Vulnerable Patients:
Primary Immunodeficiency

Despite the availability of standard immune globulin therapy, >90% of patients with PID continue to experience recurrent respiratory infections.1
90%

Recurrent infections and other risk factors can lead to a vicious cycle of repeated infections, inflammation, and airway destruction.2

Recurrent infections
Risk Factors
  • Icon Recurrent pulmonary infections2
  • Icon Excessive use of antibiotics3
  • Icon History of infection2
  • Icon Asthma/COPD4
  • Icon Chronic sinusitis2
  • Icon Bronchiectasis1
  • Icon Age5
  • Icon Environmental factors6

In a long-term study of 473 patients with PID

29%
developed chronic lung disease7
11%
developed bronchiectasis7

Despite treatment with immune globulin therapy, recurrent respiratory infections are the most common clinical feature of PID and have the greatest impact on quality of life, including physical and social functioning.2

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Failure to recognize and address these high-risk populations can result in long-term respiratory complications, increased hospitalizations, and mortality for patients.2,8

What risk factors suggest this patient needs a change?

54-year-old male patient with PID
  • Icon

    History of hypogammaglobulinemia and recurrent respiratory tract infections

  • Icon

    Recurrent bacterial and viral infections despite IVIG dose increases and prophylactic antibiotics

  • Icon

    History of bronchiectasis

Patient
REVIEW CASE HISTORY
  • Icon

    Physical Exam

    Presented at the hospital with a fever and respiratory distress

    Vital signs on presentation:
    Chart
  • Icon

    Hospital Course

    Admitted into the hospital and received IV fluids and broad-spectrum antibiotics

    • Chest X-ray was consistent with pneumonia of unknown etiology
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    On day 3, all cultures were NGTD except NP swab, which was positive for PIV.

  • Icon

    Labs/Imaging

    Chest CT scan revealed interstitial pneumonia with bronchiectasis

    PaO2 = 65 mm Hg
  • Icon

    Follow-Up

    One week after hospitalization, developed hypotension requiring vasopressors

CLICK HERE TO LEARN MORE ABOUT RVI TREATMENT GOALS AND STRATEGIES
Clinical Challenges

Immunocompromised patients face a number of clinical challenges associated with RVI, including co-infections, progression to lower respiratory tract infection, and potentially death.

LEARN MORE
Addressing RVI

Learn about the advantages and limitations of RVI management strategies in immunocompromised patients.

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Cases are hypothetical. Images and descriptions are for illustrative purposes only


COPD, chronic obstructive pulmonary disease; CT, computed tomography; IV, intravenous; IVIG, intravenous immunoglobulin; NGTD, no growth to date NP; nasopharyngeal PID, primary immunodeficiency; PIV, parainfluenza; RVI, respiratory viral infection.

References

1. Jolles S. Clin Exp Immunol. 2014;178(Suppl 1):67-69. 2. Cinetto F, et al. Eur Respir Rev. 2018;27:180019. 3. Llor C, Bjerrum L. Ther Adv Drug Saf. 2014;5(6):229-241. 4. Berger M, et al. Respir Med. 2017;132:181-188. 5. Raje N, Dinakar C. Immunol Allergy Clin North Am. 2015;35(4):599-623. 6. Pagano L, et al. J Antimicrob Chemother. 2011;66(Suppl 1):i5-i14. 7. Resnick ES, et al. Blood. 2012;119(7):1650-1657. 8. Baumann U, et al. Front Immunol. 2018;9:1837.

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