R. Ploier et al., IMMUNOLOGICAL ASPECTS OF A CHILD WITH IDIOPATHIC PULMONARY HEMOSIDEROSIS AND CELIAC-DISEASE, Klinische Padiatrie, 210(6), 1998, pp. 409-412
A case report of a girl aged 3.5 years affected by the extremly rare c
ombination of idiopathic pulmonary haemosiderosis (IPH) and coeliac di
sease (CD) is presented. It is the 13(th) such case that has been publ
ished over the last 25 years and only the 7(th) to be reported in a ch
ild. We believe that the concurrence of these two diseases is not coin
cidental, because a gluten-free diet had beneficial effects on the pul
monary symptoms not only in our case but also in other such patients.
However, the pathogenetic relation between IPH and CD remains unclear.
Although circulating immune complexes were detected in our patient's
serum, there was no evidence of their putative damaging effect on the
basement membrane of the alveolar capillaries. Furthermore, no IgA dep
osits could be demonstrated in alveolar basement membranes. Therefore
the hypothesis that there is a reaction between IgA reticulin or endom
ysial antibodies and an alveolar basement membrane antigen with consec
utive structural damage is unlikely.