title: Centrilobular fibrosis: an underrecognized pattern in systemic sclerosis.
abstract: BACKGROUND
  The impressive association of lung involvement and gastroesophageal reflux in scleroderma raises the possibility of a cause-effect relationship.
  
  
  OBJECTIVE
  To determine clinical, radiological and histopathological features of systemic sclerosis (SSc) patients according the presence or absence of centrilobular fibrosis (CLF).
  
  
  METHODS
  Twenty-eight SSc patients with lung involvement were submitted to open lung biopsy and the specimens classified for the presence of CLF (bronchocentric distribution of the lesions and intraluminal matter according to the classification of idiopathic interstitial pneumonia). HRCT, pulmonary function tests and esophageal analysis were also performed. Subsequently, cyclophosphamide was introduced for the nonspecific interstitial pneumonia subgroup and antireflux treatment was intensified for isolated CLF patients.
  
  
  RESULTS
  Isolated CLF was found in 21% of the biopsies and also found associated to nonspecific interstitial pneumonia in 84% of these patients. The other 3 cases had usual interstitial pneumonia, pulmonary hypertension and respiratory bronchiolitis-associated interstitial lung disease. The histopathological analysis revealed that all 6 patients with isolated CLF had the bronchocentric distribution and intraluminal basophilic content, with foreign bodies detected in one third of them. The central distribution of lung involvement on HRCT was found in 67% of these patients with a consistent patchy distribution (100%). Ground glass (67%) and consolidation (33%) were the predominant patterns found. The constant clinical finding in all isolated CLF cases was dyspnea, esophageal abnormalities and a moderate lung impairment (FVC: 63.83 +/- 16.31%; DLCO: 61.66 +/- 18.84%). Lung function parameters in isolated CLF patients remained stable after 1 year of exclusively intensive antireflux treatment (FVC, p = 0.23; DLCO, p = 0.59).
  
  
  CONCLUSIONS
  The novel description of CLF pattern in SSc lung disease with peculiar histological, tomographic and clinical features will certainly contribute to a more appropriate therapeutic approach.
authors.affiliation: Division of Rheumatology, University of São Paulo, São Paulo, Brazil. romy.c@uol.com.br
authors.forename: Romy B C
authors.initials: RB
authors.lastname: de Souza
authors.forename: Claudia T L
authors.initials: CT
authors.lastname: Borges
authors.forename: Vera L
authors.initials: VL
authors.lastname: Capelozzi
authors.forename: Edwin R
authors.initials: ER
authors.lastname: Parra
authors.forename: Fabio B
authors.initials: FB
authors.lastname: Jatene
authors.forename: Jorge
authors.initials: J
authors.lastname: Kavakama
authors.forename: Ronaldo A
authors.initials: RA
authors.lastname: Kairalla
authors.forename: Eloisa
authors.initials: E
authors.lastname: Bonfá
mesh_terms: D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004947:Esophagus / Q000000981:diagnostic imaging / Q000473:pathology; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D008875:Middle Aged; D011446:Prospective Studies; D054328:Proton Pump Inhibitors / Q000627:therapeutic use; D012129:Respiratory Function Tests; D012595:Scleroderma, Systemic / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D014057:Tomography, X-Ray Computed
chemical_list: D018501:Antirheumatic Agents; D054328:Proton Pump Inhibitors; D003520:Cyclophosphamide