Delta F508 and Henoch-Schönlein purpura

Question

Does Henoch-Schönlein purpura occur often in the context of CF? My son, who is now 18 years old, does not seem to get rid of it.

Also: are there any new drugs for Delta F 508?

Many thanks and kind regards.

Answer

Dear questioner,

vasculitides, to which the Henoch-Schönlein purpura belongs as well, have been described in CF patients, although they are not common but can occur as complications in CF. However, these are always individual cases and, understandably, there are no studies on this.

Several different diseases belonging to the group of vasculitides involve the small vessels (both the small veins and arteries and also the capillaries), but the larger vessels can be affected as well. The clinical picture varies greatly as well; skin, kidneys and other organs can be involved. It is assumed that the cause is an inadequate immune answer of the body with formation of immune complexes and infiltration of inflammatory cells.

Therapy does not differ from that of vasculitides in non-CF individuals, and if it really is Henoch-Schönlein purpura, the prognosis is not bad. Unfortunately you do not mention your son's symptoms and how the diagnosis was made.

Concerning your second question: there are a few new therapeutic approaches that are currently reviewed in studies. However, it would go beyond the scope of this forum to answer this question here.

Kind regards
Prof. Joachim Bargon

02.08.2012