Immunology and Rheumatology

Question 1

Primary Immunodeficiency

Answer 1

10 Warning Signs:

1. Four or more new ear infections within one year.
2. Two or more serious infections within one year.
3. Two or more months on antibiotics with little effect.
4. Two or more pneuomonias within one year.
5. Failure of an infant to gain weight or grow normally.
6. Recurrent deep skin or organ abscesses.
7. Persistent thrush in mouth or fungal infection to skin.
8. Need for intravenous antibiotics to clear infection.

9 Two or more deep-seated infections, including septicemia.

10. A family history of primary immunodeficiency.

Question 2

Secondary Immunodeficiency

Answer 2

HIV

Question 3

Juvenile Immune Arthritis (JIA)

Answer 3

Autoimmune disease with genetic and environmental components.
- Many different types including monoarthritis, polyarthritis, chronic polyarthritis, fever and rash associated with systemic JIA.

S/S: Joint pain, swelling or warmth, pain on motion of joint, uveitis.

Complications: Macrophage Activation Syndrome (MAS) or secondary Hemophagocytic Histiocytosis (HLH).

Dx: CBC-D, blood smear, ESR, CRP, BCx with fever.
- Imaging: Plain films, CT/MRI.

Tx: Supervised by rheumatologist, consults may include orthopedics, ophthalmology, hematology. Typically includes NSAIDs, DMARDs such as methotrexate, glucocorticoids, cytoxic agents such as thalidomide and cyclophosphamide, and biologic agents such as rituximab.

Question 4

Systemic Lupus Erythematosus (SLE)

Answer 4

Multisystem, inflammatory autoimmune disease characterized by autoantibodies to nuclear and cellular components.
- Very rare in childhood.

Dx: CBC-D, ESR, CRP, ANA with tiger, ENA panel (SS-A, SS-B, RNP, anti-Smith), dsDNA, coag studies, LFTs, BUN, creatinine, UA, TSH, free T4, fasting lipid panel.

Question 5

Vasculitis

Answer 5

Necrosis and inflammation of blood vessels as a result of conditions or diseases such as autoimmune or drug reactions.
- Uncommon in children!