Case Presentation:

A 35‐year‐old white man with a past history significant for gastroesophageal reflux disease presented with a 1‐year history of unexplained arthralgias and a 2‐day history of hematochezia. His hematochezia was thought to be secondary to indomethacin, which he was taking for arthralgias. Upper gastrointestinal endoscopy revealed duodenilis. Colonoscopy done to rule out inflammatory bowel disease was normal. Connective tissue disease was suspected as a cause of arthritis and evaluation was positive for tissue transglutaminase and anliendomysial antibodies. Repeat upper gastrointestinal endoscopy with duodenal biopsy revealed villous abnormalities consistent with celiac disease, for which he was started on a gluten‐free diet. His serum eosinophil count was 36%. At this time his kidney function started worsening. Serum creatinine of 1.3 mg/dL on presentation rose to 2.2 mg/dL. Urine analysis was positive for hematuria, proteinuria, and red cell casts. Lupus panel and other immunologic markers were negalive except for proteinase‐3 antibodies (c‐ANCA) that were greater than 100 μg/mL. Renal biopsy showed pauci‐immune, necrotizing and crescentic glomerulonephritis without granulomas. He was started on prednisone and cyclophosphamide, which was changed to azathioprine after clinical improvement The patient, currently, is in stage 3 chronic kidney disease with creatinine of 1.56 mg/dL. Urine microalbumin to creatinine ratio was 5799 at initiation of treatment and dropped significantly to 507. The clinical, biochemical, and endoscopic picture of celiac disease appears to have complclcly resolved with the gluten‐free diet.


Celiac disease occurs in approximately 1% of the population worldwide. The disease is underdiagnosed primarily because of a delay in diagnosis rather than delay in seeking health care. The classic presentation of celiac disease with malabsorption is decreasing, whereas other modes of presentation like iron‐deficiency anemia, unexplained arthritis, or incidental discovery at endoscopy done for reflux or dyspepsia are on the rise. There have been many case reports of arthritis being the initial manifestation of celiac disease. Several studies have shown the association of celiac disease with membranous nephropathy. IgA nephropathy, and membranoproliferative glomerulonephritis. This is one of the few reported cases of celiac disease with c‐ANCA positive, pauci‐immune, necrotizing, and crescentic glomerulonephritis without granulomas.


Clinicians should be aware of the varied presentation of celiac disease. Pauci‐immune glomerulonephritis may be associated with celiac disease Immune characteristics associated with celiac disease increase the risk of chronic kidney disease. A high index of suspicion for other autoimmune diseases and early diagnosis is necessary for favorable oulcomes.

Author Disclosure:

R. Sinnakirouchenan, none; V. Ramalingam, none; A. Shivakumar, none; A. Verma, none.