Did you know?

  • It often takes many years or even decades to diagnose PIDD2
  • Sinopulmonary manifestations and their complications are extremely common, and may be the presenting symptom in patients with PIDD3

PIDD is most commonly
diagnosed in adulthood

67% of patients were not diagnosed with PIDD until ≥35 years of age2

PIDD causes serious chronic
pulmonary health complications

54% of patients reported decline in lung function prior to PIDD diagnosis3

Pulmonologists are one of the first specialists seen and have the opportunity to minimize longer-term damage3

Read the abstract

PIDD could be behind your patients’ recurrent infections1

As many as 60% of patients with PIDD experience pulmonary complications4

  • Respiratory infections are often the first clinical warning sign of PIDD, a group of hereditary immunodeficiency disorders that includes hypogammaglobulinemia and common variable immunodeficiency (CVID)1,5
  • PIDD prevents the immune system from protecting against bacterial infections1

Recurrent lung infections are often the main cause of death in adults with PIDD1

Undiagnosed and untreated pulmonary complications of PIDD can lead to6:

  • Permanent lung damage
  • Decreased exercise tolerance
  • Increased hospitalization
  • Impaired quality of life
  • Early death

Diagnosis and treatment with immunoglobulin replacement therapy (IGRT) may help3

Did you know?

  • There was a 27% reduction of pneumonia for each 100 mg/dL increase in immunoglobulin G (IgG) level in patients diagnosed with PIDD3
  • IGRT may improve lung function in patients with primary immunodeficiency diseases who also have chronic lung disease3

Patients with PIDD may be hiding in plain sight1,7

PIDD may be the reason your patients’ infections or chronic disease are resistant to treatment. Consider PIDD in patients with1,8,9:

Recurrent or recalcitrant respiratory infections, recurrent pneumonia, or bronchiectasis1,9

Two or more new sinopulmonary infections within 1 year, in the absence of allergy8

Family history of PIDD or autoimmune disease9

Other common clinical manifestations that may signal the presence of PIDD:

  • Recurrent bronchitis10
  • Recurrent and/or unusual forms of pneumonia:1,10
  • Lymphadenopathy11
  • Interstitial lung disease associated with Common Variable Immunodeficiency (CVID)1
  • Granulomatous Lymphocytic Interstitial Lung Disease (GLILD)11
  • Lymphocytic Interstitial Pneumonia (LIP)12
  • Lung Nodules11
  • Granulomatous infiltrations13

Did you know?

European Respiratory Society guidelines recommend testing IG levels in bronchiectasis patients?14

Review guidelines

Find patients with PIDD in your practice by ordering labwork

Order these lab tests to help confirm a PIDD diagnosis and improve patient outcomes1,10,15

Order a complete blood count with differential white blood cell count for the purpose of identifying neutropenia, lymphopenia, or monocytopenia.1,14

Any one of these findings would indicate increased infection risks and the need to rule out other causes of the low levels.

Order a quantitative serum IG (IgG, IgA, and IgM) panel to determine if IG levels suggest primary immunodeficiency.1

Total IgG levels <600 mg/dL may indicate the presence of underlying PIDD16

In patients with a clinical presentation that suggests PIDD, normal serum IG levels may not rule out a PIDD diagnosis17

  • Results may appear normal even in patients with PIDD because serum IG levels are not sensitive indicators of specific immunity17
  • Consider factors that may affect serum IG levels18
    • Age
    • Sex
    • Lifestyle (alcohol use, smoking, medications) 
    • Comorbidities (metabolic syndrome, diabetes, liver disease, dyslipidemia, obesity)

Further work-up may include a vaccine challenge to test antibody titers to protein and polysaccharide vaccines.11,19

At this stage, you may want refer the patient to an immunologist for diagnosis and treatment of PIDD

Speak with a Grifols team member about PIDD, testing, and available treatment options

Get in touch

References

  1. Soler-Palacin P, de Gracia J, González-Granado LI, et al; on behalf of Lung ID-Signal Group. Primary immunodeficiency diseases in lung disease: warning signs, diagnosis, and management. Respiratory Res. 2018;19(1):219. 
  2. Immune Deficiency Foundation. Primary immune deficiency diseases in America: the fifth national survey of patients (2017). Published March 19, 2018. Accessed July 3, 2024. https://primaryimmune.org/sites/default/files/IDF%202017%20National%20Patient%20Survey%201.8.2020%20Patient.pdf
  3. Orange JS, Akhter J, Seeborg FO, Boyle M, Scalchunes C, Hernandez-Trujillo V. Pulmonologist perspectives regarding diagnosis and management of primary immunodeficiency diseases. Allergy Asthma Proc. 2016;37(6):e162-e168. 
  4. Bierry G, Boileau J, Barnig C, et al. Thoracic manifestations of primary humoral immunodeficiency: a comprehensive review. Radiographics. 2009(7);29:1909-1920 
  5. Salzer U, Warnatz K, Peter HH. Common variable immunodeficiency – an update. Arthritis Res Ther. 2012;14(5):223. 
  6. Breidenbaugh M, Lawrence MG. Racial, ethnic, and socioeconomic disparities in the diagnosis and management of primary immunodeficiencies. J Allergy Clin Immunol Pract. 2021;9(7):2926-2927. 
  7. Meyts I, Bousfiha A, Duff C, et al. Primary immunodeficiencies: a decade of progress and a promising future. Front Immunol. 2021;11:625753. Jeffrey Modell Foundation Medical Advisory Board. 10 warning signs of primary immunodeficiency.
  8. Jeffrey Modell Foundation website. Accessed July 3, 2024 https://info4pi.org/library/educational-materials 
  9. American Academy of Allergy, Asthma, & Immunology (AAAAI). Primary immunodeficiency disease overview. AAAAI website. Accessed July 3, 2024. https://www.aaaai.org/conditions-treatments/primary-immunodeficiency-disease/primary-immunodeficiency-disease-overview
  10. McCusker C, Upton J, Warrington R. Primary immunodeficiency. Allergy Asthma Clin Immunol. 2018;14(Suppl 2):61. 
  11. Costa-Carvalho BT, Grumach AS, Franco JL, et al. Attending to warning signs of primary immunodeficiency diseases across the range of clinical practice. J Clin Immunol. 2014;34(1):10-22. 
  12. Chapel H, Cunningham-Rundles C. Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions. Br J Haematol. 2009;145(6):709-727. 
  13. Jeevarathnum AC, van Niekerk A, Kriel J, Green RJ. Common variable immunodeficiency disorders: What generalists should know. Afr J Thoracic Crit Care Med. 2021;27(3):112-116. 
  14. Polverino E, Goeminne PC, McDonnell MJ, et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017;50(3):1700629
  15. Anderson JT, Cowan J, Condino-Neto A, Levy D, Prusty S. Health-related quality of life in primary immunodeficiencies: Impact of delayed diagnosis and treatment burden. Clin Immunol. 2022;236:108931. 
  16. Agarwal S, Cunningham-Rundles C. Assessment and clinical interpretation of reduced IgG values. Ann Allergy Asthma Immunol. 2007;99(3):281-283. 
  17. Bonilla F, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis cand management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-1205e78. 
  18. Gonzalez-Quintela A, Alende R, Gude F, et al. Serum levels of immunoglobulins (IgG, IgA, IgM) in a general adult population and their relationship with alcohol consumption, smoking and common metabolic abnormalities. Clin Exp Immunol. 2007;151(1):42-50. 
  19. ARUP Consult. Primary immunodeficiency diseases - immunoglobulin disorders. ARUP Consult website. Updated June 2024. Accessed July 3, 2024. https://arupconsult.com/content/immunoglobulin-disorders