Case Presentation:
GM a 35yo M with a history of pneumonia one year prior presented to our hospital with 6 weeks of cough productive of yellow‐whitish sputum and worsening shortness of breath. He endorsed a 20 lb weight loss over the prior 6 months, but denied night sweats or hemoptysis. On presentation he was febrile (102 F), tachycardic (133 bpm), his blood pressure was 100/61 and he was mildly hypoxemic (93% RA). His exam revealed bronchial breath sounds in the right lower lobe. His laboratory workup was significant for a WBC of 20k, an albumin of 2.4 and a total protein of 4.3. Chest Xray revealed an infiltrate in the right lower lobe. The patient was started on ceftriaxone and azithromycin for treatment of CAP. His admission blood cultures grew Hemophilus influenzae. Sputum was negative for AFB.
A rapid HIV test was non‐reactive. Qualitative immunoglobulin levels were sent which revealed severe deficiencies in IgA (undetectable), IgG (25 mg/dL) and IgM (undetectable). The allergy and immunology service was consulted and flow cytometry was performed which revealed normal numbers of both T and B cells but reduced numbers of NK cells. Titers for tetanus and hemophilus influenza were non‐reactive. A CT scan of the chest showed consolidation in the right middle and lower lobes and extensive bronchiectasis. A diagnosis of common variable immunodeficiency (CVID) was made and the patient was started on monthly IVIG.
Discussion:
CVID is the second most common defect in humoral immunity, with prevalence estimated to be as high as 1 in 10,000 individuals (1). There are often delays in diagnosis as long as 4‐9 years after initial presentation (1). Patients with CVID often present with recurrent upper and lower respiratory infection as well as infections of the GI tract. Autoimmunity and malignancy are also more common in CVID than the general population. Treatment of CVID with IVIG results in a marked reduction in the number of hospitalizations for recurrent pneumonia (2,3).
Pneumonia is a common reason for hospitalization and immunodeficiency should be suspected in cases of recurrent respiratory tract infections. Diagnosis should prompt referral to allergy and immunology specialist for treatment with IVIG as well as appropriate screening for gastroenterological and pulmonary complications including H. pylori, ILD, bronchiectasis and malignancy (4).
Conclusions:
CVID is relatively common and is generally under‐diagnosed. Hospitalists can play an important role in diagnosing immunodeficiency as many patients are hospitalized with the complications of these disorders. A careful history should be taken with regard to recurrent bacterial infections with a low threshold for initiating an appropriate workup.
References
Immunol Allergy Clin N Am 2008;28:367‐386.
J Allergy Clin Immunol 2002;109:1001‐4.
J Microbiol Immunol Infect 2010;43(1):11‐17.
Int Arch Allergy Immunol 2009;150:311‐324.