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Allergy Grand Rounds

Allergy Grand Rounds. Sarbjit S. Saini, M.D. JHAAC December 3, 2004. Chief complaint . 13 yr old male referred in June 2004 for evaluation of severe chronic urticaria Referred by pediatric allergist in VA Significant illnesses: include Type I DM for 2.5 yrs ADHD mood disorder.

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Allergy Grand Rounds

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  1. Allergy Grand Rounds Sarbjit S. Saini, M.D. JHAAC December 3, 2004

  2. Chief complaint • 13 yr old male referred in June 2004 for evaluation of severe chronic urticaria • Referred by pediatric allergist in VA • Significant illnesses: • include Type I DM for 2.5 yrs • ADHD • mood disorder

  3. History of Present Illness-I • June 02- lip swelling 1-2x/month, no Tx • Dec 02 -swelling of face, eyes, DIB and diffuse urticaria • Poor relief with fexofenadine & diphenhydramine • Required 4 prednisone tapers for control • March 03- seen by allergist in VA • Cetirizine, montelukast, ranitidine begun • PFTs within normal, FEV1 103% • Skin tests : + to dust mites, cats, dog, cockroach,trees, grasses, mold; +peanuts, nuts, garlic, shellfish, fish • Food RASTs all negative

  4. History of Present Illness-II • June 03- 1 d hospitalization, anxiety? • July 03- 3 of last 6 mos on prednisone • Fexofenadine + to Cetirizine, montelukast, ranitidine • CBC,WESR, AST, ANA, thyroid antibodies, H. pylori • Sept 03- hydroxyzine added, zafirulukast bid • C3, C4, CH50 checked • Lesions-erythematous, pruritic, painful with occasional bruising • Duration of lesions-minutes to 1 day

  5. History of Present Illness-III • Oct 03- cyclosporine 100 mg qd (2 mg/kg) • stopped all but prn H1 • Insulin RAST drawn, baseline labs, BP 132/78 • Nov 03-CsA helping, but hives still significant • increased to 100 mg BID CsA (4 mg/kg) • ER visit for lip swelling /DIB • 1 pred taper, prescribed an epipen • May 04 -2 pred courses, 3 uses of epipen due to DIB with urticaria • Restarted on fexofenadine,cetirizine, zafirlukast, • joint pains in knees with activity without morning stiffness and not steroid responsiveness

  6. Other atopic history • No history of eczema or food allergy • Allergic rhinitis symptoms • Exercise-related asthma age 9 treated with prn albuterol prior to activity • Reported qhs cough, but denied wheezing

  7. Medications • Zoloft, 50 mg qd** • Oxcadazepine (Trileptal) 300 mg/600 mg ** • Adderall 30 mg bid • Quetiapine (Seroquel) 200 mg qd • Fexofenadine 180 mg qd ( off 1 wk) • Cetirizine 10 mg qd ( off 1 week) • Cyclosporine 100 mg bid (off 1 wk) • Humulin 7 U/4 U, Humulin R 5 U/ 4 U • Epipen, Albuterol

  8. Past Medical History • Type I DM for 2.5 yrs • ADHD • Mood disorder, possible bipolar • exacerbated by steroids • suicidal ideation due to urticaria • Chicken pox as child • Salivary gland surgery • Normal birth history, negative history of other infections

  9. Family History Younger Sister with eczema PGM with asthma Paternal cousins with asthma Environmental Hx Apt dweller x 5 yrs Dog since 1999 3 hamsters Social Hx 7th grader Lives with mom and sister Past History

  10. Physical Exam • T-99.7, HR-121, BP-109/75, HT-5, WT-125,RR-22 • General: no obvious pubertal signs,central obesity, moon facies • HEENT: “allergic shiners”,erythematous nasal mucosa, prominent turbinates • Normal TMs, oropharynx, neck • Resp: CTA, normal I:E ratio, CV: nl S1, S2 tachy • Abdomen: benign Ext: no joint swelling • Skin: urticaria on face, arms, feet, back, chest; no pigmentation

  11. Recent labs • CBC-WBC 7.3 HCT-41.2, Plts-331 • HbA1C-8.6 (4-6) Jan 2004 • Negative studies: ANA, H. Pylori Ab,anti-thyroid peroxidase antibodies, WESR • Normal C3, C4, CH50; TSH, thyroxine, T3 and T4 • RASTS- negative for crab, lobster, fish garlic and insulin

  12. Cyclosporine related labs • Jan 2004 reduced Hct-12.1 HB- 37.2 • CsA: 37 ng/ml trough • March 2004 Normal studies • June 2004 • CsA: 46 ng/ml trough • CBC, Mg, Cr, K normal

  13. Impression/ Recommendations • Severe CIU/angioedema h/o significant steroids requirements • No clear drug (insulin), food or systemic etiology • Avoiding NSAIDs • Consider alternate diagnoses: • Hx of autoimmunity with Type I DM • Rheumatologic?-joint symptoms, bruising • Obtain a skin Biopsy to verify urticaria vs. other • Consider immunofluorescence

  14. Follow-up on Recommendations • Rheum evaluation: Repeated ANA, RF, dsDNA, ANCA, Urine and SPEP- all normal • showed IgA of < 20, no other etiology for joints • October 04 -Csa 100 mg qd and fexofenadine with good control • Prednisone used only single day since 6/2004 • No skin biopsy to date- attempted • Glucose under better control

  15. CU in children : association with thyroid autoimmunity • 187 CU pts (6- 18 yr) followed 7.5 yrs • Tests: CBC, sed, Chem, Antibodies to Hep B, HSV, EBV,CMV, mycoplasma, ASO, ANA, C3, C4, Thyroid function and antibodies,Ua, chest and sinus X-rays, food skin tests, ice cube test • Results: 8/187 antithyroid Ab (4.3%), all girls • 3x -1.27% rate seen in pediatric population • Much less than 14 to 33% range in adults • 5 +ANA, 4 + family Hx of autoimmunity Levy, et al, Arch Dis Child 2003

  16. Cyclosporine in Urticaria • CBC, Mg, K, renal function q 2 wks for first 3 months, CsA levels • Gingival hypertrophy • BP monitoring • Dose: 2-6 mg/kg/d similar to RA and psoriasis (2.5 mg/kg/day) • Tx dose 8 mg/kg/d; trough levels 100 ng/ml

  17. CsA and Urticaria-RDBCTGratten Br J Dermatol 2000:143 • 30 subjects, severe CIU unresponsive to H1 tx and positive ASST ( +HRA) • 4mg/kg CsA (n=20) or placebo (n=10) for 4 wks • All subjects followed for up to 20 wks, all on daily 20 mg cetirizine • Outcome: +< 25% of baseline UAS, relapse > 75% of UAS • Results: 8/19 + at week 4, 6 relapse wk 6 • Noted reductions in HRA and ASST

  18. CsA in CIU:Adults • Open trial in 35 CIU with 3(0-3) • Low dose CsA 3 mos, 68% response (13/19) with few SEs1 • DB trial :40 pts CsA 5 mg/kg x 8 wks, then 4 mg/kg x 8 wks vs. cetirizine 10 mg/d2 • All cetirizine crossed to active CsA • 3 pts reduced CsA for Cr rise • On tx- 22 had relapse, 10 resolved spon 12 with H1 • Off tx- 16/40 in remission at 9 mos 1Toubi,Allergy 1997; 2Di Giaccino Allergy Asthma Proc 2003

  19. Immunosuppression in Adolescents: Cyclosporin • 80% of liver, kidney, cardiac Tx > 5 yr survivors on CsA • Nephrotoxicity: 4-5 % in cardiac and liver • 10% in RA dosed > 4 mg/kg avg 19 mos • HTN (20-30%) • Hyperlipedemia (10% of cardiac) • Post-tx lymphoproliferative disease:5-17% • Cosmetic-Gingival hyperplasia, hirsutism Kelly, DA Pediatr Transplantation, 2002

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