Case Presentation: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia and heterogenous manifestations. We present a 67-year-old woman admitted inpatient for fluctuating mental status changes along with intermittent fevers and diaphoresis for several months. Her medical history is notable for congestive heart failure, obstructive sleep apnea, and chronic abdominal pain in the setting of ventral mesh hernia repair (2019) with abdominal fistulization and prior abscess formation. She denied having recurrent infections or recent travels. On presentation, she was afebrile and hemodynamically stable. Labs were notable for leukocytes of 5.9 x10(9)/L with lymphocytes of 16%, monocytes of 14%, and eosinophils of 7%; C-reactive protein at 34.7 mg/L; and low selenium at 106 mcg/L. All other laboratory values, including liver and thyroid function tests and infectious panel (urinalysis, blood cultures, (1–3)-β-D-glucan assay, HIV, viral panel) were unremarkable. On physical exam, she had three vesicular lesions on her right arm, right lower quadrant abdominal tenderness, and significant superficial wound dehiscence at her prior hernia repair site. CT scan of the abdomen and pelvis demonstrated prior hernia mesh repair without obstruction; there were no findings of acute intraabdominal pathology. At this time, her diagnosis remained in question. Upon further review of her symptoms, including abnormal wound healing, recurrent fevers, and diaphoresis, an immunologic etiology was suspected, prompting further workup. Immunoglobulin G (IgG) was found low at 285mg/dL, IgA normal at 218mg/dL, IgM borderline low at 42mg/dL. IgG stratification demonstrated low IgG1 at 169mg/dL, IgG2 at 50mg/dL, and IgG3 at 5.3mg/dL; IgG4 was normal at 8.1mg/dL. Immunophenotyping showed a significant decrease in total memory B cells (CD19+ CD27+) with decreases in both non-switched memory (CD27+ IgM+ IgD+) and switched memory (CD27+ IgM- IgD-) B cell subsets. Evaluation for underlying malignancy via serum protein electrophoresis and monoclonal gammopathy returned negative. Given her findings, we diagnosed CVID and started her on intravenous immunoglobulin therapy while inpatient, with plans to monitor IgG values in the outpatient setting.
Discussion: CVID is a multifaceted disorder characterized by hypogammaglobulinemia of IgG, IgA, and/or IgM. More than 90% of patients have recurrent bacterial infections as their presenting symptom. However, as in our case, the absence of recurrent infections does not exclude the diagnosis of CVID. Rather, we found that skin changes, namely the chronic nonhealing surgical scar and vesicular lesions, to be her presenting sign. Furthermore, consideration of her mental status changes and intermittent fevers, which are often frequently encountered inpatient complaints, prompted further workup leading to the diagnosis of her underlying CVID. Her fevers, nonetheless, were suspected to be unrecognized, self-limited infections. It is imperative to keep all signs and symptoms in mind, despite their possible ambiguity, as clinical presentations may vary greatly among patients, especially so while inpatient. Other CVID manifestations may include chronic lung disease, autoimmune disorders, malignancies, or gastrointestinal inflammatory disease.
Conclusions: The unpredictable and varied presentation of CVID can pose challenges to inpatient management; it requires a thorough evaluation based on each patient’s signs and symptoms.