Chronic Rhinosinusitis and Recurrent Nasal Polyps in Two Children with IgG Subclass Deficiency and Review of the Literature
Sasawan Chinratanapisit MD*, Prayuth Tunsuriyawong MD**, Pakit Vichyanond MD*, Nualanong Visitsunthorn MD*, Voravich Luangwedchakarn MD***, Orathai Jirapongsananuruk MD*
Affiliation : *Division of Allergy and Immunology, Department of Pediatrics, **Department of Otorhinolaryngology ***Department of Immunology, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand
Chronic rhinosinusitis (CRS) is a chronic inflammatory disorder of mucosa of the nose and the
paranasal sinuses. Two major forms of CRS can be differentiated; CRS with nasal polyps (CRSwNP) and CRS
without nasal polyps (CRSsNP). The pathophysiology and etiology of nasal polyps (NPs) are partly under-
stood. IgG subclass deficiency was shown to be associated with an increased susceptibility to infections.
However, the association between NPs and IgG subclass deficiency has never been reported.
Objectives : To report two cases of recalcitrant CRS and recurrent NPs with IgG subclass deficiency.
Case Report : Two children (6 and 8 year-old boys) were referred to the Pediatric Allergy/Immunology Clinic,
Siriraj Hospital due to a prolonged history of CRS and recurrent NPs. Both of them were treated with aggres-
sive medical (topical and systemic corticosteroids, antibiotics, leukotriene antagonist, nasal irrigation) as
well as surgical therapy, without significant improvement. Immunologic investigation in both patients showed
that IgG, IgA, and IgM level were normal. IgG subclasses level in patient No. 1 were IgG1 1,235 (280-1120)
mg/dl (79%), IgG2 235 (30-630) mg/dl (23.5%), IgG3 27.3 (40-250) mg/dl (1.74%), and IgG4 92.4 (11-620)
mg/dl (5.9%). IgG subclasses level in patient No. 2 were IgG1 1,139 (280-1120) mg/dl (82.5%), IgG2 170 (30-
630) mg/dl (12.3%), IgG3 5.6 (40-250) mg/dl (0.4%), IgG4 65.7 (11-620) mg/dl (4.8%). The diagnosis of CRS
and recurrent NPs with IgG3 subclass deficiency in the first patient and IgG2/IgG3 subclass deficiency in the
second patient were made. Patient No. 1 was given monthly IVIG therapy for the total of 7 courses and
medications were gradually tapered. Currently, the patient is doing well after the cessation of IVIG therapy for
3 months. Patient No. 2 denied the IVIG treatment and was lost to follow up.
Conclusion : We reported two cases of recalcitrant CRS and recurrent NPs in children. Immunologic work up
revealed IgG subclass deficiency. The treatment with monthly IVIG improved CRS and NPs in treated patient
which brought up the possibility of association between NPs and IgG subclass deficiency. Further study on the
direct role of IVIG in NPs will be needed in the future.
Keywords : Chronic rhinosinusitis, Immune deficiency, Immunoglobulin subclass, IVIG, Nasal polyps
