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Antibodies to carbonic anhydrase and IgG4 levels in idiopathic chronic pancreatitis: relevance for diagnosis of autoimmune pancreatitis
  1. L Aparisi1,
  2. A Farre2,
  3. L Gomez-Cambronero3,
  4. J Martinez4,
  5. G De Las Heras5,
  6. J Corts6,
  7. S Navarro7,
  8. J Mora8,
  9. M Lopez-Hoyos9,
  10. L Sabater10,
  11. A Ferrandez11,
  12. D Bautista12,
  13. M Perez-Mateo4,
  14. S Mery3,
  15. J Sastre3
  1. 1Unit of Hepatology, Universitary Clinic Hospital, Valencia, Spain
  2. 2Service of Gastroenterology, Santa Creu and Sant Pau Hospital, Barcelona, Spain
  3. 3Department of Physiology, School of Medicine, University of Valencia, Spain
  4. 4Department of Gastroenterology, University General Hospital, Alicante, Spain
  5. 5Department of Gastroenterology, Marqués de Valdecilla Hospital, Santander, Spain
  6. 6Department of Rheumatology, Universitary Clinic Hospital, Valencia, Spain
  7. 7Department of Gastroenterology, Hospital Clinic and Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain
  8. 8Department of Biochemistry, Santa Creu and Sant Pau Hospital, Barcelona, Spain
  9. 9Department of Immunology, Marqués de Valdecilla Hospital, Santander, Spain
  10. 10Department of Surgery, Universitary Clinic Hospital, Valencia, Spain
  11. 11Department of Pathology, School of Medicine, University of Valencia, Spain
  12. 12Department of Preventive Medicine, Hospital Dr Peset, University of Valencia, Spain
  1. Correspondence to:
    Dr L Aparisi
    Servicio de Hepatología, Hospital Clínico Universitario, Avda. Blasco Ibañez 17, 46010 Valencia, Spain;


Background: Increased serum antibodies against carbonic anhydrase II (CA-II Ab) or IgG4 levels have been reported in cases of autoimmune chronic pancreatitis (ACP).

Aim: To assess the relevance of serum CA-II Ab and IgG4 levels for the diagnosis of ACP in idiopathic CP (ICP) versus alcoholic CP and Sjögren’s syndrome (SS).

Subjects: This was a multicentre study involving 227 subjects divided into four groups: ICP (n = 54), normal controls (n = 54, paired by age and sex with ICP patients), alcoholic CP (n = 86), and SS (n = 33).

Methods: CA-II Ab was measured by ELISA and confirmed by western blotting. A score of easy clinical use with major clinical, morphological, and biochemical parameters for the diagnosis of ACP was applied.

Results: The percentage of patients with increased serum CA-II Ab was higher in the ICP group (28%) than in controls (1.9%) and in patients with alcoholic CP (10.5%), but lower than in patients with SS (64%). The proportion with elevated IgG4 levels was higher in the ICP group (15%) compared with controls (1.9%) and SS (0%) but not significantly different from alcoholic CP (8%). Most ICP patients (7/8) with high IgG4 levels exhibited increased CA-II Ab and a compatible ACP score. A definitive diagnosis of ACP by histological analysis was associated with other autoimmune disorders, an increase in both serum IgG4 and CA-II Ab levels, and IgG4 positive plasma cells.

Conclusions: The increase in serum IgG4 levels was strongly associated with elevated CA-II Ab levels, manifestations compatible with ACP, and lymphoplasmacytic infiltration when surgical specimens were available.

  • ACP, autoimmune chronic pancreatitis
  • CA- II Ab, antibodies to carbonic anhydrase II
  • CP, chronic pancreatitis
  • ELISA, enzyme linked immunosorbent assay
  • ICP, idiopathic chronic pancreatitis
  • SS, Sjögren’s syndrome
  • ANA, antinuclear antibodies
  • chronic pancreatitis
  • autoimmune diseases
  • autoantibodies
  • immunoglobulins
  • lymphoplamacytic infiltration

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  • Conflict of interest: None declared.

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