Pathological Features of Interstitial Pneumonia Associated with Undifferentiated Connective Tissue Disease
Y Kashima, H Taniguchi, Y Kondoh, K Ichikado, M Okamoto, K Kataoka, R Egashira, Y Soejima, T Tanaka, S Shimizu, J Fukuoka. Toyama University Hospital, Toyama, Japan; Tosei General Hospital, Aichi, Japan; Saiseikai Kumamoto Hospital, Kumamoto, Japan; Kyushu Medical Center, Fukuoka, Japan; Shiroishi Hospital, Saga, Japan
Background: NSIP pattern is the commonest pathological pattern in cases with Connective Tissue Disease (CTD). Meanwhile, cases which show clinical features of CTD but do not meet the criteria of American College of Rheumatology are now recognized as Undifferentiated CTD (UCTD). Recent report raises the question if majority of idiopathic NSIP may be the lung manifestation of UCTD. Further analysis is necessary to clarify the histological features of UCTD. Herein, we report the histological features of UCTD cases in our consultation case profile.
Design: 145 VATS in archives of Toyama University Hospital obtained from 2007-2009 were reviewed. We excluded the cases clinically diagnosed as CTD and picked up cases that meet the criteria of UCTD (both positive for serum antibody/ESR and symptoms of CTD). Eventually, 8 cases were selected. We investigated the pathological diagnoses according to ATS-ERS 2002 classification and histological findings; distribution of fibrosis (D), dense fibrosis (F), honeycombing (HC), fibroblastic focus (FF), organizing pneumonia (OP), lymphoid follicle (L), pleural thickening (P), peribronchiolar metaplasia (PBM), reactive type2 cell (RT) vasculitis (V), wall thickening of pulmonary artery (PA), hyaline membrane(HM), fibrin (FB) and normal lung (N).
Results: Eight cases consisted of 2 males and 6 females with age from 49 to 74. Smoking states were available to 7; 1 current, 1 ex-smoker and 5 never smoker. Radiological findings were available for 4 cases that revealed 3 NSIP and 1 unclassifiable pattern. Pathological diagnoses were 4 NSIP, 2 discordant UIP, 1 probable UIP and 1 LIP. As findings, diffuse distribution was found in 6/8 cases. F in 8/8, HC in 3/8, OP in 4/8, FF in 2/8, L in 8/8, P in 8/8, PBM in 7/8, RT in 7/8, V in 0/8, PA in 5/8, HM in 0/8, FB in 2/8, N in 3/8 were detected, respectively.
Conclusions: We investigated the pathological features of interstitial pneumonia associated with UCTD. We found that NSIP was the commonest pattern, although there were some cases with UIP or comorbid UIP. Common pathological findings are lymphoid follicle, pleural thickening, PBM and reactive type2. No vasculitis or hyaline membrane was found. Presence of fibroblastic foci, honeycombing or fibrin was rare.
Monday, March 22, 2010 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 234, Monday Morning