Cytomegalovirus Disease in Patients with Common Variable Immunodeficiency: Three Case Reports

Abstract

Common variable immunodeficiency (CVID) is the most frequent clinically relevant primary immunodeficiency and shows enormous heterogeneity in clinical presentation. Despite clinical immunodeficiency, opportunistic infections are not a typical manifestation of CVID. A retrospective study of 32 patients followed up for 335 patient-years was performed to determine the frequency of cytomegalovirus (CMV) disease. Symptomatic CMV infection was documented in 3 CVID patients. Patients No. 1 and 2 suffered from CMV pneumonia, with complications due to atypical mycobacteriosis in patient No. 1. Patient No. 3 suffered from CMV enteritis. A history of cancer and chronic hepatitis C infection (patient No. 1), immunosuppressive therapy for interstitial lung disease (patient No. 2) and serious enteropathy complicated with malnutrition (patient No. 3) may have contributed to the complications despite only mild abnormalities in T-cell subpopulations. The direct detection of CMV in bronchoalveolar lavage, stool or tissue samples was the most beneficial diagnostic laboratory method, whereas the detection of CMV DNA in blood did not produce positive results. Adequate treatment of CMV disease led to significant clinical improvement in all 3 patients. The frequency of CMV disease appears to be higher than previously described. In our experience, the probability of opportunistic infections in CVID patients increases with secondary comorbidities and their management.

Neurologic Complications of Common Variable Immunodeficiency

“…In general, however, the benefit of higher or more frequent dosing of IgG for viral illnesses associated with CVID is not well investigated. Multiple other factors, such as environmental (43) Adenovirus meningoencephalitis (2) [25] Bacterial meningitis (22) [1,[26][27][28][29] BKV encephalitis and optic neuritis (1) [30] CMV encephalomyelitis (1) [31] Echovirus meningoencephalitis (1) [25] Histoplasmosis (1) [28] HSV encephalitis (3) [9] Mumps encephalitis (1) Poliovirus (1) [32] Progressive Multifocal Leukoencephalopathy (6) [3][4][5][6][7][8] Toxoplasma encephalitis (2) [33,34] Varicella angiitis (1) [35] Varicella zoster encephalitis (1) [1] West Nile meningitis (1) [36] Autoimmune/Inflammatory (13) optic neuritis (1) [37] myelitis (4) [10,11,38,39] limbic encephalitis (1) [12] Guillian Barre Syndrome (1) [40] granulomatous mass (4) [41][42][43][44]45] chronic inflammatory demyelinating polyneuropathy (1) [39] acute disseminated encephalomyelitis (2) [14,46] Unknown etiology (10) encephalopathy (4...…”

Autoimmunity in Primary Antibody Deficiencies

“…CVID is the most common clinically significant PAD, defined by recurrent bacterial infections (and rarely by virus infections) and hypogammaglobulinemia [60,61,62,63,64]. The cellular alterations in CVID comprise a spectrum of B- and T-cell abnormalities, including defects in B-cell differentiation into plasma cell and memory B cells, defects in Treg and Breg cells, accelerated T-cell apoptosis, and abnormality in cytokine production secondary to gene polymorphisms [6,65,66,67,68].…”

Pan-Digestive Tract Colonization by Cytomegalovirus in Common Variable Immunodeficiency

“…We read with interest the paper by Kralickova et al [1] on cytomegalovirus (CMV) infections in common variable immunodeficiency (CVID). We fully agree with the authors' conclusions that infections due to CMV are more frequent than previously reported.…”

“…In CVID patients, viral infections, such as those due to norovirus and CMV, have been reported to be of pathogenic importance in gastrointestinal (GI) damage in up to 5% of patients [2,3,4]. In the paper by Kralickova et al [1], the frequency of systemic CMV reached 10%, one third with localization in the GI tract. Information on the frequency of infectious complications of the GI tract in CVID comes mainly from one French study on 252 patients where Giardia lamblia was the predominant infectious agent, at least in patients with undetectable serum IgA levels [5].…”