Eosinophilic gastroenteritis in Churg-Strauss syndrome Daegu Catholic Medical Center Dept. of Internal Medicine Jin Tae Jung

Case (F/40) C/C: abdominal distension & generalized edema for 15 days P/I: whole abdominal pain with gradually aggravating abdominal distension, generalized edema & diarrhea(#4-5/d) for 15days PHx: DM (4-5 yrs ago Dx.: not under medication) asthma (+, intermittently medication at LMC) allergy (-) FHx: unremarkable

Physical exam V/S: BP 144 / 90, PR 80, BT 36.8℃, RR 26 G/A: acute ill appearance, alert Eye: anicteric sclera, not pale conjunctiva Chest: regular HB without murmur clear BS without rale Abdomen: soft & distended tenderness(+) with shifting dullness Liver - firm, 2 FB palpable Extremity: pitting edema (++/++)

Laboratory findings CBC: 28,500 / 11.3 / 60,000 (Eo 36.9%, 10,516/ul) LFT: Tp/alb 9.1 / 2.4 g/dl AST/ALT 22 / 26 IU/l TB 0.9 mg/dl ALP 341 IU/l BUN/Cr: 30 / 2.3 mg/dl IgE 1000 IU/ml Stool: OB (-), protozoa (-), RBC/ WBC (-)/(-) Skin test for CS & PW (-) Paracentesis: RBC 5-10, WBC 1,300 (poly : 30%, Eo 3.7%)

Abd-pelvic CT diffuse intestinal wall edema generalized edema & ascites enlarged LNs in mesenteric & paraaortic regions

Endoscopy diffuse mucosal erythema and edema, whole stomach & duodenum

Colonoscopy multiple patchy erythema & mucosal edema from terminal ileum to rectum

Biopsy extravascular eosinophil infiltration with small vessel vasculitis – stomach, duodenum, terminal ileum, colon Stomach Terminal ileum Ascending colon

Progress (I) Final Dx.: eosinophilic gastroenteritis(mucosal type), NIDDM → steroid 50mg start with insulin : resolved symptom & discharged on 10th hospital day. 2nd admission (2 months later, 청주성모병원) - Dx.: acute MI → balloon PTCA at pLAD - BM asp.: hypercellular marrow (cellularity 70%) eosinophil (15.8%), no cluster of malignant cells. 3rd admission (2 months later, DCMC) - C/C: abdominal pain & distension (1 month after steroid stop) severe dyspnea attack on admission day ABGA : 7.409 / 37.8 / 59.4 / 23.4 / 91.1% → steroid 50mg restarted: discharged after 7 days without symptom.

Progress (II) 4th admission (3 months later, DCMC) - C/C: facial swelling, generalized edema & dyspnea → ER visit (15days after steroid discontinuation) → admitted to ICU after endotracheal intubation - P/E: facial edema with conjunctival edema, lip swelling, bronchial wheezing - Consult Rheumatology under impression of vasculitis or angioedema.

Laboratory findings(4th Adm.) CBC: 19,700/9.8/64,000 (Eo 2.9%, 571/ul) IgE: 1000 IU/ml ESR: 120 → 10 mm/hr CRP : 321.8 → 12.4 mg/l Tp/alb : 7.5/2.3 g/l, BUN/Cr : 28 / 2.1 mg/dl ABGA : 7.416 / 28 / 78.6 / 17.6 / 96% Ig G/A/M : 3036 / 61 / 45 mg/dl lupus anticoagulant : (-) RF : 12.9 IU/ml ASO : 33.2 IU/ml ANA : 1:80, homogenous Anti ds-DNA : 1.62 IU/ml ANCA (Anti MPO / PR-3 Ab : 55 / 53) C3/C4 : <30 / <6 mg/dl CH50 : 4.0 U/ml EMG : peripheral motor polyneuropathy in peroneal & tibial N.

Rt. maxillary & ethmoidal sinusitis Chest PA Facial CT Rt. maxillary & ethmoidal sinusitis

Chest CT Abd-pelvic CT - multiple enlarged LNs - pleural effusion, both Abd-pelvic CT - generalized edema - scanty ascites - paraaortic lymphadenopathy

Progress (III) Final Dx. : Churg-Strauss syndrome → high dose steroid pulse therapy (1g/d for 3 days) → resolved her symptoms of angioedema & dyspnea → discharged after 12 days without symptom. OPD follow up with tapering of steroid (2 months later) → weakness of Lt. forearm developed → brain CT : acute brain infarction → pt refused admission because of low economic status → warfarin started & slight improvement in weakness

low density in Rt Frontoparietal region Brain CT low density in Rt Frontoparietal region