Abstract
BackgroundRare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis. Granulomas also occur in inborn errors of immunity (IEI) such as common variable immunodeficiency (CVID) with B cell maturation defects and hypogammaglobulinemia. CVID shares various features with sarcoidosis and drug-induced secondary hypogammaglobulinemia; careful consideration of differential diagnosis between these conditions is warranted.Case presentationA 29-year-old female with epilepsy developed dysphagia, dyspnea and impaired exercise tolerance. Obstruction caused by swollen lingual tonsil and edema in the epiglottis and arytenoid mucosa were found. Lingual tonsil and epiglottis biopsies displayed non-necrotizing granulomas. There was no evidence of viral, bacterial, mycobacterial or fungal infections. Chest X-ray, computerized tomography of chest and ultrasound of neck and abdomen remained unremarkable. Positron emission tomography/computed tomography (PET/CT) showed laryngeal enhancement. Empiric antimicrobials combined with prednisolone were insufficient to control her disease. In immunological evaluation, the patient had normal counts of B and T cells. Proportions of CD27+ memory B cells (30.3%) and IgD−IgM−CD27+ switched memory B cells (7.2%; normal range 6.5–29.2%) were normal. Percentage of activated CD21low B cells was high (6.6%; normal range 0.6–3.5%). IgG (3.5 g/L; normal range 6.77–15.0 g/l) and all IgG subclass concentrations were low. Anti-polysaccharide responses were impaired, with 3/10 serotypes reaching a level of 0.35 µg/ml after immunization with Pneumovax®. The findings were consistent with hypogammaglobulinemia resembling CVID, possibly secondary to antiepileptic medication. Her dyspnea and dysphagia responded favorably to subcutaneous IgG and rituximab.ConclusionsTonsillar granulomas can be the presenting and only clinical feature of B cell deficiency, highlighting the diversity of symptoms and findings in primary or secondary immunodeficiencies.
Highlights
Rare tonsillar granulomas may be caused for example by infections, malignancies or sarcoidosis
Hodgkin’s lymphoma, toxoplasmosis, fungal infection and squamous cell carcinoma are associated with pharyngeal granulomas [1, 2]
Our patient presented with unusual granulomatous inflammation of the lingual tonsil and epiglottis causing dysphagia and airway obstruction; her condition shares features with primary and druginduced B cell deficiency as well as sarcoidosis
Summary
Tonsillar granulomatous inflammation is rare, most commonly caused by tuberculosis or sarcoidosis. No evidence for acute or chronic viral, bacterial, mycobacterial or fungal infections, including hepatitis B and C, human immunodeficiency virus and tularemia, was found Due to her swollen lingual tonsil and laryngeal mucosa causing airway obstruction, she was hospitalized and received empiric cefuroxime (1.5 g thrice daily) and methylprednisolone (75 mg once daily, height 173 cm, weight 63 kg, prednisolone dose 1.2 mg/kg) intravenously. Due to symptomatic upper airway obstruction, dysphagia, lack of evident infection and unsatisfactory response to prednisolone treatment and possible granulomatous CVID, the patient received immunoglobulin replacement and rituximab therapy (Mabthera® 100 mg, 200 mg and 500 mg on three consecutive days combined with 100 mg hydrocortisone and 1 g paracetamol), with a favorable response within. She continues to receive subcutaneous immunoglobulin replacement with serum IgG levels in the range of 10 to 12 g/l
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