Zusammenfassung
Das benigne Gelenkhypermobilitätssyndrom (BGHS) repräsentiert ein gutartiges Overlap-Syndrom
von nicht lebensbedrohlichen Komponenten der seltenen und besser definierten kongenitalen
mesodermalen Dysplasien: Marfan-Syndrom, Ehlers-Danlos-Syndromen und Osteogenesis
imperfecta. BGHS-Patienten leiden vorwiegend an Arthralgien, Kreuzschmerzen, weichteilrheumatischen
Symptomen, rezidivierenden Gelenkluxationen und extraartikulären Manifestationen wie
Angst, Stress, kutanen Striae, erhöhter Blutungsneigung, Mitralklappenprolaps, Spontanpneumothorax,
Harninkontinenz, Beckenbodeninsuffizienz etc. Die BGHS simuliert oftmals chronisch
entzündlich-rheumatische Erkrankungen. Am Beginn der Therapie steht die richtige Diagnosestellung.
Die Patienten müssen wissen, dass der Arzt ihr Leiden erkannt hat und versteht. Lebensumstände,
welche die Symptome verschlechtern, sollten nach Möglichkeit ausgeschaltet werden,
da dies die Patienten vor unnötigen, potenziell schädlichen Therapien schützt. Jüngst
wurden Arbeiten veröffentlicht, die Prolotherapie als wirksam in der Behandlung nachwiesen.
Physikalische Therapie gilt als eine tragende Säule, evidenzbasierte Empfehlungen
können derzeit infolge des Fehlens von Daten nur durch Anwendung von Analogieschlüssen
gegeben werden. Die unterschiedlichen Ansatzpunkte der physikalischen Therapie werden
umrissen.
Abstract
The benign joint hypermobility syndrome (BJHS) is a hereditary benign overlap disorder
of connective tissue, which incorporates features seen in the Marfan syndrom, Ehlers-Danlos
syndroms and osteogenesis imperfecta. Patients with BJHS usually present arthralgia,
back pain, overuse soft tissue lesions, recurrent joint dislocation or subluxation
and even extraarticular features, e. g. anxiety, stress, skin striae, easy bruising,
mitral valve prolapse, pneumothorax, urinary incontinence and pelvic floor prolapse.
The BJHS often simulates chronic inflammatory rheumatic diseases. There are many ways
in which BJHS patients can be helped: Getting the diagnosis right is a start. Patients
need to know that they have a condition that physicians recognize and understand.
They should be encouraged to seek out factors in their daily activities that seem
to provoke or aggravate their symptoms to prevent patients from treatment with unnecessary,
potentially harmful treatment modalities. There are recent trials showing Prolotherapy
to be an effective treatment. Physical therapy is said to be a mainstay of treatment,
but currently evidence based recommendations can be given only based on conclusions
by analogy. There are different levels of physical intervention, which are outlined
in this paper.
Schlüsselwörter
Rheumatische Erkrankungen - Gelenkhypermobilität - Physikalische Therapie - Beratung
Key words
Rheumatic diseases - hypermobility - joint - physical therapy techniques - medical
history taking - punctures
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Dr. Hans Mayr
Institut für Physikalische Medizin, Rehabilitation und Rheumatologie in der Krankenanstalt
„Sanatorium Hera”
Löblichgasse 16
1090 Wien · Österreich
Email: pmr-rheuma@hera.co.at