Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis

Report of 6 and 12 cases with similar histopathology

Bijayee Shrestha, Hiroshi Sekiguchi, Thomas V. Colby, Paolo Graziano, Marie Christine Aubry, Thomas C. Smyrk, Andrew L. Feldman, Lynn D. Cornell, Jay H. Ryu, Suresh T. Chari, Amylou C. Dueck, Eunhee S. Yi

Research output: Contribution to journalArticle

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Abstract

Autoimmune pancreatitis (AP) is one manifestation of a systemic, steroid-responsive disease with elevated serum IgG4 and characteristic histopathology, including increased IgG4-positive (+) plasma cells in the tissue. The histopathology of pulmonary IgG4 disease has not been well established. Six lung biopsies from patients with documented AP were studied, along with 12 additional cases showing similar pulmonary histopathology. For comparison, we examined Erdheim-Chester disease (n=3), pulmonary Sjögren syndrome (n=19), inflammatory myofibroblastic tumor (n=10), various inflammatory and interstitial lung disease (n=61), and nodal or extranodal Rosai-Dorfman disease (RD) in adults (n=8). All cases were stained for IgG4 and scored as 1, 2, and 3 as described in AP according to the following criteria: 0, 30. Five lung biopsies from AP patients showed IgG4 score of 3, and 1 had a score of 2. Consistent findings in lung biopsies of AP patients included endothelialitis of pulmonary vessels, active fibrosis, lymphangitic inflammatory infiltrates rich in plasma cells and histiocytes with or without nodule formation, and fibrinous pleuritis. Prominent lymphatic dilatation with histiocytes showing emperipolesis of lymphocytes was also seen. All 12 additional cases showing these histologic features also had the IgG4 score of 2 or 3. Among other conditions, an IgG4 score of 2 or 3 was seen in 6 of 8 RD, 4 of 10 inflammatory myofibroblastic tumors, and 8 of 61 inflammatory and interstitial lung disease, but in none of the rest. In conclusion, distinctive pulmonary histopathology was associated with increased IgG4+ cells in both AP patients and those unknown for AP status. The significance of increased IgG4+ cells in high proportion of RD cases merits further study as does overlap of RD and IgG4 disease.

Original languageEnglish
Pages (from-to)1450-1462
Number of pages13
JournalAmerican Journal of Surgical Pathology
Volume33
Issue number10
DOIs
Publication statusPublished - Oct 2009

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Plasma Cells
Pancreatitis
Immunoglobulin G
Lung
Sinus Histiocytosis
Histiocytes
Interstitial Lung Diseases
Biopsy
Emperipolesis
Erdheim-Chester Disease
Pleurisy
Lung Diseases
Dilatation
Neoplasms
Fibrosis
Steroids
Lymphocytes

Keywords

  • Autoimmune pancreatitis
  • Erdheim-Chester disease
  • IgG4
  • Inflammatory and interstitial lung disease
  • Inflammatory myofibroblastic tumor
  • Rosai-Dorfman disease
  • Sjögren syndrome

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine
  • Surgery

Cite this

Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis : Report of 6 and 12 cases with similar histopathology. / Shrestha, Bijayee; Sekiguchi, Hiroshi; Colby, Thomas V.; Graziano, Paolo; Aubry, Marie Christine; Smyrk, Thomas C.; Feldman, Andrew L.; Cornell, Lynn D.; Ryu, Jay H.; Chari, Suresh T.; Dueck, Amylou C.; Yi, Eunhee S.

In: American Journal of Surgical Pathology, Vol. 33, No. 10, 10.2009, p. 1450-1462.

Research output: Contribution to journalArticle

Shrestha, Bijayee ; Sekiguchi, Hiroshi ; Colby, Thomas V. ; Graziano, Paolo ; Aubry, Marie Christine ; Smyrk, Thomas C. ; Feldman, Andrew L. ; Cornell, Lynn D. ; Ryu, Jay H. ; Chari, Suresh T. ; Dueck, Amylou C. ; Yi, Eunhee S. / Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis : Report of 6 and 12 cases with similar histopathology. In: American Journal of Surgical Pathology. 2009 ; Vol. 33, No. 10. pp. 1450-1462.
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