Post on 07-Aug-2015
A CONCEALED INVADER Dr Murali Krishna MD DM
Panelists
Dr Rakesh Kochar DM Dr Naresh Bhat DM Dr T G Balachander MS, MCH Dr M Hariharan DM Dr R Ravi Kumar MD
History
A 48 year old male, well to do businessman from Chennai.
Presented with complaints of fresh bleed per rectum.
Massive, painless H/o of low grade fever for 3-4 days
before 15 days of onset of this bleed. History use of NSAIDs frequently.
History
No history of Constipation Chronic acid peptic complaints Use of antiplatelets or anti coagulants Diarrhoea/wt. loss/loss of appetite Non alcoholic and non smoker.
He was seen in other hospital for this complaints
There gastroenterologist did gastroscopy and colonoscopy, normal upper GI study.
Colonoscopy - altered blood in the entire colon and terminal ileum.
They did CT abdomen , cecal bleed with unidentified cause was found
Later CT angiography was performed and no active ooze was seen
Selective gel foam embolisation of the cecal branch of SMA was performed in view of continuing bleed.
When bleeding still persisted he was taken up for surgery
Intraoperative enteroscopy was performed and blood in the cecum, but no blood was seen in the distal ileum.
Right hemicolectomy was resorted as a desperate measure to stop bleeding.
Third post op day when the bleed recurred patient was brought to this hospital
On interviewing he said he had rumble every time before he passes blood PR
Examination Palor Tachycardia, normal BP Systemic examination –
Operative wounds Hurried bowel sounds.
Investigations
CBC Hemoglobin - 6.9 gm% Neutrophils 82 % Lymphocytes 8 % Eosinophils 5 % Monocytes 5 % WBC Count 7.18 10³/mm³ Platelet -272 10³/mm³ ESR- 96 mm/hr
LFT, RFT were normal
Course
Urgent CT angiography was done but was of hardly any help because of inconclusive findings
Patient was managed in ICU, PRCs were transfused.
Capsule endoscopy: No active bleed but altered blood near the anastomotic site anastomotic ulcer was seen.
Remained stable for next 3 days with no fall in Hb.
Stepped down to the ward.
He was started on liquid diet – tolerated, escalated to semisolids but ...
Started having colicky abdominal pain along with obstipation and abdominal distension
Examination – BS absent, rest normal
X- ray abdomen, USG abdomen – Normal
CECT was done for persistent pain and constipation
CECT
Diffuse submucosal wall edema involving the distal jejunal and ileal loops causing a target appearance to the bowel.
The distal superior mesenteric artery had beading with one or two small aneurysm in the smaller branches.
The vasa recta supplying the ileal loops appeared diffusely narrow.
Pulmonary thromboembolism involving the basal lobes.
Course
Multidisciplinary inputs were taken– pulmonlogist, hematologist and rheumatologist.
Anticoagulants started for the PTE
For vasculitis, complete vasculitis screen was performed and in absence of any markers and beading PAN concluded as possible diagnosis.
Steroids were started for the vasculitis.
Anticoagulants were titrated to adjust INR between 2-3.
Nutritional supplements were given.
Patient was later discharged asymtomatic.
PAN – Poly-Arteritis Nodosa Polyarteritis nodosa (PAN) is a systemic
necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries
Prevalence - range from 2 to 33 per million
Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome in a urban multiethnic population in 2000 J of Rheumat
Most cases of polyarteritis nodosa (PAN) are idiopathic but also can be in hepatitis B virus (HBV) infection, hepatitis C virus (HCV) infection, hairy cell leukemia
Histolopathology
Polyarteritis nodosa is characterized by segmental transmural inflammation of muscular arteries
In contrast to some other forms of systemic vasculitis, PAN does not involve veins
Presentation
GI involvement - 14 to 65% of patients with PANGastrointestinal involvement in polyarteritis nodosa. Ebert EC, Hagspiel KD, Nagar M,
Schlesinger N Clin Gastroenterol Hepatol. 2008;6(9):960
Abdominal pain – 25 % Gastrointestinal bleeding – 7 % Peritonitis – 4 % Intestinal infarction, pancreatitis, and duodenal
ulcer – 2 % each Cholecystitis – 1 %Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in
165 patients.AUGuillevin L, Le Thi Huong Du, Godeau P, Jais P, Wechsler BSO Br J Rheumatol. 1988;27(4):258.
Diagnosis
Arteriography is a primary modality used to diagnose PAN, being positive in more than 60% of patients
Saccular aneurysms form in the weakened portion of the vessel wall.
Vascular lesions tend to occur at branching points
CT scan shows bowel wall thickening with the target sign
The small intestine is the most commonly affected part of the GI tract, followed by the mesentery and colon.
Unlike some other vasculitides PAN is not associated with antineutrophil cytoplasmic antibodies (ANCA)
Tissue biopsy may confirm the diagnosis.
Common biopsy sites include the sural nerve, muscle, and skin lesions if present.
Corticosteroids +/-cyclophosphamide, are the mainstay of treatment for hepatitis B– and C–negative PAN
Cyclophosphamide, added particularly in the presence of severe GI involvement, reduces the incidence of relapse but does not change the 10-year survival rate
Fauci, A.S., Katz, P., Haynes, B.F. et al. Cyclophosphamide therapy of severe systemic necrotizing vasculitis. N Engl J Med. 2009; 301: 235–238
Prognosis
If left untreated, patients with PAN have a dismal prognosis.
Corticosteroids and cyclophosphamide have decreased mortality dramatically
The 1-year survival rate is 85% with treatment
Poor prognostic factors are proteinuria, renal insufficiency, cardiomyopathy, severe GI manifestations,
central nervous system involvement
Guillevin, L., Lhote, F., Gayraud, M. et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome (A prospective study in 342 patients) . Medicine. 1996;75: 17–28
Thank you