Small airway disease associated with Sjögren’s syndrome: Clinico-pathological correlations

SummaryBackgroundRelationships among clinical, physiological, imaging and pathological findings of small airway disease associated with Sjögren’s syndrome have remained unclear.Subjects and methods: We retrospectively studied 14 patients who underwent surgical lung biopsy and who were diagnosed with small airway disease associated with primary or secondary Sjögren’s syndrome. We compared clinical, bronchoalveolar lavage, physiological, imaging and pathological findings between primary and secondary Sjögren’s syndrome. We scored HRCT and pathological abnormalities and investigated correlations among physiological, HRCT and pathological data, changes in physiological parameters and in HRCT scores after two years of treatment, as well as correlations between these values and pathological scores.ResultsBronchoalveolar lavage fluid, physiological, imaging and pathological findings of the airways did not significantly differ between primary and secondary Sjögren’s syndrome. Air trapping on HRCT negatively correlated with MEF50 and MEF25. Although lymphoid cell infiltration and peribronchiolar fibrosis were the most common pathologies, constrictive change scores correlated negatively with MEF50 and MEF25, positively with air trapping scores and negatively with improvements after therapy in MEF50, MEF25 and air trapping.ConclusionsConstrictive change was the most significant determinant of physiological and imaging presentations and of changes in these factors after therapy for small airway disease associated with Sjögren’s syndrome

Pathologic Features and the Classification of Interstitial Pneumonia of Unknown Etiology

Currently, five types of interstitial pneumonia of unknown etiology or IIP have been described; DAD, UIP, BOOP, DIP and LIP. A summary of the features of clinical course, prognosis and therapeutic effects to steroid hormone treatment of the five types of interstitial pneumonia of unknown etiology or IIP is given in Table 15. Among the previous mentioned [table] 52 open lung biopsy cases of interstitial pneumonia of unknown etiology or IIP, for which an open lung biopsy was necessary for diagnosis, the majority of those cases (81%) was IPF (UIP) while idiopathic BOOP occurred as the second in frequency (13%). These data will help in understanding the clinical outcome and the therapeutic response to steroid hormone treatment in cases of interstitial pneumonia of unknown etiology or IIP, because idopathic UIP is slowly progressive and usually not responsive to steroid while idiopathic BOOP is usually responsive to steroid hormone treatment and may regress even spontaneously. Cases of IPF (UIP) should have revolutional modalities for therapy

Comorbid connective tissue diseases and autoantibodies in lymphangioleiomyomatosis: a retrospective cohort study

Abstract Background Lymphangioleiomyomatosis (LAM) and connective tissue diseases (CTDs) occur more frequently among women than men. We investigated the frequency of comorbid CTD and positive serum autoantibody findings in patients with LAM. Methods A total of 152 patients with LAM were prospectively and consecutively registered in the National Hospital Organization Kinki-Chuo Chest Medical Centre cohort. The clinical data were retrospectively analysed, and patients were categorised into the following three groups: a CTD group, a non-CTD-autoantibody-positive group, and a non-CTD-autoantibody-negative group. Results All patients were women. We identified five patients with comorbid CTDs (3.3%): Sjögren’s syndrome (SjS) (n = 3), systemic lupus erythematosus (n = 1), and rheumatoid arthritis (n = 1). One patient with SjS was also diagnosed with antiphospholipid antibody syndrome. The positive rate for anti nuclear antibody was 31.5% and 6.9% at dilution of 1:40 or higher, and those of 1:160 or higher, respectively.  It tended to be lower in patients with LAM than in healthy women. The positive rate for anti-SS-A and anti-SS-B antibody was 7.9% and 1.8%, respectively. No significant differences in age, type of LAM, smoking status, serum vascular endothelial growth factor D level, respiratory function, treatment, or prognosis were observed among the three groups. Conclusions Comorbid CTDs, especially SjS, in LAM patients should be considered

[(18)F]FDG Uptake and PCNA, Glut-1, and Hexokinase-II Expressions in Cancers and Inflammatory Lesions of the Lung

PURPOSE: The aim of this study was to evaluate the relationships among [(18)F]fluorodeoxyglucose ([(18)F]-FDG) uptake, Glut-1 and HK-II expressions, and grade of inflammation in resected lung lesions. MATERIALS AND METHODS: Sixty patients had undergone preoperative (18)F-FDG-PET imaging and thoracotomy. For semiquantitative analysis of (18)F-FDG uptake, partial volume effect corrected maximum standardized uptake values (pSUVs) were calculated. Immunohistochemical staining was performed in resected specimens using anti-Glut-1, anti-HK-II, and anti-proliferative cellular nuclear antigen (PCNA) antibodies, and immunoreactivities were scored as G-, H-, and P-indexes on a five-point scale (0: 0%; 1: ∼20%, 2: ∼40%; 3: ∼60%; 4: ∼80%, and 5: ∼100% percentages of strongly immunoreactive cells). Grade of inflammation was also evaluated. RESULTS: The malignant lesions had higher pSUV and higher G- and H-indexes than nonmalignant lesions. pSUVs correlated with the G- (p < .001), H- (p < .01), and P-indexes (p < 0.01) in malignant lesions. In adenocarcinomas, cancers with lower differentiation showed higher expression of Glut-1 and HK-II than those with higher differentiation. A positive linear regression was observed between pSUVs and the grading of inflammation in nonmalignant lesions (p < 0.05). CONCLUSIONS: Our study indicates that (18)F-FDG uptake in lung cancer correlates well with Glut-1, HK-II, and PCNA expression. For nonmalignant lesions, the presence of a higher inflammatory process correlated with (18)F-FDG uptake