Centrilobular Fibrosis: An Underrecognized Pattern in Systemic Sclerosis

Souza, Romy Beatriz Christmann de; Borges, Camila Aparecida; Capelozzi, Vera Luíza; Parra, Edwin Roger; Jatene, Fábio Biscegli; Kavakama, Jorge; Kairalla, Ronaldo Adib; Bonfá, Eloísa

doi:10.1159/000156958

Cited by 52 publications

(48 citation statements)

References 61 publications

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“…In patients with CLF alone and treated exclusively with intensive antireflux therapy, the lung disease remained stable for a period of 1 year. (26) In the present study, the prevalence of GERD was 35.7%, and 77.7% of the patients presented at least one typical GERD symptom. Although this prevalence rate is high in relation to that observed in the general population, most studies using esophageal pH-metry in IPF have demonstrated even higher prevalence rates.…”

Section: Resultssupporting

confidence: 47%

Prevalência da doença do refluxo gastroesofágico em pacientes com fibrose pulmonar idiopática

et al. 2009

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Objective: To determine the prevalence of gastroesophageal reflux disease (GERD) and to evaluate its clinical presentation, as well as the esophageal function profile in patients with idiopathic pulmonary fibrosis (IPF). Methods: In this prospective study, 28 consecutive patients with IPF underwent stationary esophageal manometry, 24-h esophageal pH-metry and pulmonary function tests. All patients also completed a symptom and quality of life in GERD questionnaire. Results: In the study sample, the prevalence of GERD was 35.7%. The patients were then divided into two groups: GERD+ (abnormal pH-metry; n = 10) and GERD− (normal pH-metry; n = 18). In the GERD+ group, 77.7% of the patients presented at least one typical GERD symptom. The pH-metry results showed that 8 (80%) of the GERD+ group patients had abnormal supine reflux, and that the reflux was exclusively in the supine position in 5 (50%). In the GERD+ and GERD− groups, respectively, 5 (50.0%) and 7 (38.8%) of the patients presented a hypotensive lower esophageal sphincter, 7 (70.0%) and 10 (55.5%), respectively, presenting lower esophageal dysmotility. There were no significant differences between the groups regarding demographic characteristics, pulmonary function, clinical presentation or manometric findings. Conclusions: The prevalence of GERD in the patients with IPF was high. However, the clinical and functional characteristics did not differ between the patients with GERD and those without.Keywords: Pulmonary fibrosis; Gastroesophageal reflux; Prevalence; Manometry; Esophageal pH monitoring. ResumoObjetivo: Determinar a prevalência da doença do refluxo gastroesofágico (DRGE) e sua apresentação clínica, além do perfil funcional do esôfago em pacientes com fibrose pulmonar idiopática (FPI). Métodos: Foram avaliados prospectivamente 28 pacientes com FPI. Os pacientes foram submetidos à esofagomanometria estacionária, pHmetria de 24 h e testes de função pulmonar, assim como responderam a questionários sobre sintomas e qualidade de vida em DRGE. Resultados: A prevalência de DRGE foi de 35,7%. Os pacientes foram divididos em dois grupos: grupo DRGE+ (pHmetria anormal; n = 10), e grupo DRGE− (pHmetria normal; n = 18). No grupo DRGE+, 77,7% dos pacientes apresentavam pelo menos um sintoma típico de DRGE. Nesse grupo de pacientes, 8 (80%) apresentaram refluxo em posição supina, e 5 (50%) apresentaram refluxo exclusivamente nessa posição. Nos grupos DRGE+ e DRGE−, respectivamente, 5 (50,0%) e 7 (38,8%) dos pacientes apresentaram hipotonia do esfíncter esofágico inferior, assim como 7 (70,0%) e 10 (55,5%) apresentaram hipomotilidade esofágica. Não houve diferenças significativas entre os grupos quanto a características demográficas, função pulmonar, apresentação clínica ou achados manométricos. Conclusões: A prevalência de DRGE nos pacientes com FPI foi elevada. Entretanto, as características clínicas e funcionais não diferiram significativamente entre os pacientes com e sem DRGE.Descritores: Fibrose pulmonar; Refluxo gastroesofágico; Prevalência; Manome...

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Section: Resultssupporting

confidence: 47%

Prevalência da doença do refluxo gastroesofágico em pacientes com fibrose pulmonar idiopática

et al. 2009

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“…Some studies have suggested that GOR may contribute to idiopathic pulmonary fibrosis [8,9] and SSc-ILD [10-12] through repeated microaspiration of gastric content into the respiratory tract. Other arguments supporting this hypothesis are the preferential localisation of SSc-ILD in the lower lung zones [19] and the frequent centrilobular localisation of fibrosis in SSc patients with GOR [21]. However, the preliminary studies provide a low level of evidence due to their cross-sectional design or short follow-up periods, small numbers of patients and, in some studies, absence of multivariate analysis.…”

Section: Lung Involvement Of Systemic Disease M Gilson Et Almentioning

confidence: 99%

Prognostic factors for lung function in systemic sclerosis: prospective study of 105 cases

Gilson¹,

Zerkak²,

Wipff³

et al. 2009

Eur Respir J

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The aims of the present study were to identify prognostic factors for systemic sclerosis (SSc)-related interstitial lung disease and to clarify the possible causative role of manometric oesophageal involvement.Consecutive SSc patients underwent pulmonary function tests and oesophageal manometry. They were included in the study if pulmonary function tests were repeated .12 months after baseline. The primary end-point was a decrease of o10% of the predicted value in forced vital capacity (FVC). The secondary end-points were a decrease of o15% pred in lung carbon monoxide diffusing capacity (DL,CO) and a decrease of o20% pred in FVC.Of the 105 patients (45 diffuse SSc; median disease duration 2.0 yrs), 23 (23%) had a FVC of ,80% pred, 60 (59%) had a DL,CO of ,80% pred and 57 (54%) showed severe oesophageal hypomotility at baseline. Over 72¡46 months, 29 (28%) patients displayed a decrease of o10% pred in FVC, 39 (40%) of 98 patients displayed DL,CO decline and 19 (18%) patients displayed a decrease of o20% pred in FVC. On multivariate analysis, diffuse SSc was a significant predictor for a decrease of o10% pred in FVC (p50.01). No other predictor of a decrease in pulmonary function was identified.Only diffuse SSc was predictive of a decrease in pulmonary function in this early-SSc cohort. This does not support preliminary data suggestive of a causative role of oesophageal involvement.

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“…The histopathological characteristics of microaspiration-related changes have been poorly studied. Bronchiolocentric organizing pneumonia, foreign bodies, intraluminal basophilic content have been described in surgical lung biopsies of ILD-SSc with possible microaspiration (16,17). However, those histological features probably represent a small subgroup of ILD-SSc, while the vast majority of these patients show "pure" non specific interstitial pneumonia (NSIP) histopathologic pattern and GER is virtually present in all.…”

Section: The Role Of Pepsinmentioning

confidence: 99%

The role of gastro-esophageal reflux (GER) in systemic sclerosis and lung fibrosis

Lauretis¹

2014

SOB

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SummarySystemic sclerosis (SSc) is an autoimmune condition characterized by tissue fibrosis of the skin and internal organs. A significant subgroup of SSc patients develops progressive interstitial lung disease (ILD) leading to pulmonary fibrosis similar to IPF (idiopathic pulmonary fibrosis). Gastro-esophageal reflux (GER) has been suggested as a driving factor in the pathogenesis of both SSc-ILD and IPF. The esophagus is affected in 50-82% of patients with SSc. Gastric reflux may be liquid, gaseous, or particulate; acid or nonacid; distal (localized to the distal oesophagus) or proximal (reaching the proximal oesophagus and pharynx). Reflux to the proximal oesophagus, which is intuitively linked to microaspiration into the lungs, appears to be quite common in patients with ILD-SSc and IPF. Importantly, a significant proportion of GER reflux is asymptomatic. Concentration of pepsin and bile acids in bronchoalveolar lavage (BAL) and exhaled breath condensate (EBC) have been investigated as biomarkers of microaspiration in various respiratory diseases. The confirmation of a causative link of microaspiration in the genesis and progression of lung fibrosis would have a major impact in the management of ILD-SSc patients. It is likely that proteases such as pepsin, and not the acidity, are the primary target for future therapies. Potent inhibitors specific for proteases, e.g. pepstatin, are available and have been tested in phase-III clinical trials.

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Centrilobular Fibrosis: An Underrecognized Pattern in Systemic Sclerosis

Cited by 52 publications

References 61 publications

Prevalência da doença do refluxo gastroesofágico em pacientes com fibrose pulmonar idiopática

Prevalência da doença do refluxo gastroesofágico em pacientes com fibrose pulmonar idiopática

Prognostic factors for lung function in systemic sclerosis: prospective study of 105 cases

The role of gastro-esophageal reflux (GER) in systemic sclerosis and lung fibrosis

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