Cystic Fibrosis with Cytomegalovirus Induced Pneumonitis Case Series

Cystic fibrosis (CF) is a multisystem disease characterized by recurrent respiratory tract infections and progressive respiratory failure. It remains one of the most common life-threatening autosomal recessive condition in children .In cystic fibrosis (CF) it has been found thatan incidence of up to five times of Viral Respiratory Infection ( VRI ) per patient-year. Viral infections in this population are associated with increased respiratory symptoms and disease progression. Up to 64% of pulmonary exacerbations in pediatric CF populations are associated with respiratory viruses, yet the literature on VRI in adults with CF is sparse. Cytomegalovirus (CMV) is a DNA virus and a member of the herpes family of viruses, which includes herpes simplex virus, Epstein–Barr virus (EBV) and varicella–zoster virus. Like these other viruses, once primary infection has occurred, CMV establishes itself in the host in a latent form with periodic episodes of reactivation occurring throughout life. CMV is a common infection with most individuals acquiring infection at some time. Both primary and no primary infections are associated with viral shedding in urine, saliva, semen, cervical secretions, breast milk and other body fluids. In healthy individuals, symptoms of CMV infection are often mild and nonspecific, or absent altogether, and rarely cause serious illness. However, CMV infection can be life threatening to immune compromised individuals, such as those with advanced HIV infection, transplant recipients or very premature infants.

CMV infection is an uncommon cause of respiratory complications in immune competent patients, although CMV pneumonia is being recognized more frequently in immune competent adults and children. It is a leading cause of morbidity and mortality in immune suppressed patients as well as very premature and congenitally infected infants. In lung transplant recipients, it is associated with acute syndromes and graft rejection. There are no available data on the prevalence and impact of CMV in patients with CF. We describe four cases of primary CMV infection causing significant pulmonary exacerbation in Pediatric with CF. The presentation in last three cases out of four reported here was remarkably similar, although in case one seroconversion had not yet occurred and was diagnosed after failure to respond to broad-spectrum antibiotics and sweat chloride test. The characteristic features were lymphocytosis, intermittent fever unresponsive to intravenous (I.V).antibiotics and a persistent weight loss but last three declines in lung function. In our unit, viral throat swabs are routinely taken in all patients presenting with acute pulmonary exacerbations and lymphocytosis is not associated with respiratory viral infections such as respiratory syncytial virus, influenza and par influenza. The diagnosis of primary CMV infection was based on the presence of CMV IgM and CMV PCR. Last two patients had significant viral loads and low CMV IgG avidity. The first three patients with the highest lymphocyte counts were treated with valganciclovir, and showed significant clinical improvement last three patients return of lung function back to baseline. Currently, there is little evidence using ganciclovir or valganciclovir for treatment of severe CMV infection in immunocompetent patients. In summary, we have demonstrated that respiratory viruses especially CMV Viruses are common pathogens in Pediatric with CF and are strongly linked to pulmonary exacerbations. So we should have ultimately leaded to effective new treatments to prevent virus-induced exacerbations in patients with CF.

Keywords: Cystic Fibrosis, Cytomegalovirus, Pneumonitis.