STUDIES GAVE : An interesting cause of iron deficiency anemia

Case report A 55-year-old female presented to the OPD clinic with dyspnea on exertion, easy fatigability, and melena. She was referred from hematology department due to the occurrence of persistent anemia. No history of hematemesis, abdominal pain or chronic drug intake was reported. She had difficulty in getting up from sitting position, multiple joint pain, and Raynaud’s phenomenon. She had been transfused twice for anemia in the last one year. She had attained menopause and had normal bowel habits.


Case report
A 55-year-old female presented to the OPD clinic with dyspnea on exertion, easy fatigability, and melena.She was referred from hematology department due to the occurrence of persistent anemia.No history of hematemesis, abdominal pain or chronic drug intake was reported.She had difficulty in getting up from sitting position, multiple joint pain, and Raynaud's phenomenon.She had been transfused twice for anemia in the last one year.She had attained menopause and had normal bowel habits.
Physical examination revealed that the patient was severely pale with sclerodactyly and facial skin tightening.Her BP was 160/100 mm Hg.She had symmetrical proximal muscle weakness of both lower and upper limbs (power of 3/5).No skin lesions suggestive of dermatomyositis were reported.Other systems examinations were within normal limits.
Her hemoglobin was 5.6 g/dl with normal total count and platelets.Peripheral smear showed microcytic hypochromic anemia and the stool examination was positive for occult blood.Results of upper gastrointestinal (UGI) endoscopy showed hyperemic streaks alternating with normal mucosa (watermelon stomach) (Fig. 1 and 2), suggestive of gastric antral vascular ectasia (GAVE).Her creatine phosphokinase (CPK) was 126 IU/L and the levels of other enzymes such as LDH, SGOT, and SGPT were normal.The findings of electromyography (EMG) were normal with no feature suggestive of inflammatory myositis or myopathy.She was positive for ANA by ELISA and for CENP-B (Anti Centromere Ab) by immunoblot.The patient had undergone Argon laser photocoagulation (Fig. 3 and  4) and packed cells transfusion, and the treatment had contributed to symptomatic improvement.
Based on the clinical investigations and history, she was diagnosed to have limited cutaneous systemic sclerosis with GAVE.The initial presentation of severe iron deficiency anemia was due to intermittent GI bleeding.

Discussion
GAVE is a rare cause for upper GI loss and subsequent iron deficiency anemia.The syndrome was first described by Ryder et al. in 1953 and the term 'watermelon stomach' (endoscopic appearance) was first coined by Jabbari et al. 1,2,3 The disease is under-recognized many a times and misdiagnosed as antral gastritis. 4It is more common in elderly females and usually diagnosed during endoscopy as prominent flat or raised erythematous patches radiating in a spoke-like fashion from the pylorus to the antrum.The three macroscopic patterns for GAVE disease described by Chawla et al. are watermelon stomach, honeycomb stomach, and well-demarcated, round-or mushroomshaped lesion. 5e histology characteristics involve fibromuscular Abstract Gastric antral vascular ectasia (GAVE) is a rare cause for chronic severe gastrointestinal bleeding requiring repeated transfusions.We present here the case of 55-year-old female who presented with severe iron deficiency anemia with melena.The disease was further diagnosed as GAVE due to the presence of watermelon stomach on endoscopy with features of limited scleroderma.The patient showed symptomatic improvement on treatment with Argon laser photocoagulation and blood transfusion.
hyperplasia of lamina propria with intravascular fibrin thrombi and increase in luminal size of mucosal vessels. 4lthough several theories including achlorhydria, hypergastrinemia, and low pepsinogen levels have been proposed; the pathophysiology of GAVE remains unclear. 8ne of the pathogenetic mechanisms proposed is partial prolapse of loosely attached gastric mucosa of antrum due to vigorous gastric peristalsis.So there is intermittent obstruction of submucosal blood vessels and hence vascular ectasia. 2,4  etiology of GAVE is unknown, but the disease is associated with liver cirrhosis (in 30% of patients), systemic sclerosis, primary biliary cirrhosis, chronic renal failure, atrophic gastritis, diabetes mellitus, Addison's disease, familial Mediterranean fever, rectal vascular ectasia, marrow transplantation, and hypothyroidism. 4,6  closest differential diagnosis of GAVE is portal gastropathy.The disease is commonly found in antrum (rather than fundus in portal gastropathy) with no relation to portal hypertension and no response to beta blockers. 9-Omar et al. have reported that the association between severe bleeding from GAVE in patients with CREST syndrome is very rare. 10In Scleroderma: Cyclophosphamide Or Transplant (SCOT) trial, 23 out of 103 (22.3%) patients with early diffuse systemic sclerosis were found to have GAVE on endoscopy.¹¹Anti-topoisomerase I (anti-Scl70) was less frequently associated with GAVE, whereas a negative association between anti-U1 ribonucleoprotein (RNP) antibodies and the disease has been reported. 11he present case had chronic bleeding requiring multiple transfusions, limited scleroderma, and positive centromere protein (CENP)-B antibodies.
Treatment is often challenging and involves corticosteroids, hormonal therapy, octreotide, and tranexamic acid.Literature evidence also indicates the use of alpha-interferon, calcitonin, serotonin antagonist, and cyproheptadine for treatment.Surgical procedures like antrectomy and shunt procedures may be beneficial in cirrhotic patients.The various endoscopic methods employed are Nd:YAG laser, Argon plasma coagulation, heater probe, sclerotherapy, Argon laser, and bipolar electro-cautery. 4