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A Patient with Recurring Infections

A Patient with Recurring Infections. Julia Wright, M.D. Clinical Associate Professor of Medicine Section of General Internal Medicine. Case Illustration. 62-year-old woman with PMH rheumatoid arthritis, seizures and malabsorption

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A Patient with Recurring Infections

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  1. A Patient with Recurring Infections Julia Wright, M.D. Clinical Associate Professor of Medicine Section of General Internal Medicine

  2. Case Illustration • 62-year-old woman with PMH rheumatoid arthritis, seizures and malabsorption • Healthy before gastrectomy; recurrent hospitalizations for multiple acute issues, predominantly infectious. • Infections are recurrent, relapsing, do not respond to outpatient management—very challenging

  3. Evaluating the Patient with Recurrent Infections • Criteria for “recurrent” • Causes • Therapeutic failure: resistant organism, atypical infection • Patient-specific: underlying structural or immunologic impairment

  4. Case Patient’s Evaluation • Immunodeficiency suspected—recurrent sinopulmonary infections, poorly responsive, severe, multisystem illnesses, ? Family history. Frequently neutropenic, rarely febrile • Results of immunoglobin panel = • IgA 138 (85-450 mg/dL) • IgG 302 (700-1600 mg/dL) • IgM 44.3 (40-230 mg/dL) • T cell profile = • CD4% 318 (>490) • CD8 62 (>180) • CD3 53 (>840)

  5. Common Variable Immunodeficiency (syndrome) • Paradoxically named—1:40 patients with recurrent infections have CVID • Variable immunodysregulation; T cell and B cell defects; monocyte as well • A syndrome—common endpoint of multiple conditions/defects

  6. Clinical Features: Multisystem Disease • Infections: • B cell predominant; encapsulated organisms, sinopulmonary • All types, recurrent, refractory or poorly responsive • Unusual/nonpathologic (septic arthritis, meningitis) • Autoimmune: • CVD, IBD, ITP, hepatitis, thyroid, cytopenias • Inflammatory: • Malabsorption (GI atrophy), granulomatous disease • Malignancy: • T and B cell lymphoma, MALT

  7. CVID Specifics • Phenotype varies—course, infection types • Serologies negative; cultures may be negative • Subtypes—several classification schemata

  8. Diagnostic Criteria for CVID • Immunodeficiency: • Ig levels: IgG less than 500, 2 SD below mean • B cell function: • Illness/immunization status and serology • Post-vaccination Ig levels • Exclusion of other diagnoses: • Primary immunodeficiencies • Drugs • Protein losing states • Lymphoproliferative d/o • Post-infection; asplenia

  9. Practical Approach • Clinical Features, Family History, Medications • Ig levels; T cell Profile • Flow cytometry; Ig subclass • Post-vaccination Ab response • Exclusion—Clinical Grounds (flow cytometry, HIV, complement, UA, SPEP, CBC, sprue, genetic testing?)

  10. Management • Vaccination • Infections • Prevention • Treatment • Biopsy enlarged lymph nodes • Pulmonary • PFTs and CT, chest physiotherapy, surgery

  11. Treatment: Immune Globulin • Data on Efficacy • Decreased infections, end organ damage/autoimmune • Dosing and frequency • Risks: • Infection • Dementia?!! • Infusion Reactions

  12. Other Treatments • Immunomodulating Therapies • Gene Therapy? • Indications for glucocorticoids • Indications for prophylactic antibiotics

  13. Case Patient’s Results • Abnormal T cells, nonresponsive to pneumovax/Td • Our patient’s course: • Phenytoin induced? Not reversible; progressive • Two other patients

  14. Conclusions • A rare disease that is not rare in the primary care setting • Multisystem disease • Easy to diagnose—at first • Treatment is challenging • Refer, refer, refer.

  15. References Weiler CR, Bankers-Fulbright JL. Common Variable Immunodeficiency: Test Indications and Interpretations. Mayo Clin Proc. 2005;80(9):1187-1200. Carrock Sewell WA, Buckland MS, Jolles SRA. Therapeutic Strategies in Common Variable Immunodeficiency. Drugs. 2003;63(13):1359-1371. Spickett GP. Current perspectives on common variable immunodeficiency (CVID). Clinical and Experimental Allergy. 2001;31:536-542. Travin, et al. Reversible Common Variable Immunodeficiency Syndrome Induced by Phenytoin. Arch Intern Med. 1989;149:1421-1422.

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