Fatal scleroderma renal crisis caused by gastrointestinal bleeding in a patient with scleroderma, Sjögren's syndrome and primary biliary cirrhosis overlap

N. Szigeti, G. Fábiían, L. Czirják

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

A 57-year-old female patient presented with a long history of overlapping autoimmune disease, including limited cutaneous systemic sclerosis, Sjögren's syndrome and primary biliary cirrhosis. Another unusual finding was that the mild skin involvement (limited cutaneous systemic sclerosis, subcutaneous calcinosis) was combined with serious internal organ involvement, including honeycombing and finally scleroderma renal crisis. The most important finding was, that two decades later she developed severe telangiectasia of the gastrointestinal and urinary tract. Furthermore, a specific type of vascular malformation, i.e. gastric watermelon stomach was also found. The chronic gastrointestinal bleeding primarily derived from a water-melon stomach caused protein overload, which provoked the onset of the scleroderma renal crisis that finally led to the patient's death.

Original languageEnglish
Pages (from-to)276-279
Number of pages4
JournalJournal of the European Academy of Dermatology and Venereology
Volume16
Issue number3
DOIs
Publication statusPublished - 2002

Fingerprint

Biliary Liver Cirrhosis
Systemic Scleroderma
Hemorrhage
Kidney
Skin
Stomach
Gastric Antral Vascular Ectasia
Cucurbitaceae
Calcinosis
Telangiectasis
Vascular Malformations
Urinary Tract
Autoimmune Diseases
Gastrointestinal Tract
Water
Proteins

Keywords

  • Primary biliary cirrhosis
  • Scleroderma renal crisis
  • Sjögren's syndrome
  • Systemic sclerosis
  • Telangiectasia

ASJC Scopus subject areas

  • Dermatology
  • Infectious Diseases

Cite this

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abstract = "A 57-year-old female patient presented with a long history of overlapping autoimmune disease, including limited cutaneous systemic sclerosis, Sj{\"o}gren's syndrome and primary biliary cirrhosis. Another unusual finding was that the mild skin involvement (limited cutaneous systemic sclerosis, subcutaneous calcinosis) was combined with serious internal organ involvement, including honeycombing and finally scleroderma renal crisis. The most important finding was, that two decades later she developed severe telangiectasia of the gastrointestinal and urinary tract. Furthermore, a specific type of vascular malformation, i.e. gastric watermelon stomach was also found. The chronic gastrointestinal bleeding primarily derived from a water-melon stomach caused protein overload, which provoked the onset of the scleroderma renal crisis that finally led to the patient's death.",
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author = "N. Szigeti and G. F{\'a}bi{\'i}an and L. Czirj{\'a}k",
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AU - Szigeti, N.

AU - Fábiían, G.

AU - Czirják, L.

PY - 2002

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N2 - A 57-year-old female patient presented with a long history of overlapping autoimmune disease, including limited cutaneous systemic sclerosis, Sjögren's syndrome and primary biliary cirrhosis. Another unusual finding was that the mild skin involvement (limited cutaneous systemic sclerosis, subcutaneous calcinosis) was combined with serious internal organ involvement, including honeycombing and finally scleroderma renal crisis. The most important finding was, that two decades later she developed severe telangiectasia of the gastrointestinal and urinary tract. Furthermore, a specific type of vascular malformation, i.e. gastric watermelon stomach was also found. The chronic gastrointestinal bleeding primarily derived from a water-melon stomach caused protein overload, which provoked the onset of the scleroderma renal crisis that finally led to the patient's death.

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KW - Systemic sclerosis

KW - Telangiectasia

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