Allergy in patients with lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease caused by proliferation of abnormal smooth muscle-like cells, which can result in polycystic lungs destruction, formation of renal angiomyolipomas, lymphadenopathy, cystic lymphangiomas, abdominal lymphangioleiomyomas, chylothorax, and chyloperitoneum. The disease affects mainly women in childbearing age and occurs in two forms: as a sporadic lymphangioleiomyomatosis (sLAM) or associated with tuberous sclerosis complex (TSC/LAM).Pulmonary symptoms of LAM are frequently masked by allergy or asthma. The aim of the study was to present the differences between patients with and without allergic symptoms. Material: The study group comprised of women 89 with sLAM, and 25 women and one man with TSC/LAM. The assessment was performed at the time of first presentation in our Department. Results: 31 patients with LAM (29 with sLAM and 2 with TSC/LAM; p=0.011) displayed allergy symptoms. There were no differences between groups in mean age, smoking habits, time of diagnosis delay, presence of: pneumothorax in anamnesis lymphangioma, renal angiomyolipoma, and chylothorax, and mean serum concentration of VEGF-D. LAM patients with allergy had more severe lung impairment than patients without allergy [mean FVC(l) 3.26 vs. 3.28; p=0.65, mean FEV1(1.95 vs. 2.47l; p=0.005, mean DL,CO (4.42 vs. 5.84 mL/min/mmHg; p=0.007]. Allergic patients were more frequently treated with sirolimus than those without (81% vs. 49%; p=0.002). Conclusions: LAM patients with allergy more frequently: suffered on sporadic LAM, had severe lung function impairment, and were treated with mTOR inhibitors than patients without allergy.