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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 - PowerPoint PPT Presentation
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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
• Healthy before gastrectomy; recurrent hospitalizations for multiple acute issues, predominantly infectious.
• Infections are recurrent, relapsing, do not respond to outpatient management—very challenging
Evaluating the Patient with Recurrent Infections
• Criteria for “recurrent”• Causes
– Therapeutic failure: resistant organism, atypical infection
– Patient-specific: underlying structural or immunologic impairment
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)
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
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
CVID Specifics
• Phenotype varies—course, infection types
• Serologies negative; cultures may be negative
• Subtypes—several classification schemata
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
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?)
Management
• Vaccination• Infections
– Prevention– Treatment
• Biopsy enlarged lymph nodes• Pulmonary
– PFTs and CT, chest physiotherapy, surgery
Treatment: Immune Globulin
• Data on Efficacy– Decreased infections, end organ
damage/autoimmune– Dosing and frequency– Risks:
• Infection• Dementia?!!• Infusion Reactions
Other Treatments
• Immunomodulating Therapies
• Gene Therapy?
• Indications for glucocorticoids
• Indications for prophylactic antibiotics
Case Patient’s Results
• Abnormal T cells, nonresponsive to pneumovax/Td
• Our patient’s course:– Phenytoin induced? Not reversible; progressive
• Two other patients
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.
References1. Weiler CR, Bankers-Fulbright JL. Common Variable
Immunodeficiency: Test Indications and Interpretations. Mayo Clin Proc. 2005;80(9):1187-1200.
2. Carrock Sewell WA, Buckland MS, Jolles SRA. Therapeutic Strategies in Common Variable Immunodeficiency. Drugs. 2003;63(13):1359-1371.
3. Spickett GP. Current perspectives on common variable immunodeficiency (CVID). Clinical and Experimental Allergy. 2001;31:536-542.
4. Travin, et al. Reversible Common Variable Immunodeficiency Syndrome Induced by Phenytoin. Arch Intern Med. 1989;149:1421-1422.