Recurrent and Sustained Viral Infections in Primary Immunodeficiencies

Abstract

Viral infections are commonplace and often innocuous. Nevertheless, within the population of patients with primary immunodeficiencies (PIDDs), viral infections can be the feature that drives a diagnostic evaluation or can be the most significant morbidity for the patient. This review is focused on the viral complications of PIDDs. It will focus on respiratory viruses, the most common type of viral infection in the general population. Children and adults with an increased frequency or severity of respiratory viral infections are often referred for an immunologic evaluation. The classic teaching is to investigate humoral function in people with recurrent sinopulmonary infections, but this is often interpreted to mean recurrent bacterial infections. Recurrent or very severe viral infections may also be a harbinger of a primary immunodeficiency as well. This review will also cover persistent cutaneous viral infections, systemic infections, central nervous system infections, and gastrointestinal infections. In each case, the specific viral infections may drive a diagnostic evaluation that is specific for that type of virus. This review also discusses the management of these infections, which can become problematic in patients with PIDDs.

Keywords: enterovirus; herpes; morbidity; norovirus; papillomavirus; primary immunodeficiency; virus.

Figure 1

Chest radiograph of a term male infant with X-linked severe combined immune deficiency and RSV pneumonitis, which was rapidly fatal despite adjunctive use of IVIG and inhaled ribavirin. He was treated with an infusion of maternal haplo-identical hematopoietic stem cells at 18 days of life. He was admitted at 10 days old (A) and died at 27 days old (B) due to worsening respiratory status. Note the absent thymus.

Figure 2

Bilateral plantar warts on a patient who had experienced 5 years of immune suppression for a cardiac transplant. Deep palmoplantar warts such as those in the top panel are referred to as myrmecila and can be painful. The small black markings are characteristic and represent small blood vessels that have grown into the exophytic lesion. Photo credit: Marissa J. Perman, MD.