1. DEPARTMENT OF MEDICAL GASTROENTEROLOGY “A CASE OF PULSELESS GI BLEED” PROFESSOR: Dr.L.THAYUMANAVAN UNIT ASST.PROFESSORS Dr.A.S.A.JAGANATHAN,M.D.,D.M Dr.M.KANNAN M.D., D.M. Dr.R.RAMANI M.D., D.M.

2. Ms.Suganya, 15yr old was admitted with c/o: 1. Bleeding P/R: 4 months 2. Abdominal pain: 4 months History of present illness: H/o bleeding P/R: 4 months H/o abdominal pain H/o tenesmus H/o fever H/o loss of weight H/o loss of appetite H/o headache H/o claudication pain of the Right hand

3. No h/o vertigo/dizziness/blurring of vision No h/o joint pain Past History: Not a knownDiabetic/Hypertensive/Epileptic/ Asthmatic/ Tuberculous patient No h/o similar episodes Personal History student takes mixed diet

4. Menstrual history: attained menarche at 13yrs of age regular cycles, 3/30 Family History: No other family members suffering from similar illness

5. GENERAL EXAMINATION Conscious Afebrile Pallor No icterus/cyanosis/clubbing/lymphadenopathy No pedal edema

6. Vital signs Conscious Pulse: 112/min, regular absence of both radial and brachial pulse of Right upper limb. Bilateral carotid thrill+ BP:142/84mmHg, Left upper limb could not be recorded in the Right upper limb 150/80mmHg, Right lower limb Temp:98.4F RR:16/min Urine output: Adequate

7. Systemic examination CVS: JVP normal. S1,S2+, haemic murmur+ RS: NVBS+, No added sounds P/A: soft, no organomegaly BS+, Renal artery bruit+ CNS :NFND FUNDUS: NORMAL

8. SUMMARY YOUNG FEMALE CHRONIC DYSENTERY ABSENT PULSE IN THE RIGHT UPPER LIMB CAROTID AND RENAL ARTERY BRUIT

9. PROVISIONAL DIAGNOSIS CHRONIC DYSENTERY - ? PROCTOCOLITIS RENOVASCULAR HYPERTENSION

10. INVESTIGATIONS Hb: 7.4g% TC: 24,000 DC: P57, L40, E3 ESR: 90mm1st hr Urine: albumin- nil sugar- nil deposits: 2-3pus cells/hpf

11. RBC count: 2.4million/cu.mm Platelet count: 2.6lakhs/cu.mm PCV: 24% Peripheral Smear: – Normochromic normocytes with hypochromic microcytes, neutrophilic leucocytosis, normal platelet count and morphology. LFT:Normal RFT:Normal ECG:No abnormality detected Xray chest PA view:Normal

12. ECHO: Normal Study USG Abdomen: Normal study OGD: normal study Colonoscopy: – Erythema, edema of mucosa, ulcerations, contact bleed and easy friability of mucosa which extended upto the caecum. – Multiple biopsies were done IMPRESSION: SUGGESTIVE OF “PANCOLITIS”

13. COLONIC BIOPSY Shows colonic mucosa with dense infiltration of predominant lymphocytes, plasma cells and few polymorphs in the lamina propria. There is a loss of mucin content in the glands with few crypt abscess. There is no tuberculous granuloma formation or dysplasia IMPRESSION: SUGGESTIVE OF “IDIOPATHIC PROCTOCOLITIS”

14. DOPPLER STUDY(done at G.R.H.) RENAL ARTERY: showed normal blood flow bilaterally

15. DOPPLER …. Upper limb and lower limb: revealed features of diffuse wall thickening of arch of aorta, both carotids and right subclavian artery. – Right subclavian: 80-95% stenosis, few cm distal to its origin with elevated PSV at the junction of narrowing. Distal to it the brachial and radial arteries showed monophasic flow.

16. Right vertebral: showed turbulent flow Both carotids: showed significant thickening in proximal and midcarotids, 60% stenosis in the right carotid and 80% stenosis in the left carotid. Internal and external carotids showed monophasic flow.

17. MR angiogram(done at K.G.S. scan) Normal aortic arch and thoracic aorta Common origin of innominate artery and left common carotid artery Mild diffuse narrowing of the proximal portion of both carotids(L>R) Complete occlusion of the Right subclavian artery after the origin of vertebral artery with distal flow formed by collaterals.

18. Minimal narrowing of the second part of the left subclavian artery. Both common carotid artery bifurcation, cervical and intracranial course of ICA, both MCA both ACA are normal. Both vertebral artery, basilar artery and both PCA showed no obvious pathology Significant narrowing of Left side pulmonary arteries seen. Diffuse thickening of the infrarenal segment of abdominal aorta noticed with significant proximal stenosis of both renal arteries(80-90%)

19. Mild narrowing of coeliac trunk and diffuse narrowing of SMA and its branches. Aortic bifurcation and both common iliac arteries appear normal Mild narrowing of the external iliac and common femoral arteries seen on both sides. Superficial femoral, popliteal, anterior tibial and posterior tibial arteries: Normal.

27. IMPRESSION SUGGESTIVE OF TAKAYASU’S ARTERITIS

28. SPECIALTY OPINION CARDIOLOGY: Suggestive of Takayasu’s arteritits OPTHAL OPINION: Normal Fundus NEPHROLOGY OPINION: ?Renal artery stenosis

29. FINAL DIAGNOSIS “TAKAYASU’S ARTERITIS WITH ULCERATIVE COLITIS”

30. management Prednisolone 30mg/day Asacol 400mg thrice daily Amlodipine 2.5mg twice daily Supportive measures

31. Follow up(2months later) Azathioprine 50mg once daily Steroids(decreasing doses) Antihypertensives

32. DISCUSSION

33. CAN THESE BE TOGETHER?

34. Takayasu’s arteritis is more prevalent in Japan and South East Asia Ulcerative colitis is more prevalent in Western countries with rising incidence in India in recent times The association of Takayasu’s arteritis with Ulcerative colitis is rarely reported. Both are chronic inflammatory diseases of unknown aetiology

35. Only 3 cases of co-existence have been reported from India. COMMON PATHOPHYSIOLOGY: Takayasu’s arteritis may accompany ulcerative colitis as an extraintestinal manifestation. higher freq. of HLABw52 and DR2 and A24 cross-reactivity between auto-antigens in the arterial wall and colonic mucosa Role of bacteria? microbes or their structural components with high sequence homology to humans may provoke chronic autoimmune process at both arterial wall and colonic mucosa of genetically susceptible individuals.

36. “Follow-up radiographic studies revealed no change in the aneurysms, however, the radial pulses gradually recovered in 3 years, condition remained uneventful with stable cardiac function and blood pressure for 13years of follow-up until a sudden lethal aneurysmal rupture” - Akira Hokama et al (Internal medicine 42:897-898, 2003)

37. REASONS FOR PRESENTATION RARITY OF TAKAYASU’S ARTERITIS RARITY OF ULCERATIVE COLITIS TO HIGHLIGHT THE ASSOCIATION OF TAKAYASU ARTERITIS WITH IDIOPATHIC PROCTOCOLITIS WHICH IS A RARITY.

38. THANK YOU