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Intracardiac Thrombi in Morbus Adamantiades-Behçet in Two Swedish Patients

Raffaele Da Mutten1, Alexander Borg1,2, Katerina Chatzidionysiou1,2

  • 1Division of Rheumatology, Department of Medicine Solna, Karolinska Institutet, 17176 Stockholm, Sweden.

Journal of Clinical Medicine
|August 26, 2023
PubMed

Insights

Morbus Adamantiades-Behçet (MAB) is a rare inflammatory disease. Two Swedish patients with MAB presented with uncommon cardiac and neurological symptoms, highlighting rare initial manifestations of this condition.

Area of Science:

  • Rheumatology and Immunology
  • Cardiology
  • Neurology

Background:

  • Morbus Adamantiades-Behçet (MAB) is a rare inflammatory disorder with typical symptoms including oral/genital aphthosis, erythema nodosum, and vasculopathy.
  • Cardiac involvement is rare in MAB, occurring in approximately 2% of cases.
  • MAB prevalence is higher along the Silk Road, with lower rates in Scandinavia (0.64-4.9 per 100,000).

Observation:

  • This report details two rare cases of MAB in Swedish patients.
  • Both patients, aged 62 and 35, presented with intracardial thrombi.
  • One patient also experienced cerebrovascular insults causing visual field deficits and peripheral nerve involvement.

Findings:

  • The presented cases highlight unusual initial manifestations of MAB, including significant cardiac and neurological involvement.
  • These findings are notable given the patients' Scandinavian origin and the rarity of such symptoms as primary MAB presentations.
  • The cases underscore the diverse and sometimes atypical clinical spectrum of Morbus Adamantiades-Behçet.

Implications:

  • These rare cases expand the understanding of MAB's clinical presentation, particularly in non-endemic regions.
  • Recognizing uncommon initial symptoms is crucial for timely diagnosis and management of MAB.
  • Further research into MAB's varied manifestations may improve diagnostic strategies and patient outcomes.
Keywords:
Morbus Adamantiades–Behçetautoinflammationintracardiac thrombusrheumatology

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