one case due to coronary arteritis with a large anteroseptal myocardial infarct; in 11 cases SV caused multifocal microinfarcts of the myocardium (myocardiocytolysis); in 3 cases vasculitis of the pulmonary and bronchial arterioles and small arteries led to vasculogenic rheumatoid pneumonia with disseminated (multifocal) lobular-sublobular pneumonia (1). In 2 cases SV caused brain infarcts (2), in one patient it caused necrosis of the intestines; in another case thrombosis of the main renal artery led to renal insufficiency and incipient renal necrosis and was the cause of death.
SV with or without fibromuscular and/or intimal proliferation and consecutive interstitial fibrosis led to death in all (100%) of 11 SSc patients. Six patients died of uremia (due to complex nephropathy) and 5 patients died of circulatory failure (due to endo-myocardial fibrosis or myocardiocytolysis - with or without honeycomb lung) (3).
Conclusions Our data indicate that in RA the heart is the most endangered vital organ by SV, whereas in SSc the kidneys, heart and lung are the most critical targets, and other organs seem to be less threatened.
The course of the disease, more precisely mortality due to SV, depends on the location of the affected vessels (and involved organs), but not on the severity of SV (number of involved vessels). Mild or moderate SV in the heart, lung, kidneys or brain may be lethal even in an early stage of the disease.
Bély M, Apáthy Άgnes: Rheumatoid Arthritis with Multifocal Migratory Vasculogenic Pneumonia Refractory to Antibiotics. Ann Rheum Dis 2012;71 (Suppl 3):176. http://www.abstracts2view.com/eular/
Kiss G, Kelemen J, Bély M, Vértes P: Clinically diagnosed fatal cerebral vasculitis in long-standing juvenile rheumatoid arthritis. Virchows Arch 448, 381-383 (2005).
Apáthy Άgnes, Bély M: Mortality and histological characteristics of SSc. Annals Rheum Dis 66. Suppl.II. 198-199 (2007) http://www.abstracts2view.com/eular/
Disclosure of Interest None declared
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