Centrilobular fibrosis: an underrecognized pattern in systemic sclerosis.
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.
de Souza
Romy B C
RB
Division of Rheumatology, University of São Paulo, São Paulo, Brazil. romy.c@uol.com.br
Borges
Claudia T L
CT
Capelozzi
Vera L
VL
Parra
Edwin R
ER
Jatene
Fabio B
FB
Kavakama
Jorge
J
Kairalla
Ronaldo A
RA
Bonfá
Eloisa
E
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
D018501:Antirheumatic Agents; D054328:Proton Pump Inhibitors; D003520:Cyclophosphamide