1717. Primary Immune Deficiencies in Adulthood: Fifteen Years of Experience from Iran
Session: Poster Abstract Session: Studies of the Interface of Host-Microbial Interaction
Saturday, October 5, 2013
Room: The Moscone Center: Poster Hall C
Background:

We describe the clinical presentation and outcome for 91 adult patients with previously unrecognized PIDs. 


Methods:

All patients more than 15 years old diagnosed as PID between October 1998 and September 2012 in NRITLD were enrolled. Secondary causes of immune deficiency were excluded, including: human immune deficiency virus infection, cancer, malnutrition, immunesuppressive therapy, diabetes mellitus, chronic kidney disease, collagen vascular disorders, etc. Diagnosis was confirmed by standard immunologic analysis.


                     

Results:

The frequency of humoral, cell-mediated, phagocytic and complement defects were 53.8, 11, 34 and 1.1%, respectively. Overall, the most common disorders were common variable immune deficiency (CVID, 43 cases) and chronic granulomatous disease (CGD, 25 cases). Fourteen patients (including five and four patients with CVID and CGD, respectively) died during follow-up. Respiratory infections were the most common complication followed by involvements of gastrointestinal tract, skin, lymph node, liver, bone and central nervous system The common organisms isolated in humoral deficiencies were pneumococcus, giardia, Hemophilus influenza, varicella zoster (VZV) and human papilloma viruses. Some other special findings were spindle cell pseudo-tumor in an interleukin 12-receptor (IL-12-R) deficient patient, IL-12** P40 deficiency with recurrent Salmonella infection and disseminated BCG, IL12R deficiency with MDR-TB, IL12R deficiency with chylous peritonitis and obliterating bronchiolitis and psoriasis in a CGD patient.

Conclusion:  This series provides unique data regarding PIDs presenting in adulthood, and serves as a timely reminder that physicians must consider the diagnosis of PIDs in their adult patients with recurrent, unusual, chronic and opportunistic infections; unusual autoimmune disorders, some kinds of malignancies and granulomatous diseases.  It seems, this is the first case series of PID in adults but certainly underestimated, much more work and attention needed.          

Ilad Alavi Darazam, M.D., Nritld, National Research Institute of Tuberculosis and Lung Diseases, Shahid Beheshti University of Medical Sciences, Tehran, Iran, Davood Mansouri, MD, MPH, Clinical Tuberculosis and Epidemiology Research Center, NRITLD, Masih Daneshvari Hosp., Shahid Beheshti University of Med. Sci., Tehran, Iran and Nahal Mansouri, MD, Medicine, UMDNJ-New Jersey Medical School, Newark, NJ

Disclosures:

I. Alavi Darazam, None

D. Mansouri, None

N. Mansouri, None

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