Primary immunodeficiency: a call for multidisciplinary care – Authors' reply

Abstract

R G Stirling indicates that insufficient information was provided in the section on management and treatment in our Seminar on common variable immunodeficiency. The primary focus of the review was on new advances in diagnostic methods and some of the molecular defects recently implicated in the pathogenesis of common variable immunodeficiency. Excellent reviews on the management and treatment of common variable immunodeficiency are available.1Yong PF Tarzi M Chua I Grimbacher B Chee R Common variable immunodeficiency: an update on etiology and management.Immunol Allergy Clin North Am. 2008; 28: 367-386Summary Full Text Full Text PDF PubMed Scopus (68) Google Scholar We agree that there can be substantial morbidity and mortality in common variable immunodeficiency, and therefore the emphasis on early diagnosis of disease and detection of complications continues to be a priority. Moreover, there is a paucity of prospective studies analysing treatment of infections in common variable immunodeficiency. Stirling notes that prophylactic antibiotics have not shown significant benefit in children and adults with bronchiectasis. However, the nine studies in that systematic review2Evans DJ Bara AI Greenstone M Prolonged antibiotics for purulent bronchiectasis in children and adults.Cochrane Database Syst Rev. 2007; 2 (CD001392.)Google Scholar did not select for patients with immunodeficiency. In fact, two of the studies excluded patients with immunodeficiencies. Thus, the effect of prophylactic antibiotics in patients with bronchiectasis and common variable immunodeficiency has not been rigorously assessed. In one study of 248 patients with common variable immunodeficiency,3Cunningham-Rundles C Bodian C Common variable immunodeficiency: clinical and immunological features of 248 patients.Clin Immunol. 1999; 92: 34-48Crossref PubMed Scopus (1251) Google Scholar most of the patients (46 of 57) died of causes other than respiratory infection. Thickett and colleagues4Thickett KM Kumararatne DS Banerjee AK Dudley R Stableforth DE Common variable immune deficiency: respiratory manifestations, pulmonary function and high-resolution CT scan findings.QJM. 2002; 95: 655-662Crossref PubMed Scopus (160) Google Scholar reported similar findings where seven of eight patients with common variable immunodeficiency died of causes other than bacterial respiratory disease. For patients with recurrent infections despite immunoglobulin replacement, liberal early antibiotic use or perhaps, in select cases, prophylactic antibiotics, might be required on the basis of the clinician's judgment. In the 2005 American Academy of Allergy, Asthma, and Immunology practice parameters for the diagnosis and management of primary immunodeficiencies,5Bonilla FA Bernstein IL Khan DA et al.Practice parameter for the diagnosis and management of primary immunodeficiency.Ann Allergy Asthma Immunol. 2005; 94: S1-S63Summary Full Text PDF PubMed Scopus (435) Google Scholar the use of prophylactic antibiotics is listed as category C evidence (obtained from non-experimental descriptive studies). It is reasonable therefore to suggest the following: 1Until further evidence is obtained, clinicians should use clinical judgment in applying knowledge from treatments that have shown success in similar disease processes—eg, pulmonary toilet (chest physiotherapy) in bronchiectasis on the basis of the success shown in patients with cystic fibrosis patients.2Early treatment of severe infections is likely to reduce morbidity and mortality.3Antibiotics should be targeted to specific bacterial infections.4Anatomical issues such as bronchiectasis, radiation fibrosis, cancer, and ostiomeatal occlusion should be considered and assessed as appropriate for patients with suboptimum response to antibiotic treatment for sinopulmonary disease.5Patients with complications of common variable immunodeficiency such as bronchiectasis require a coordinated medical approach using multidisciplinary care.6Patients with common variable immunodeficiency are probably best served by regular follow-up by physicians experienced in the care of such patients. MAP and JBH have participated in studies on safety and efficacy of IgPro10 and IgPro20 in primary immunodeficiency sponsored by ZLB Behring. No personal funding was received. They also participated in another IgPro study by ZLB Behring. JL was a previous participant in the IgPro study listed above. DM was a participant in the IgPro20 study listed above. RSA has no conflict of interest. Primary immunodeficiency: a call for multidisciplinary careThe Seminar by Miguel Park and colleagues (Aug 9, p 489)1 gives an excellent update on emerging knowledge of the heterogeneous antibody deficiency syndrome common variable immunodeficiency. What the paper fails to do, however, is help identify key management issues and illuminate care plans. In three short sentences antibiotic treatment is recommended then dismissed without specific guidance. This shortfall is common2 and reflects the diverse specialist needs of individuals with common variable immunodeficiency. Full-Text PDF