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2017 ; 141
(7
): 960-969
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Interstitial Pneumonia With Autoimmune Features: Value of Histopathology
#MMPMID28467213
Adegunsoye A
; Oldham JM
; Valenzi E
; Lee C
; Witt LJ
; Chen L
; Montner S
; Chung JH
; Noth I
; Vij R
; Strek ME
; Husain AN
Arch Pathol Lab Med
2017[Jul]; 141
(7
): 960-969
PMID28467213
show ga
CONTEXT: - Patients with idiopathic interstitial pneumonia may display evidence
of autoimmunity without meeting criteria for a defined connective tissue disease.
A recent European Respiratory Society/American Thoracic Society statement
proposed research criteria for interstitial pneumonia with autoimmune features
(IPAF), which includes findings from the clinical, serologic, and morphologic
domains. OBJECTIVES: - To investigate the importance of histopathologic criteria
within the morphologic domain and to report our methodology for identifying these
features. DESIGN: - Patients with idiopathic interstitial pneumonia at the
University of Chicago who underwent surgical lung biopsy or lung transplantation
were assessed for IPAF histopathologic features, using the initial pathology
interpretation in the electronic records. A focused rereview of available slides
by a pulmonary pathologist was then performed for patients who failed to meet
IPAF criteria on initial pathology assessment. RESULTS: - Of 422 patients with
idiopathic interstitial pneumonia, 176 (41.7%) underwent surgical lung biopsy or
lung transplant. Forty-six of those 176 patients (26.1%) met IPAF criteria by
initial pathology interpretation and a positive clinical or serologic feature. Of
the remaining 130 patients, 73 (56.2%) met either the clinical or serologic
domains without meeting the morphologic domain, whereas 36 (27.7%) had slides
available for pathology rereview. This rereview demonstrated nonspecific
interstitial pneumonia in 8 of 36 patients (22.2%) and lymphoplasmacytic
infiltrates in 6 of 36 patients (16.7%), resulting in an additional 7 of 36
patients (19.4%) with idiopathic interstitial pneumonia that met the IPAF
criteria. In IPAF, pulmonary vasculopathy was the most prevalent finding (45 of
84; 53.6%) and predicted increased mortality (hazard ratio, 2.5; P = .04).
CONCLUSIONS: - Using a methodological approach to identifying IPAF pathology, we
demonstrate a significant increase in the number of patients meeting IPAF
criteria because of focused pathologic review and highlight the prognostic value
of the IPAF pathologic findings.
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