Evaluation and management of scleroderma lung disease using bronchoalveolar lavage

Silver, Richard M.; Miller, K. Scott; Kinsella, Margaret B.; Smith, Edwin A.; Schabel, Stephen I.

doi:10.1016/0002-9343(90)90425-d

Cited by 221 publications

(136 citation statements)

References 24 publications

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“…5,6 SSc infl ammation, represented by ground-glass opacities (GGOs) and sampled by BAL, 7-10 could provide a mechanism for nerve sensitization and has been used to predict progression to fi brosis. [9][10][11][12] However, more evidence has indicated that fi brosis originates, progresses, and responds to CYC treatment independent of the appearance of GGOs or their resolution, 13,14 and the fi ndings of early studies correlating BAL to disease progression and response to CYC have not been confi rmed. 15,16 Rather, CYC appears to directly affect the mechanical process of fi brosis independent of infl ammation, 1,2,[15][16][17] suggesting that fi brosis alone 14 can cause cough.…”

Section: Discussionmentioning

confidence: 99%

Correlation of Cough With Disease Activity and Treatment With Cyclophosphamide in Scleroderma Interstitial Lung Disease

Theodore

Tseng

et al. 2012

Chest

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Nonproductive cough is a characteristic symptom of interstitial lung diseases (ILDs), including scleroderma (SSc). In the Scleroderma Lung Study (SLS), a double-blind, randomized, placebo (PLA)-controlled trial of oral cyclophosphamide (CYC) administered for 1 year followed by an additional year of follow-up without treatment, 1 73% of the 158 study participants complained of cough at the time of enrollment, making it the second-most-common nonRaynaud symptom, exceeded only by dyspnea. After 12 to 18 months of therapy initiation, patients who received CYC showed signifi cant improvement in lung function, skin scores, dyspnea, disability, and various aspects of health-related quality of life, compared with those who received PLA. The benefi cial effects on all parameters except dyspnea were lost at 24 months (1 year after discontinuation of CYC). 2 The pathogenesis of cough in SSc and other ILDs is poorly understood. Cough is mediated through

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Section: Discussionmentioning

confidence: 99%

Correlation of Cough With Disease Activity and Treatment With Cyclophosphamide in Scleroderma Interstitial Lung Disease

Theodore

Tseng

et al. 2012

Chest

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“…Increased numbers of lymphocytes and mast cells may also be seen [33]. Early studies correlated increased granulocytes in BAL with increased response to immunosuppression presumably because this represented active alveolitis [34,35]. Subsequently, BAL granulocytosis has been shown to correlate with the degree of ground glass opacity seen on HRCT [30] and with more advanced interstitial disease [36].…”

Section: Bronchoalveolar Lavage (Bal)mentioning

confidence: 99%

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

Schol

Carr²,

Frech³

et al. 2012

Rheumatology

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“…Considerable heterogeneity exists in the severity and progression of lung disease. However, a subset of patients with SSc ILD follows a course of progressive decline (4)(5)(6). In these patients, inflammatory changes in the lung occur early in the course (7)(8)(9) and progress over time (10,11).…”

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confidence: 99%

“…Bronchoalveolar lavage (BAL) has been used in SSc to define a population of patients whose lung function will likely decline without therapy (4,6,10,14). The best correlates of subsequent decline in forced vital capacity (FVC) have included the percentage of polymorphonuclear leukocytes (PMNs) and eosinophils on a standardized BAL from either the right middle lobe (RML) or lingula (4,14,24).…”

mentioning

confidence: 99%

“…The best correlates of subsequent decline in forced vital capacity (FVC) have included the percentage of polymorphonuclear leukocytes (PMNs) and eosinophils on a standardized BAL from either the right middle lobe (RML) or lingula (4,14,24). However, these cellular components of BAL have also been seen in other ILDs, such as idiopathic NSIP, idiopathic pulmonary fibrosis (25), and cryptogenic organizing pneumonia (26).…”

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confidence: 99%

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Bronchoalveolar Lavage and Response to Cyclophosphamide in Scleroderma Interstitial Lung Disease

Strange¹,

Bolster²,

Theodore³

et al. 2008

Am J Respir Crit Care Med

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135

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Rationale: The presence of inflammatory cells on bronchoalveolar lavage is often used to predict disease activity and the need for therapy in systemic sclerosis-associated interstitial lung disease. Objectives: To evaluate whether lavage cellularity identifies distinct subsets of disease and/or predicts cyclophosphamide responsiveness. Methods: Patients underwent baseline lavage and/or high-resolution computed tomography as part of a randomized placebo-controlled trial of cyclophosphamide versus placebo (Scleroderma Lung Study) to determine the effect of therapy on forced vital capacity. Patients with 3% or greater polymorphonuclear and/or 2% or greater eosinophilic leukocytes on lavage and/or ground-glass opacification on computed tomography were eligible for enrollment. Measurements and Main Results: Lavage was performed in 201 individuals, including 141 of the 158 randomized patients. Abnormal cellularity was present in 101 of these cases (71.6%) and defined a population with a higher percentage of men (P 5 0.04), more severe lung function, including a worse forced vital capacity (P 5 0.003), worse total lung capacity (P 5 0.005) and diffusing capacity of the lung for carbon monoxide (P 5 0.004), more extensive ground-glass opacity (P 5 0.005), and more extensive fibrosis in the right middle lobe (P 5 0.005). Despite these relationships, the presence or absence of an abnormal cell differential was not an independent predictor of disease progression or response to cyclophosphamide at 1 year (P 5 not significant). Conclusions: The presence of an abnormal lavage in the Scleroderma Lung Study defined patients with more advanced interstitial lung disease but added no additional value to physiologic and computed tomography findings as a predictor of progression or treatment response. Clinical trial registered with www.clinicaltrials.gov (NCT 000004563).

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Evaluation and management of scleroderma lung disease using bronchoalveolar lavage

Cited by 221 publications

References 24 publications

Correlation of Cough With Disease Activity and Treatment With Cyclophosphamide in Scleroderma Interstitial Lung Disease

Correlation of Cough With Disease Activity and Treatment With Cyclophosphamide in Scleroderma Interstitial Lung Disease

Interstitial Lung Disease in Systemic Sclerosis: Diagnosis and Management

Bronchoalveolar Lavage and Response to Cyclophosphamide in Scleroderma Interstitial Lung Disease

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