10.21 SFGH morning report pearls: adult primary immunodeficiencies/fever and rash

  • Clinical predictors of children with primary immunodeficiencies: : lymphopenia, otitis media >7, failure to thrive, failure to grow normally, pneumonia >1.
  • secondary immune disorders more prevalent than primary immunodeficiencies in adults
    •          consider DM, HIV, nephrotic syndrome, cirrhosis, IBD, autoimmune disease, med effect from immunosuppressants
  • primary immunodeficiency presenting in adulthood: (from AGM article: evaluation of the adult with suspected immunodeficiency)

Host Defense Affected Clinical example Organisms
B cells common variable immunodeficiency encapsulated organisms:

streptococcus pneumonia, haemophilus influenza, Moraxella catarrhalis, giardia, campylobacter

Neutrophils  Chronic granulomatous disease staph aureus, aspergillus, gran-negative bacteria
Complement Terminal complement deficiency (C5-C9) Neisseria
Early complement deficiency (C2, C3, C4) encapsulated organisms
NK cells NK cell deficiency herpes viruses, HPV
  • Primary immunodeficiency with skin or pulmonary infections: hyper IgE syndrome and DOCK8, chronic granulomatous disease, GATA2 deficiency, CVID.

fever and rash emergencies:

– meningococcal infection

  • rash oftentimes spares the palms and soles (vs RMSF)

– bacterial endocarditis

– Rocky mountain spotted fever

– toxic shock syndrome

Rashes that involve palms and soles: (pneumonic CARS, “you drive CARS with your palms and soles) thanks Ali!)

  •  CA – Coxsackievirus A
  • R – Rickettsia Rickettsii
  • S – Syphilis (secondary)
  • staph and strep (not part of mneumonic, but can cause desquamating rash on palms)

References: Ten warning signs for primary immunodeficiency in adults. http://www.info4pi.org/aboutPI/index.cfm?section=aboutPI&content=warningsignsadult&CFID=40450642&CFTOKEN=62808651

Azar AE, Ballas ZK. Evaluation of the adult with suspected immunodeficiency. Am J Med 2007; 120:764.

Lankisch et al. The Duesseldorf Warning signs for primary immunodeciency: Is it Time to Change the Rules? Journal of Clinical Immunology. April 2015. Volume 35: 3; 273-279.

evernote link: https://www.evernote.com/shard/s643/sh/f35636c9-8399-4a74-b894-7670a5486b5a/e69ba025a1454d34eb9f82bbf441a4a8

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