File:Morbus Fabry Skin Rash 01.jpg
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DescriptionMorbus Fabry Skin Rash 01.jpg |
English: Skin rash during infusion of recombinant α-galactosidase A in a patient with positive IgE antibodies to agalsidase beta: In year 2002, a 39-year-old male Fabry patient (GLA mutation p.Ala121Pro) was initially treated with agalsidase beta (1 mg/kg EOW). ERT was changed to agalsidase alfa (0.2 mg/kg EOW) after 18 months due to poor tolerance (mild laryngeal edema, urticaria and chills during infusions). Two years later, a rash appeared on both arms during agalsidase alfa infusions. In 2007, concomittant deterioration of kidney function on agalsidase alfa (mGFR decreased from 85 to 70 mL/min/1.73 m2) led to switch ERT back to agalsidase beta. No data was obtained with respect to antibodies (IgG or IgE) to agalsidase alfa. After 1 year of agalsidase beta therapy, extensive skin rash and bronchospasm appeared during the infusions despite premedication (hydroxyzine, paracetamol and oral steroids) and minimal infusion rates (0.05 - 0.2 mg/min) and kidney function kept on deteriorating (mGFR eq. 54 mL/min/1.73 m2). The patient tested positive for IgE to agalsidase beta and ERT was discontinued. Mutation p.Ala121Pro is not responsive to the ASSC deoxygalactonojirymicin [424]. Both rechallenge protocol and concomitant use of immunosuppressive therapy and ERT are currently being considered.
Deutsch: Hautausschlag bei einem 39-jährigen männlichen Patienten nach der Infusion von α-galactosidase A. Der Patient hat Immunglobulin E gegen Agalsidasebeta gebildet. Während den Infusionen bildeten sich trotz der Verabreichung von oralen Steroiden, Paracetamol und Hydroxyzin großflächige Hautausschläge und Bronchospasmen. Dabei verschlechterte sich die Nierenfunktion weiter. Die Enzymersatztherapie wurde daher abgebrochen. Bei dem Patienten liegt eine Mutation vom Typ p.Ala121Pro vor, die sich nicht mit dem in der klinischen Entwicklung befindlichen Migalastat behandeln lässt. Zum Zeitpunkt der Aufnahme wurde eine Kombination von Immunsuppression und Enzymersatztherapie für die weitere Behandlung diskutiert. |
Date | article published: 22 November 2010 [1] |
Source | D. P. Germain: Fabry disease. In: Orphanet journal of rare diseases Vol. 5, 2010, 30, PMID 21092187. PMC 300961. (Review) |
Author | D. P. Germain |
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current | 12:47, 17 September 2011 | 1,200 × 914 (122 KB) | Kuebi (talk | contribs) | {{Information |Description={{en|Skin rash during infusion of recombinant α-galactosidase A in a patient with positive IgE antibodies to agalsidase beta: In year 2002, a 39-year-old male Fabry patient (GLA mutation p.Ala121Pro) was initially treated with |
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