SESSION TITLE: Infectious Disease Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: We present a 36-year-old-male admitted for community acquired pneumonia with a history of idiopathic hypertrophic pachymeningitis. Further investigation revealed extensive history of multiple respiratory tract infections, paired with a chest x-ray demonstrating bronchial wall thickening, proving chronic inflammatory changes. This increased suspicion of underlying immunodeficiency, which was confirmed by low IgG and IgM levels.
CASE PRESENTATION: Patient is a 36-year-old male with history of idiopathic hypertrophic pachymeningitis who presented with fevers, chest pain, cough and malaise. Chest x-ray showed signs of multifocal pneumonia as well as bronchial wall thickening. What first seemed to be a common community acquired pneumonia was the tip of the iceberg once further history was pieced together from the patient and his extensive past medical records. He had been treated for sinusitis, pneumonia, and meningitis at least every two years since the age of five. Once the diagnosis of this rare type of meningitis was made, all the subsequent presenting symptoms, respiratory system included, were attributed to meningitis. This frustrated the patient greatly. The history of recurrent infections and the presence of bronchial wall thickening on the chest x-ray prompted a workup for an immunodeficiency. The patient’s total IgG level (327 mg/dl) and IgG subclass levels, IgG1 (174 mg/dl), IgG2 (97 mg/dl), and IgG4 (1 mg/dl), were very low and his IgM level (76 mg/dl) was within the lower limit of normal. He was diagnosed with common variable immunodeficiency (CVID) and was discharged with a follow-up for intravenous immunoglobulin infusion.
DISCUSSION: CVID is a syndrome encompassing a heterogeneous collection of disorders that present with recurrent bacterial infections due to impaired B-cell differentiation and thus defective immunoglobulin production (1). These hypogammaglobulinemias include reduced IgG levels, and reduced or low normal IgA or IgM levels. Patients present between the ages of 20-40 with recurrent respiratory tract infections, including sinusitis, otitis media, bronchitis, and pneumonia, as well as bronchiectasis and interstitial lung diseases (2). Lastly, 73% of patients with CVID acquire structural pulmonary complications, most frequently presenting as bronchiectasis and bronchial wall thickening, the later being the final piece that triggered work up and diagnosis of CVID in our patient.
CONCLUSIONS: Recurrent pneumonia, although easily perceived as run-of-the-mill, can be a harbinger of an underlying immune-deficient state, as in our case. Careful history and a broad differential diagnosis is key to unmasking such states.
Reference #1: Tam JS. and Routes JM. Common variable immunodeficiency. Am J Rhinol Allergy. 2013;27(4):260-265.
Reference #2: Touw CM, van de Ven AA, de Jong PA, Terheggen-Lagro S, Beek E, Sanders EAM, van Montfrans JM. Detection of pulmonary complications in common variable immunodeficiency. Pediatr Allergy Immunol. 2010;21(5):793-805.
DISCLOSURE: The following authors have nothing to disclose: Shalin Kothari, Miruna Carnaru, Wajihuddin Syed, Amit Dhamoon
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