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AB0241 Type and incidence of systemic vasculitis in progressive systemic sclerosis
  1. M. Bély1,
  2. Ά. Apáthy2
  1. 1Department of Pathology, Policlinic of The Order of The Brothers of Saint J
  2. 2Department of Rheumatology, Saint Margaret out-patients clinic Budapest III., Budapest, Hungary


Objectives The aim of this postmortem study was to determine the type and incidence of systemic vasculitis (SV) with or without fibromuscular and/or intimal proliferation and successive adventitial fibrosis (FIP) in blood vessels of different size in patients with progressive systemic sclerosis (SSc).

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 and FIP in blood vessels of different size was determined histologically.

Results (1) SV with or without FIP was observed in all of 11 SSc patients with different prevalence.

(2) Two types of vasculitis were observed: non-specific vasculitis with or without FIP, and fibrinoid necrotic vasculitis with or without FIP. Various types of vasculitis existed in all patients simultaneously in different vessels or combined in the same vessel.

(3) Vessels of all sizes: arterioles (a), small arteries (A), medium size arteries (AA), venules (v), small veins (V), and medium size veins (VV) were involved, with varying frequency.

Severity of systemic vasculitis in relative % of the total sum is summarized in Table.

Conclusions Non-specific and fibrinoid necrotic vasculitis are characteristic of SSc. Both types may be accompanied by fibromuscular and/or intimal proliferation and successive adventitial fibrosis (FIP).

Non-specific and fibrinoid necrotic vasculitis may exist simultaneously in different vessels, or may be present in the same vessel.

Both types of vasculitis may be accompanied by obliterative thrombosis and focal (globular) calcification.

Acute, subacute, subchronic and chronic stages of non-specific and fibrinoid necrotic vasculitis existed simultaneously in different or in the same vessels, corresponding to the recurrent and progressive nature of these pathological processes (1). The dominancy of chronic stages refers to a rarely relapsing, slowly progressive process (1)

Blood vessels of all sizes may be affected by vasculitis; however, the dominant involvement of arterioles and small arteries is characteristic of SSc.

Systemic vasculitis is accompanied by focal, multifocal (more or less confluent) or diffuse progressive interstitial fibrosis and sclerosis, depending on the stage of the disease.

Histological differences of vasculitis or vascular changes may help in identification of autoimmune disorders. Dominant non-specific vasculitis or relatively high frequency of fibrinoid necrotic vasculitis with fibromuscular and/or intimal proliferation in its typical form is supporting the diagnosis of SSc. The recurrent nature of vasculitis and successive interstitial fibrosis and sclerosis side by side in different (acute, subacute, subchronic, chronic) stages of the pathological process confirm the autoimmune origin of vasculitis.

  1. Bély M, Apáthy Άgnes: Characteristics of Systemic Vasculitis in Progressive Systemic Sclerosis. Annals Rheum Dis 2007 (Suppl II), 66:201. Available from:

Disclosure of Interest None Declared

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