Objectives The aim of this study was to determine: the prevalence and severity of systemic vasculitis (SV) with or without fibromuscular and/or intimal proliferation (FIP) in various organs of SSc patients.
Methods 12 organs (heart, lungs, liver, spleen, kidneys, pancreas, gastrointestinal tract, adrenal glands, skeletal muscle, peripheral nerve, skin and brain) of 11 patients (female 10, average age 53.6 years; male 1, age of 65 years) with SSc was studied. SSc was confirmed clinically according to the criteria of the ACR. The prevalence of SV with or without FIP in blood vessels of various organs was determined histologically. The “severity” of vasculitis was evaluated by semi-quantitative, visual estimation on a 0 to 3 plus scale.
Results SV with or without FIP was present in all organs (100%) of 11 SSc patients. SV involved several and in some organs only very few blood vessels of different size (including capillaries). The severity of vasculitis (the number of involved vessels) in various organs was diverse. Severity of systemic vasculitis in various organs is summarized in Table 1.
Conclusions The basic pathological process in SSc is systemic non-specific or fibrinoid necrotic vasculitis with or without FIP. SV will be accompanied by focal, multifocal (more or less confluent) or diffuse progressive interstitial fibrosis and sclerosis, depending on the stage of the disease, involving various organs with different severity. The high prevalence of SV with or without FIP and the consecutive fibrosis of the kidney, spleen, lung, pancreas, or heart identify the main targets of autoimmune processes in SSc prior to the more visible skin involvement. The mortality of SSc depends on the localization of SV - with or without FIP - and consecutive fibrosis. Involvement of the kidneys, heart and lung is the most frequent cause of a lethal outcome. Although the pancreas, spleen and gastrointestinal tract, as well as the skin are always involved at death, they are less critical targets of SV. This finding is supported by our previous study in which the leading causes of death were uremia (in 6 of 11 patients due to complex nephropathy) or circulatory failure (in 5 of 11 patients due to endo-myocardial fibrosis or myocardiocytolysis - with or without honeycomb lung). The high prevalence of SV and FIP in the pancreas may cause multifocal acute pancreatitis without lethal outcome. The high prevalence of SV and FIP in the G-I tract (with consecutive sub- mucosal fibrosis) denotes a good site for histological diagnosis of SSc, like the skin biopsy.
Apáthy Άgnes, Bély M: Mortality and histological characteristics of SSc - A retrospective study of 12 autopsy patients (Abstract). Annals Rheum Dis 2007 (Suppl II), 66:198-199. Available from: http://www.abstracts2view.com/eular/
Disclosure of Interest None Declared