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A patient, an adolescent male, presented to us with complaints of recurrent respiratory tract infections since childhood. Differentials considered were cystic fibrosis (CF), bronchial asthma with allergic bronchopulmonary aspergillosis (ABPA), primary ciliary dyskinesia (PCD) and primary immunodeficiency disorders. Sweat chloride test, total IgE and Aspergillus fumigatus specific serum IgE and IgG levels were normal ruling out CF and ABPA. Nasal nitric oxide (NO) screening test showed reduced NO levels, and high-speed video microscopy of nasal scrapings showed stiff beating cilia with reduced ciliary beat frequency confirming the diagnosis of PCD. Immunodeficiency workup showed reduced serum IgG, IgA and IgM, when repeated on two separate occasions when the patient was not harbouring any active infection, suggestive of pan-hypogammaglobulinaemia. Thus, a diagnosis of coexistent PCD and pan-hypogammaglobulinaemia was made. Detection of immunodeficiency disorders is important in patients with PCD as they may benefit from immunoglobulin replacement.
A patient, an adolescent male, presented to us with complaints of recurrent respiratory tract infections since childhood. Differentials considered were cystic fibrosis (CF), bronchial asthma with allergic bronchopulmonary aspergillosis (ABPA), primary ciliary dyskinesia (PCD) and primary immunodeficiency disorders. Sweat chloride test, total IgE and Aspergillus fumigatus specific serum IgE and IgG levels were normal ruling out CF and ABPA. Nasal nitric oxide (NO) screening test showed reduced NO levels, and high-speed video microscopy of nasal scrapings showed stiff beating cilia with reduced ciliary beat frequency confirming the diagnosis of PCD. Immunodeficiency workup showed reduced serum IgG, IgA and IgM, when repeated on two separate occasions when the patient was not harbouring any active infection, suggestive of pan-hypogammaglobulinaemia. Thus, a diagnosis of coexistent PCD and pan-hypogammaglobulinaemia was made. Detection of immunodeficiency disorders is important in patients with PCD as they may benefit from immunoglobulin replacement.
IgG4-related sclerosing disease (IgG4-RSD) is characterized by extensive IgG4-positive plasma cell and T-lymphocyte infiltration of various organs. However, the nasal cavity is an extremely rare site of involvement. The authors experienced three cases of igG4-RSD in the nasal cavity. A 54-year-old woman presented with a nasal cavity mass on endoscopic exam and computed tomography (CT) revealed not only a hypoenhancing mass in the right nasal cavity but also a pituitary gland macroadenoma. Endoscopic removal and endoscopic TSA were performed. A 20-year-old man diagnosed with chronic sinusitis, underwent endoscopic sinus surgery. A 26-year-old woman showed thickening of the nasal septum and cribriform plate, and an infiltrating soft tissue lesion in right the alveolar process on CT and an endoscopic biopsy was performed. Pathologic specimens of all cases showed IgG4-positive plasma cell infiltration, consistent with IgG4-RSD. Serum IgG4 levels were checked in two of the cases, and the results were within normal range. All cases were further treated with methylprednisolone and all showed clinical improvement.KEY WORD: IgG4-related sclerosing disease.
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