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Table 1 Patient characteristics

From: A case series in patients with enteropathy and granulomatous diseases

Patient A B C
Major complaints Sicca syndrome, arthralgias, chronic diarrhoea, weight loss Dyspnoea on exertion, chronic diarrhoea, weight loss, gait abnormalities Chronic diarrhoea since childhood, weight loss, arthralgias
Malabsorptive syndrome    
Age at dx 33 years 34 years 35 years
Duodenal histology Total villous atrophy Total villous atrophy, crypt hyperplasia, intraepithelial lymphocytosis Total villous atrophy, crypt hyperplasia
Serology Tg-IgA positive Tg-IgA repeatedly negative, AIE-75 antibodies elevated Gliadin-IgA/IgG elevated
Response to GFD Temporary improvement of symptoms, normalization of histologic and serologic findings No improvement of symptoms or histologic findings Temporary improvement of symptoms, normalization of histologic and serologic findings
Currently on GFD Yes No Yes
Granulomatous disease    
Age at dx 36 years 22 years 35 years
Disease manifestations Pulmonary disease Pulmonary disease, abdominal lymphadenopathy Erythema nodosum, abdominal lymphadenopathy
Histologic findings Bronchial epithelioid-cell granulomas Bronchial and abdominal epithelioid-cell granulomas Abdominal epithelioid-cell granulomas
Other diagnoses Sjögren’s syndrome, collagenous colitis Warm antibody hemolytic anaemia, autoimmune hepatitis, CIDP, progressive spastic paraparesis  
  1. dx, diagnosis; Tg, transglutaminse; AIE-75, autoimmune enteropathy related antigen, 75 kDa; CIDP, chronic inflammatory demyelinating polyneuropathy