Tubulointerstitial nephritis (TIN) is the most common form of renal involvement in IgG4-related disease. It is characterized by a dominant infiltrate of IgG4-positive plasma cells in the interstitium and storiform fibrosis. Demonstration of IgG4-positive plasma cells is essential for diagnosis, but the number of IgG4-positive cells and the ratio of IgG4-positive/IgG-positive plasma cells may vary from case to case and depending on the methods of tissue sampling even in the same case. IgG4-positive plasma cells can be seen in TIN associated with systemic lupus erythematosus, Sjögren syndrome, or anti-neutrophil cytoplasmic antibody–associated vasculitis, which further add diagnostic confusion and difficulties. To have a more clear view of IgG4-TIN and to delineate differential points from other TIN with IgG4-positive plasma cell infiltrates, clinical and histological features of IgG4-TIN and its mimickers were reviewed. In the rear part, cases suggesting overlap of IgG4-TIN and its mimickers and glomerulonephritis associated with IgG4-TIN were briefly described.
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Lymphocytic phlebitis of gastrointestinal (GI) tract is a rare diseaes. Approximately 50 cases of lymphocytic phlebitis of the GI tract have been reported. Most of these involved the colon or small intestine and presented as acute abdomen. We report the second case of lymphocytic phlebitis of the stomach. A 73-year-old female complaining of dizziness had endoscopic and computed tomography findings strongly suggested gastric cancer, while gastric biopsy was negative for carcinoma. The partial gastrectomy specimen showed lymphocytic phlebitis involving veins in the submucosa, muscularis propria, and serosa while the adjacent arteries were spared. The veins were mainly surrounded by lymphocytes. When a patient has a lesion in the GI tract that is suggesting cancer without biopsies revealing any carcinoma, the pathologist should recommend a deeper biopsy for a proper examination of the submucosa.
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Localized enterocolic lymphocytic phlebitis is characterized by selective phlebitis involving the small to medium-sized veins and venules, infiltration exclusively by lymphocytes, and no other systemic vasculitis or inflammatory bowel disease. This vasculitis can be a rare cause of intestinal ischemia. We experienced a case of enterocolic lymphocytic phlebitis in a 72-year-old woman, who presented with abdominal pain and distension. The resected colon and terminal ileum showed striking lymphocytic phlebitis affecting the veins and venules of the bowel and mesentery which resulted in ischemic injury of the bowel. This vasculopathy was the only demonstrable cause of ischemia.
Arteritis and arteriolitis was not found. There is no clinical or laboratory evidence or a history of extraintestinal vasculitis. The etiology of this clinicopathological entity has not been elucidated. Herein, we report the clinicopathological findings in this patient who presented with ischemic intestinal necrosis caused by localized intestinal lymphocytic phlebitis associated with thrombosis.
A 55-year-old woman suffered from upper abdominal pain for two months and remained refractile against the anti-ulcer regimen. The palliative gastrectomy specimen revealed multiple shallow ulcerations on the thickened mucosal folds mainly in the antrum and body along the greater curvature where multiple, whitish nodules were found in the submucosa.
Microscopically, individual submucosal nodules clearly corresponded to the necrotizing granulomatous vasculitis which were featured with diffuse fibrinoid necrosis of arterial walls accompanying granuloma formation and heavy infiltration of neutrophils, eosinophils, histiocytes and giant cells. Similar vasculitic lesions involved venules and arterioles. There were scattered vasculitic changes in the liver biopsy specimens and omentum. There were no clinical presentations or serological support of systemic involvement including systemic lupus erythematosus, Henoch-Schoenlein purpura, cryoglobulinemia or Churg-Strauss granulomatous vasculitis. We conclude that this is a hitherto undescribed primary necrotizing granulomatous vasculitis predominantly involving the stomach.
Cytomegalovirus (CMV) infection is an uncommon association with idiopathic inflammatory bowel disease (IBD) often leading to a variety of serious complications. A total of 41 resected cases of IBD were examined to elucidate the pathologic features of intestinal CMV infection which was assessed by histologic examination and confirmed by immunohistochemistry with CMV antibody. Six cases were positve for CMV antibody; five cases in 19 ulcerative colitis (UC, 26.3%) and one case in 22 Crohn's disease (CD, 4.5%). Of 7 cases of the steroid-treated UC group, five cases were superinfected with CMV (71.4%) but none in 12 cases of the steroid-untreated UC group. All of the five CMV-positive cases in UC showed deep ulceration and transmural inflammation, while none of 10 UC cases without above features were CMV positive. Fibrinoid necrosis and thrombi were found in 83.3% of the CMV infected group, while none in the CMV-negative group of UC cases (p=0.01). We conclude that IBD, particularly UC, is susceptible to the CMV infection when steroid hormone is administered, and that deep colonic ulceration, transmural inflammation and fibrinoid necrosis of vasculature may suggest superinfection of CMV in UC patients. It seems that deep colonic ulceration may be the consequence of an ischemic change following vascular luminal occlusion or vasculitis by CMV infection.
A case of Wegener's granulomatosis is described, with special attention focused on the typical histologic findings and involvement of both middle ear and lung. The patient is a 37-year-old man presented with four-month history of cough and sputum. He had a past history of surgery of both ears because of otitis media followed by left facial palsy. Chest radiographs showed variable sized ill defined nodules in both lower lobes with internal airspace consolidation.
Histologic preparations of the open lung biopsy specimens demonstrated a diffusely scattered palisading micro and macrogranulomas with central focus of neutrophils and necrotic collagen surrounded by histiocytes, histiocytic giant cells. Fibrinoid necrosis involved blood vessels and lung parenchyma. Chronic inflammation, diffuse granulation tissue formation and irregular fibrosis are also found in the lung parenchyma. The histologic findings of middle ear which was previously biopsied showed scattered palisading ill defined microgranulomas mixed with fibrotic tissue.
There have been a few reported cases of pancreatic pseudocyst with involvement of adjacent structures including the stomach, duodenum, and even the mediastinum. An intrasplenic pancreatic pseudocyst is an uncommon complication of acute or chronic pancreatitis. We report a case of pancreatic pseudocyst with splenic involvement in a 69-year-old man with a review of literatures. Abdominal CT revealed a 4 cm sized cyst at the pancreatic tail which was extending into the splenic hilum and splenic parenchyma.
Distal pancreatectomy and splenectomy was performed.
Pathologic findings demonstrated a pseudocyst of pancreatic tail which communicated with the infacted splenic parenchyma. Also vasculitis, endothelial hyperplasia and medial calcification of the splenic artery were observed.