Diffuse Large B Cell Lymphoma Mimicking Granulomatosis with Polyangiitis

Table 1

Analysis of cases with gastrointestinal symptoms in the early course of granulomatosis and polyangiitis (one year or less).

Author, year of publication	Gender, age (years)	Presenting symptoms	Disease duration	APR elevation	Concomitant respiratory system/kidney/other involvement	CT/endoscopic findings	Biopsy findings	ANCA
Yoshikawa et al., 2014 [3]	Male, 30	Oral ulcer, bloody stool	1 month	ESR, CRP	Fever	CT: thickening of the transverse colon wall	Superficial inflammatory cell infiltration and edema noted in lamina propria mucosae	PR3
Sinnott et al., 2013 [4]	Male, 29	Bloody diarrhea	2 months	ESR, CRP	Lung/kidney	CT: inflammatory changes in caecum and ascending colon	No bowel biopsy	PR3
Dag et al., 2013 [5]	Male, 29	Bloody stool, rectal bleeding	6 months	CRP, ESR	Lung, kidney, joints, skin	CT: distal ileum perforations; Colonoscopy: ulcers in ileum, cecum, colon	Active chronic inflammation and ulcer bases	PR3
Qian et al., 2010 [6]	Female, 79	Bloody diarrhea	Presenting symptom	Not reported	Pulmonary hemorrhage, acute renal failure	Colonoscopy: pancolitis with numerous ulcers of varying sizes	Mucosal ulcerations with foci of acute inflammation and hemorrhage; Kidney biopsy: crescentic and necrotizing glomerulonephritis	PR3
Samim et al., 2010 [7]	Male, 35	GI bleeding	1-2 weeks	CRP	Sinusitis/kidney/arthritis/skin rash	CTA: bleeding from one mesenteric artery; Endoscopy: mucosal ulcerations	Central ulcerative inflammation and occluded small arteries in intestinal wall	PR3
Marie et al., 2010 [8]	Male, 31	Epigastric pain, rectal bleeding	Before diagnosis of GPA	ESR, CRP	Arthritis, purpura	Colonoscopy: multiple rectal and sigmoidal ulcerations with spontaneous bleeding	Inflammatory cell infiltrates surrounding small vessels	PR3
Deniz et al., 2007 [9]	Male, 44	Acute abdomen	4 weeks	Not reported	Sinusitis, lung cavitation nodules	CT: intra-abdominal free air and fluid, small bowel wall thickening, chronic splenic infarction	Ulcerations, necrotizing transmural granulomatous inflammation, fibrinoid necrosis of small to medium-sized submucosa vessels	ANCA
Kuwahara et al., 2006 [10]	Male, 30	Bloody diarrhea, oral and anal ulcers	Before diagnosis of GPA	CRP	Nasal bleeding, skin and mucosal ulcers, conjunctivitis	Colonoscopy: multiple ulcerations with irregular shapes, macroscopic bleeding, pseudopolyposis in terminal ileum, colon, rectum	Inflammation and epithelioid granuloma with multinuclear giant cells	PR3
Socas Macías et al., 2005 [11]	Male, 28	Oral ulcers, diarrhea, recurrent bowel obstruction	Before diagnosis of GPA	Not reported	Nasal and upper airway, skin rash with necrosis, glomerulonephritis	Colonoscopy and GIT X-rays: ulcers and structures due to ulceration in terminal ileum and colon (signs compatible with Crohn's disease)	Endoscopic biopsy: features compatible with Crohn’s disease in large bowel and terminal ileum; Autopsy: small vessel vasculitis (arterioles and venules), occasional histiocytes around vessels forming loose granulomas with scattered multinuclear giant cells	Negative
Chow et al., 2003 [12]	Male, 46	Melena	4 weeks	Not reported	Sinusitis, glomerulonephritis	Endoscopy: patchy ulceration of small bowel mucosa; Nuclear medicine scan: proximal jejunal blood loss	Resected jejunum: small vessel vasculitis of bowel, mucosal ulceration, submucosa inflammation, fibrosis	c-ANCA
Fallows et al., 2000 [13]	Female, 34	Odynophagia	4 weeks	ESR	Otitis, conjunctivitis, skin rash and ischemia, arthritis, mild kidney involvement	Esophagoscopy: multiple punch-out ulcers	Fibrinoid necrosis with ulcerations and inflammatory cells infiltration surrounding small blood vessels	c-ANCA
Shaikh et al., 2006 [14]	Male, 44	Abdominal pain, peritonitis	Several weeks	ESR	Polyarthralgia, skin rash, polyneuropathy	X-ray and abdominal CT: pneumoperitoneum; Laparotomy (three times): multiple perforations in ileum, colonic ischemia	Resected ileum: vasculitis with fibrinoid necrosis	c-ANCA
Walton and Leggat, 1956 [15]	Female, 42	Oral and tongue ulcers	10 months	Not reported	Fever, anosmia and nasal ulcers, deafness, lung infiltrates and pleurisy, arthritis, rash, neuritis, kidney involvement	ND	Autopsy (data restricted to GIT): multiple spleen solitary granulomata similar to those in lung, signs of vasculitis in pancreas	ND
Tokuda et al., 1989 [16]	Male, 37	Gingival ulcers, weight loss, abdominal pain, distention, ascites, peritonitis due to ileum perforation	Nasal symptoms 2 years; systemic disease several weeks	CRP	Rhinorrhea, arthralgia, fever, nasal blockade, necrotizing glomerulonephritis	X-ray: abdominal ascites	Biopsy from perforated ileum, granulomatous vasculitis	ND
Srinivasan and Coughlan, 1999 [17]	Female, 56	Crampy abdominal pain, perforation, weight loss	8 weeks	ESR, CRP	Rhinorrhea, arthralgia, fever, nasal bridge swelling	Barium study: normal; Laparotomy: areas of perforation	Granulomatous reaction surrounding perforation without inflammation	c-ANCA
Geraghty et al., 1986 [18]	Male, 46	Weight loss, palate ulcerations, peritonitis	4–8 weeks	ESR	Fever, lung and kidney involvement, fingers and toes ischemia	Chest X-ray: free air in abdominal cavity; Laparotomy: inflammation of entire small bowel with numerous areas of necrotic ulceration, multiple perforations in terminal ileum	Autopsy: large perforations of ascending colon and multiple punched out ulcers of distal small bowel and proximal large bowel; granulomata in lungs, spleen, and prostate	RF
Haworth and Pusey, 1984 [19]	Female, 43	Mouth ulcer, bloody mucous diarrhea, weight loss, anorexia	11 months	Not reported	Sinusitis, otitis, deafness, arthritis, iritis, rash, kidney involvement	Sigmoidoscopy: ulcerated rectal mucosa with spontaneous bleeding; Barium meal: narrowed terminal ileum and cecum; Barium enema: ulcerated and ragged cecum	Biopsy: neutrophilic infiltration in rectal and sigmoid mucosa	RF
McNabb et al., 1982 [20]	Male, 50	Pharyngeal and mouth ulcers, abdominal pain, vomiting, peritonitis	Nine months symptoms, GIT symptoms a week after diagnosis	ESR	Arthralgia, nasal blockade, epistaxis, pleurisy, hemoptysis, fingers nodules, proteinuria, renal failure	Laparotomy: multiple ileum ulcers	Biopsy: nonspecific ulcerations	RF
Aymard et al., 1990 [21]	Male, 46	Anal ulcerations	Six months before systemic vasculitis	ESR	Weight loss, polyarthralgia, otalgia, hearing loss, lt. peripheral fascial nerve palsy, hemoptysis	Colonoscopy: normal; X-rays of small intestine normal; Chest X-ray: right pulmonary apical lesion with cavitation; CT: mastoiditis and otitis media	Necrotizing vasculitis, ulcerations and microgranuloma	ANCA
Akça et al., 2005 [22]	Male, 56	Tongue plaques, intestinal perforation	Before diagnosis	ESR, CRP	Pulmonary nodules, severe skin vasculitis	Chest and abdomen X-ray: free air under the diaphragm	Mucosal ulceration, mixed inflammatory cells infiltration and transmural infarcts	c-ANCA

Notes. APR = acute phase reactant; CT = computed tomography; ANCA = anti-neutrophil cytoplasmic antibody; ESR = erythrocyte sedimentation rate; CRP = C-reactive protein; PR3 = proteinase 3; GI = gastrointestinal; CTA = computed tomographic angiography; GPA = granulomatosis with polyangiitis; c-ANCA = cytoplasmic anti-neutrophil cytoplasmic antibody; ND = not done.