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• W1972503540 abstract "INTRODUCTION Wegener's granulomatosis (WG) is an autoimmune disorder characterized by a necrotizing vasculitis of small to medium size vessels. The disease is rare in childhood. Affected individuals typically present with respiratory and renal involvement (1,2). Common presenting features include fever, malaise, sinusitis, epistaxis, arthritis, cough, hemoptysis and glomerulonephritis. Common cutaneous findings include ulcers and palpable purpura. Subglottic stenosis and nasal deformity are often seen (1). A positive anti-neutrophil cytoplasmic antibody (ANCA) with a cytoplasmic immunofluorescent staining pattern (c-ANCA) with specificity to proteinase 3 (PR-3) antigen is present in nearly all patients with WG and in a minority of patients with a similar vasculitic disorder, microscopic polyangiitis (MPA). The presence of necrotizing granulomas in affected tissues is diagnostic for WG. Angiography revealing evidence of vasculitis is also quite helpful (3,4). Gastrointestinal manifestations are rare, especially at initial presentation and in isolation from other organ involvement. The incidence of gastrointestinal involvement ranges from 0% to 5% (5), with the most common symptoms including profuse diarrhea (6), abdominal pain (7) and bloody diarrhea (8). Many of the reported gastrointestinal symptoms have occurred in adults who have an established diagnosis of WG. These individuals typically have gastrointestinal complications secondary to intestinal wall ischemia, infarction with potential perforation, mucosal ulceration with potential gastrointestinal hemorrhage or aneurismal rupture (9-11). Gastric bleeding as a possible complication in adults with WG has been reported (11). Significant gastrointestinal involvement as a presenting symptom in a young child with Wegener's granulomatsosis is extremely rare. In 1984 Sokol et al. reported a 16-year-old female with WG whose initial presentation mimicked inflammatory bowel disease (12). We report an unusual intestinal presentation of ANCA-positive small vessel vasculitis in a 6-year-old girl. PATIENT AND METHODS A 6-year-old Caucasian female presented to her local hospital with a 1-week history of watery stools and fever to 40°C. She had decreased appetite and had lost >10% of her body weight. White blood cell count on admission was 10 K/mm3 (normal range, 4 to 10.3 K/mm3) with 19% band forms. She had hyponatremia and hypokalemia. Stool was negative for Clostridium difficile. She was given intravenous ceftriaxone. The next day she developed abdominal distention, and a computed tomography scan revealed colonic wall thickening with increased luminal liquid and gas. She was transferred to our institution for further evaluation and management. Family and social history were negative. Medical history was remarkable for a urinary tract infection 1 month before the onset of her current illness. This resolved after 10 days of amoxicillin/clavulanate. Nine days before the onset of fever and diarrhea, she had experienced 1 day of self-limited emesis after having been exposed to several of her classmates with similar symptoms. Physical examination on admission to our hospital showed a moderately ill and dehydrated child. She denied any pain. There were no oral lesions or lymphadenopathy. There were no ocular or cardiac abnormalities. Lungs were unremarkable. Her abdomen was distended and tympanitic but soft and nontender. The rectum was dilated and filled with dark green, liquid stool that was weakly hemoccult positive. Abdominal radiographs demonstrated a large, dilated colon with intestinal wall thickening. Her admission laboratory tests were significant for an erythrocyte sedimentation rate of 73 mm/h (normal range, 0 to 20 mm/h), a C-reactive protein of 15.2 mg/dL (normal <0.5 mg/dL), a white blood cell count of 3.8000/mm3, serum albumin of 2.6 g/dL (normal range, 2.9 to 4.2 g/dL), platelets of 264,000/mm3 (normal range, 150,000 to 400,000) and hemoglobin of 10.8 g/dL (normal range, 11.9 to 15 g/dL). A diagnosis of acute enterocolitis was made and intravenous ampicillin, gentamicin and metronidazole were started. Rectal irrigations using 10 cc/kg of normal saline were performed every 2 hours using a 28-French rectal tube. Through the initial hospital course her erythrocyte sedimentation rate and C-reactive protein remained increased and she experienced fever as high as 40.2°C daily. Blood and stool cultures remained negative. Urinalysis was also negative. When repeated, abdominal radiographs revealed a markedly dilated colon with thumbprinting and a luminal diameter of 7 cm. She had multiple watery stools and exhibited abdominal distention between each bowel movement. On hospital day 4 she had a sigmoidoscopy to the splenic flexure showing mucosal edema and possible pseudomembranes; therefore, she was switched from intravenous to oral metronidazole. Aspirated stool was again negative for Clostridium difficile toxin. Histology of colonic biopsies revealed active colitis with a neutrophilic infiltrate and surface damage suspicious for an infectious etiology. She continued to spike fevers despite negative cultures and antibiotics and had a persistently elevated erythrocyte sedimentation rate and C-reactive protein. She had a normal Doppler study of her mesenteric blood vessels on hospital day 7. A thyroid-stimulating hormone test was normal, ruling out hyperthyroidism as a cause of fever, weight loss and diarrhea. Because vasculitis was suspected, an ANCA was drawn and rheumatology consultation was requested. Rheumatologic evaluation included negative tests for serum anti-nuclear antibodies and circulating rheumatoid factor. However, on hospital day 8 her ANCA screen was reported as positive with a cytoplasmic staining pattern. The c-ANCA titer was 1:640 (negative, <1:40). Further evaluation of the positive c-ANCA by enzyme-linked immunosorbent assay revealed that antibodies to PR-3 were present (226 units; normal range, 0 to 20 units). Anterior-posterior and lateral chest radiographs showed clear lung fields with normal pulmonary vasculature and no pleural effusion. A tentative diagnosis of WG was made and she was started on 15 mg/kg per day of intravenous methylprednisolone and 1 mg/kg per day of oral cyclophosphamide. Her fevers and diarrhea resolved within 24 hours of initiating this therapy and she remained afebrile for the duration of her hospitalization. An echocardiogram on hospital day 9 showed a small pericardial effusion, coronary artery dimensions at the upper limit of normal for age and no coronary aneurysms. Ophthalmologic examination revealed no retinal vessel involvement. After beginning steroid therapy, her erythrocyte sedimentation rate and C-reactive protein improved immediately, diarrhea ceased and her bowel distention resolved. Computerized tomography of the sinuses was normal. Three days later, direct nasopharyngoscopy, bronchoscopy and aortic and mesenteric angiography were performed. The endoscopic evaluation of the airway was negative. Examination of the oropharynx under anesthesia revealed a small, healing buccal ulcer, but no diagnostic abnormality was found histologically. Angiography revealed multiple irregularities of the tertiary branches of the superior mesenteric artery, consistent with small vessel vasculitis. No changes were made in the anti-inflammatory regimen and the child was discharged home to take the following orally administered medications: prednisone 2 mg/kg per day, oral cyclophosphamide 1.5 mg/kg per day, ranitidine 30 mg twice daily and trimethoprim/sulfamethoxazole for Pneumocystis pneumonia prophylaxis. DISCUSSION Vasculitis with high titer antibodies to PR-3 is nearly always a result of WG. Isolated gastrointestinal involvement in WG has been reported but it is rare (5). Our patient did not have any respiratory tract or renal involvement. The most common findings of WG include the following: 1) necrotizing granulomatous inflammation of the upper or lower respiratory tracts, 2) systemic or focal necrotizing vasculitis involving both arteries and veins and 3) focal necrotizing glomerulonephritis (13). According to the American College of Rheumatology, the presence of two or more of the following is required to make the diagnosis: nasal or oral inflammation, abnormal chest radiograph, abnormal urinary sediment and granulomatous inflammation on biopsy (1). Our patient had none of these criteria; rather, she presented with prolonged fever, general malaise and watery stools. An additional unusual feature is her youth. Most patients present during adolescence or adulthood. Another ANCA-positive vasculitis syndrome to be considered in this patient is MPA. Most such patients exhibit an ANCA with a perinuclear-staining pattern (p-ANCA) with antibodies to myeloperoxidase. However, 30% exhibit a pattern identical with our patient, c-ANCA/PR-3 (13). WG can present without typical respiratory and renal manifestations. Absence of nasopharyngeal or cutaneous lesions in a patient with visceral disease makes it difficult to obtain biopsy evidence of vasculitis. It has been reported that the only histopathologic finding of WG may be a nonspecific inflammatory reaction without evidence of vasculitis or necrotizing granulomas (13). Vasculitis in these cases is best demonstrated by angiography. One other ANCA-positive small vessel vasculitis is Churg-Strauss syndrome, which is typically associated with p-ANCA and usually accompanied by asthma (2). These patients tend to have a peripheral eosinophilia as well as an eosinophilic infiltrate in biopsy specimens. Our patient has subsequently experienced chronic cough but no bronchospasm, eosinophilia or other findings consistent with Churg-Strauss syndrome. SUMMARY This patient presented with severe diarrhea and intestinal vasculitis. Her disease can be narrowed to vasculitides involving small vessels and further still to c-ANCA/PR-3 positive vasculitis. Of the three possible causes, Churg-Strauss can be immediately ruled out on the basis of clinical criteria. Most patients with MPA have a different laboratory profile characterized by p-ANCA and a positive anti-myeloperoxidase antibody (2). Approximately 30% of patients with MPA, however, have the same antibody findings as our patient, viz., c-ANCA/PR-3 positive. There are rare reports of intestinal complications such as intestinal bleeding in individuals with MPA (14). Whether WG and the PR-3 positive type of MPA are different disorders is unclear. Distinguishing characteristics between the two disorders include the younger age of onset in MPA (7 to 9 years of age in MPA versus adolescents to adults in WG) (1) and the presence of necrotizing granulomas in WG. Treatment for both of these conditions is the same, and a definitive distinction at diagnosis is not essential. Because these vasculitic disorders are very aggressive, early and vigorous treatment is critical. The median survival in typical untreated WG involving lungs or kidneys is only 5 months. Survival improves to 12.5 months with the use of steroids. In a National Institutes of Health series, 91% of patients treated with both steroids and cyclophosphamide experienced symptomatic improvement and 75% went into remission. Fifty percent of patients with an initial remission had one or more relapses, and patient survival at 2 years was 87% (13). Regardless of the label given to our patient's vasculitis, her isolated intestinal presentation is unusual. Vasculitis must be considered in any child with prolonged fever, marked acute phase response and clinical, radiographic and endoscopic evidence of colitis. Prompt diagnosis and treatment is essential." @default.
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• W1972503540 title "An Unusual Intestinal Presentation of C-ANCA/PR-3 Positive Vasculitis in a Child" @default.
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