Isolated immunoglobulin A (IgA) deficiency presenting with cytomegalo virus pneumonia

Abstract

A 5-month-old infant with an uneventful perinatal period was presented with lower respiratory tract symptoms and abnormal X-ray findings of 1-month duration. At admission, the baby was in respiratory distress requiring oxygen therapy and supportive care. Despite optimal supportive care and antibiotic therapy, he continued to be symptomatic necessitating further investigations for nonresolving pneumonia. His bronchoalveolar lavage and urine were positive for cytomegalovirus (CMV) and workup for immunodeficiency showed undetectable immunoglobulin A(IgA) levels. Rest of the workup was inconclusive. We investigated the mother for a carrier status, but the workup was negative for CMV and/or other intrauterine infections. In view of severe CMV-associated lung disease, the baby was started on ganciclovir therapy for 6 weeks on which he recovered completely. Although the associated isolated IgA deficiency may be an incidental finding in this case, it is indeed intriguing and requires further research.

Keywords: Cytomegalovirus, immunoglobulin A deficiency, persistent pneumonia

How to cite this article:
Bhatt GC, Sankar J, Dubey N K. Isolated immunoglobulin A (IgA) deficiency presenting with cytomegalo virus pneumonia. Ann Trop Med Public Health 2013;6:343-4

 

How to cite this URL:
Bhatt GC, Sankar J, Dubey N K. Isolated immunoglobulin A (IgA) deficiency presenting with cytomegalo virus pneumonia. Ann Trop Med Public Health [serial online] 2013 [cited 2020 Aug 8];6:343-4. Available from: https://www.atmph.org/text.asp?2013/6/3/343/121003

 

Introduction

Severe cytomegalovirus infection (CMV) may be congenital [1] or acquired as in premature infants [2] and immunocompromised patients.The immunoglobulin deficiencies commonly reported with CMV infection are human immunodeficiency virus (HIV) disease, hyper-IgM syndrome, Chediak-Higashi syndrome, etc. [3] Isolated immunoglobulin A(IgA) deficiency has never been previously reported to be associated with CMV infection. This 5-month-old infant was an intriguing case, who presented with a nonresolving pneumonia due to undiagnosed CMV infection and was found to be IgA deficient. We chose to report this case to draw attention to this rare association of CMV infection with isolated IgA deficiency.

Case Report

A 5-month-old infant is a product of a nonconsanguineous marriage with an uneventful perinatal period presented with history of cough and breathing difficulty for 30 days. The child was apparently well before this, and the family history was unremarkable. For these complaints, he was treated at a local hospital for 2 weeks with intravenous (IV) antibiotics. Two days after his discharge, he presented to our hospital with severe respiratory distress. At admission, the child was sick looking with a heart rate of 172/min, respiratory rate of 86/min with subcostal and intercostal retraction, and SpO 2 80% at room air. Auscultation of the chest revealed bilateral crepitations and rhonchi. Rest of the systemic examination was normal. The child was immediately started on oxygen therapy, nebulization with salbutamol, and empiric antibiotic therapy with IV antibiotics following which child gradually improved over the next 2 days. In view of his prolonged symptomatology and no response to an appropriate course of antibiotics previously, the child was investigated further. His chest X-ray revealed bilateral hyperinflated lung fields.Hematological investigations revealed microcytic anemia (hemoglobin 9.6 g/dl) with leucopenia (total leucocyte count 3300/mm 3 ; lymphocytes 50%). His immunological profile showed undetectable IgA levels with normal immunophenotyping for T-cell and B-cell markers. Gastroesophageal reflux (GER) scan showed mild GER. Rest of the workup such as echocardiography and viral titers were normal. Underlying lung and airway problems were ruled out by detailed history and confirmed by negative sweat chloride test and computed tomographic scan of chest.

After a week of antibiotics and antireflux therapy, the child again developed severe respiratory distress with shock. The blood gas estimation at this point showed mixed metabolic and respiratory acidosis. He was ventilated and managed for shock with fluids and inotropes. Antibiotics were upgraded to cefoperazone with sulbactum and vancomycin in view of a possibility of nosocomial sepsis. A search was made for possible cause of sudden deterioration, and cultures were sent for bacterial and virological studies.His bronchoalveolar lavage and urine were positive for CMV by reverse-transcriptase polymerase chain reaction (RT-PCR). Workup for intrauterine infections was positive for CMV (immunoglobulin M [IgM]) only. We investigated the mother for a carrier status, but the workup was negative for CMV and/or other intrauterine infections. The child was started on ganciclovir (5 mg/kg, twice daily for first 2 weeks and 5 mg/kg once daily for next 4 weeks) on which the child’s condition improved over the next 3-4 days. The child was extubated after 9 days of ventilation. Ganciclovir was continued for 6 weeks, and baby was discharged after 22 days of hospital stay and on follow-up after 3 months of discharge,he is asymptomatic and doing well.

Discussion

CMV infection may be acquired during delivery, through contact with infected cervical secretions or after birth. [1],[2] Transmission sources of CMV include saliva, breast milk, cervical and vaginal secretions, urine,stools, blood, and tissue or organ transplants. Transmission occurs by direct person-to-person contact, but indirect transmission is possible via contaminated fomites. [1],[2],[3] In the index case, we are noncommital about the source of CMV as similar case reports of congenitally acquired CMV infection have been previously reported. [4]

The diagnosis of CMV pneumonitis is usually based on symptoms of fever, dyspnea, hypoxemia, and diffuse infiltrates on chest radiograph in combination with the detection of CMV in the bronchoalveolar lavage fluid. Active CMV pneumonitis is best confirmed by virus isolation from bronchoalveolar washings which was diagnostic in the index case. [3]

Although cell-mediated immunity is the primary mechanism of host defense against CMV infection, humoral immunity is required to eliminate the infection successfully. Persistent pneumonia due to CMV has been reported in patients with severe combined immunodeficiency, [5] X-linked hyper-IgM syndrome, [6] and common variable immunodeficiency, [7] but no cases of persistent pneumonia due to isolated IgA deficiency have been reported.

The indications of treatment in CMV infections are usually limited to immunocompromised patients, HIV infections, and congenital CMV infections. However, Ganciclovir may be considered in cases of monophasic CMV meningoencephalitis, ocular involvement, and severe lung involvement. [8]

Our patient was started on ganciclovir therapy for three reasons:(1) as the source of infection was not clear (congenital or acquired),(2) presence of severe pulmonary disease (requiring mechanical ventilation), and (3) presence of isolated IgA deficiency. The dosage used in our patient was based on the dosage recommended by Petros, et al[8] in patients with severe lung involvement.

The prognosis of patients with isolated IgA deficiency is good if it is not associated with a significant disease, and the mainstay of treatment in isolated IgA deficiency is the treatment of associated diseases. [9]

Thus, in conclusion,CMV infection may be associated with isolated IgA deficiency, and outcome is good with early antiviral therapy in cases of persistent pneumonia due to the same.

References

 

1. Hamprecht K, Maschmann J, Vochem M, Dietz K, Speer CP, Jahn G. Epidemiology of transmission of cytomegalovirus from mother to preterm infant by breastfeeding. Lancet 2001;357:513-18.
2. Stagno SB. Cytomegalovirus infections. In: Remington JS, Klein JO, editors. Infectious Diseases of the Fetus and Newborn Infant. 6 th ed. Philadelphia: WB Saunders; 2005. p. 739-81.
3. Stagno S. Cytomegalovirus. In: Behrman RE, Kliegman RM, Jenson HB, Stanton FB, editors. Nelson Textbook of Pediatrics. 18 th ed. Philadelphia: WB Saunders;2008. p.1377-9.
4. Meier J, Lienicke U, Tschirch E, Krüger DH, Wauer RR, Prösch S. Human cytomegalovirus reactivation during lactation and mother-to-child transmission in preterm infants. J Clin Microbiol 2005;43:1318-24.
5. Verma S, Sharma PK, Sivanandan S, Rana N, Saini S, Lodha R, et al. Spectrum of primary immune deficiency at a tertiary care hospital. Indian J Pediatr 2008;75:143-8.
6. Heinold A, Hanebeck B, Daniel V, Heyder J, Tran TH, Döhler B, et al. Pitfalls of “hyper”-IgM syndrome: a new CD40 ligand mutation in the presence of low IgM levels. A case report and a critical review of the literature. Infection2010;38:491-6.
7. Al-Muhsen SZ. Delayed presentation of severe combined immunodeficiency due to prolonged maternal T cell engraftment. Ann Saudi Med 2010;30:239-42.
8. Rafailidis PI, Mourtzoukou EG, Varbobitis IC, Falagas ME. Severe cytomegalovirus infection in apparently immunocompetent patients: A systematic review. Virol J 2008;5:47.
9. Schaffer FM, MonteiroRC, VolanakisJE, Cooper MD. IgA deficiency. Immunodefic Rev 1991;3:15-44.

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/1755-6783.121003

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