TY - JOUR
T1 - Distinctive pulmonary histopathology with increased IgG4-positive plasma cells in patients with autoimmune pancreatitis
T2 - Report of 6 and 12 cases with similar histopathology
AU - Shrestha, Bijayee
AU - Sekiguchi, Hiroshi
AU - Colby, Thomas V.
AU - Graziano, Paolo
AU - Aubry, Marie Christine
AU - Smyrk, Thomas C.
AU - Feldman, Andrew L.
AU - Cornell, Lynn D.
AU - Ryu, Jay H.
AU - Chari, Suresh T.
AU - Dueck, Amylou C.
AU - Yi, Eunhee S.
PY - 2009/10
Y1 - 2009/10
N2 - 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, <5 (per high power field); 1, 5 to10; 2, 11 to 30; and 3, >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.
AB - 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, <5 (per high power field); 1, 5 to10; 2, 11 to 30; and 3, >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.
KW - Autoimmune pancreatitis
KW - Erdheim-Chester disease
KW - IgG4
KW - Inflammatory and interstitial lung disease
KW - Inflammatory myofibroblastic tumor
KW - Rosai-Dorfman disease
KW - Sjögren syndrome
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UR - http://www.scopus.com/inward/citedby.url?scp=70349666758&partnerID=8YFLogxK
U2 - 10.1097/PAS.0b013e3181ac43b6
DO - 10.1097/PAS.0b013e3181ac43b6
M3 - Article
C2 - 19623032
AN - SCOPUS:70349666758
SN - 0147-5185
VL - 33
SP - 1450
EP - 1462
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
IS - 10
ER -