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

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Presentation on theme: "Allergy Grand Rounds Sarbjit S. Saini, M.D. JHAAC December 3, 2004."— Presentation transcript:

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 Past History 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

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


14 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

15 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

16 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

17 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

18 CsA and Urticaria-RDBCT Gratten 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: + 75% of UAS Results: 8/19 + at week 4, 6 relapse wk 6 –Noted reductions in HRA and ASST

19 CsA in CIU:Adults Open trial in 35 CIU with 3(0-3) –Low dose CsA 3 mos, 68% response (13/19) with few SEs 1 DB trial :40 pts CsA 5 mg/kg x 8 wks, then 4 mg/kg x 8 wks vs. cetirizine 10 mg/d 2 –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 1 Toubi,Allergy 1997; 2 Di Giaccino Allergy Asthma Proc 2003

20 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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