A NEW DIAGNOSIS OF CVID IN A PEDIATRIC PATIENT PRESENTING WITH STREPTOCOCCUS PNEUMONIAE MENINGITIS

Abstract

Introduction Common Variable Immune Deficiency (CVID) can present with a range of organ system involvement, including normal number of B-cells but decreased IgG, eventual decline in IgA and/or IgM, and poor/absent response to vaccinations. Case Description We report a 15-year-old female, admitted during travel, with psoriasis, recurrent otitis media, pneumonia, and sinusitis presenting with headache, fever, and emesis for two days. CSF meningitis panel was positive for Streptococcus pneumoniae. Immunologic workup revealed hypogammaglobulinemia with subsequent increase in serum IgG levels after IVIG administration. Lymphocyte subset and B-cell panel revealed decreased switched memory B-cells. Total complement and lymphocyte proliferation to mitogens were normal. Outpatient records from lab work obtained one month prior to presentation demonstrated profound hypogammaglobulinemia and the absence of specific antibody titers to vaccines, consistent with CVID. Her hospital course was complicated by acute mental status changes and diffuse cerebral edema, endotracheal tube fungal infection, posterior reversible leukoencephalopathy syndrome, multiple arterial and venous thrombi, and nephrolithiasis. Six months following discharge she is receiving monthly IVIG with no sequelae. Discussion Patients with CVID are at increased risk for autoimmune disorders (such as psoriasis), granulomatous disease of the lungs, bowel lymphoid nodular hyperplasia, enteropathy, and malignancies (lymphoma, gastric carcinoma). While CVID is most commonly diagnosed in the second or third decade of life, 20% of patients are diagnosed earlier than age 20. Close surveillance of pediatric patients presenting with recurrent sinopulmonary infections is needed, as early recognition is crucial in order to initiate immunoglobulin replacement and prevent long-term sequelae or death from severe infections. Patient's Immunoglobulin Levels Prior to and During Admission The patient's significant hypogammaglobulinemia found upon review of outpatient lab work performed one month prior to presentation can also be seen at the onset of meningitis. At the time of presentation, the patient had neither been diagnosed nor treated for CVID. After IVIG administration the patient's IgG levels increased as expected leading to clinical improvement.