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Akuter Mesenterialinfarkt bei Polychondritis

Acute mesenteric ischemia in a patient with relapsing polychondritis

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Zusammenfassung

Wir berichten über eine 43-jährige Patientin mit einer rezidivierenden Polychondritis, die mit einer progredienten Verschlechterung des Allgemeinzustandes, Leistungsschwäche und Gewichtsverlust zur stationären Aufnahme kam. 2 Jahre zuvor wurde aufgrund der Befundkonstellation einer nasalen Chondritis mit Veränderungen der Nasenkontur, einer Laryngitis, einer Skleritis und einer unspezifischen Allgemeinsymptomatik (Fieber, Entzündungszeichen, Leistungsinsuffizienz) die Diagnose einer Polychondritis gestellt. Daraufhin erfolgte eine immunsuppressive Therapie initial mit Azathioprin, nachfolgend mit Methotrexat in Kombination mit Ciclosporin sowie Glucocorticoiden in unterschiedlicher Dosierung. Während des stationären Aufenthaltes bei uns kam es zu einer über Tage progredienten abdominellen Schmerzsymptomatik und Verschlechterung des Allgemeinzustandes mit der Entwicklung eines akuten Abdomens mit Ileus, Darmperforation und Peritonitis. Im Rahmen der akuten chirurgischen Intervention erfolgte nach Revaskularisation der A. mesenterica superior durch einen aorto-mesenterialen Bypass die Resektion eines ischämisch-nekrotischen Ileums mit Stomaversorgung. Ursache der Ischämie waren ein vaskulitischer Verschluss der A. mesenterica superior und eine Stenose des Truncus coeliacus. Die Polychondritis ist häufig assoziiert mit anderen Autoimmunerkrankungen, insbesondere unterschiedlichen vaskulitischen Manifestationen. Der dargestellte Fall einer chronisch progredienten mesenterialen Ischämie vaskulitischer Genese ist eine seltene Manifestation der Polychondritis und sollte bei ungewöhnlicher abdomineller Symptomatik in die differentialdiagnostischen Erwägungen einbezogen werden.

Summary

We report about a 43-year old woman with relapsing polychondritis, admitted with progressive malaise, fatigue, anorexia and profound weight loss. Two years ago a nasal chondritis with characteristic changes of the nasal profil, scleritis, laryngitis and unspecific general symptoms (fever, fatigue, signs of a systemic inflammation) developed and relapsing polychondritis was diagnosed. The patient was treated initially with azathioprine followed by methotrexate in combination with ciclosporine and glucocorticoids. During the hospitalization her condition slowly worsened, and crampy abdominal pain developed subsequently with massive tenderness, rebound and guarding. The clinically presumed diagnosis of ileus and peritonitis was radiomorphologically confirmed with signs of enteric perforation and a laparatomy was performed. A complete occlusion of the A. mesenterica superior and stenosis of the truncus coeliacus were diagnosed followed by a revascularization with an aorto-mesenteric bypass and subsequent resection of the necrotic ischemic ileum. An association with various autoimmune disorders including vasculitic syndromes is well known for relapsing polychondritis. The case demonstrated a progressive mesenteric ischemia with the acute exacerbation caused by a vasculitic mesenterial occlusion. The unusual presentation should be considered in the differential diagnosis of uncommon abdominal symptoms during the course of relapsing polychondritis.

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Authors and Affiliations

  1. Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Universitätsklinikum Charité Berlin, Berlin

    O. Schultz, G. R. Burmester & F. Buttgereit

  2. Chirurgische Klinik, Franziskus-Krankenhaus Berlin, Berlin

    R. I. Rückert

  3. Medizinische Klinik mit Schwerpunkt Rheumatologie und Klinische Immunologie, Universitätsklinikum Charité Berlin, Schumannstr. 20–21, 10117, Berlin, Germany

    O. Schultz

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  1. O. Schultz
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  4. F. Buttgereit
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Schultz, O., Rückert, R.I., Burmester, G.R. et al. Akuter Mesenterialinfarkt bei Polychondritis. Z. Rheumatol. 65, 63–68 (2006). https://doi.org/10.1007/s00393-005-0001-8

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  • DOI: https://doi.org/10.1007/s00393-005-0001-8

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