Abstract
Churg Strauss syndrome (CSS) is an uncommon systemic small vessel vasculitis usually associated with bronchial asthma, blood an d tissue eosinophilia and frequently with pulmonary infiltrates. Mannose-binding lectin (MBL) is one of the important serum proteins activating complement system through lectin pathway.
It als o participates in opsonization of infection agens, apoptosis and in modulation of inflammation. Its deficiency predisposes the patient to repe ated respiratory infections and higher susceptibility to other infectious and autoimmune diseases.
The role of MBL could be substituted by the o ther parts of the immune system and that is why the immunodeficiency in adult patients is not profound. The course of the autoimmune disease migh t be worsen by MBL deficiency.
In the accessible literature we found no reports on MBL in patient with CSS. We describe a 34-year-old female diagnosed with CSS who previously suffered from atypical bronchial asthma for 14 years.
The di agnosis was based on pulmonary manifestations (asthma, pulmonary granulomatous infiltrations), laboratory and histological findings (eosino philia, high ESR, granulomatous eosinophilic inflammation). MBL deficiency was accidentally revealed in this patient.
Genotype LXA/LXA assoc iated with low levels of MBL was detected by DNA analysis. Treatment with prednison resulted in dissolutions of many disease symptoms and stabilization of the pulmonary disease.
We consider the association of MBL deficiency with CSS to be coincidental. The patient is, however, s uffering from frequent infections of mucous surfaces in which the MBL deficiency may play a role.