High-Resolution CT Scan Findings in Patients With Symptomatic Scleroderma-Related Interstitial Lung Disease

Goldin, Jonathan G.; Lynch, David A.; Strollo, Diane C.; Suh, Robert D.; Schraufnagel, Dean E.; Clements, Philip J.; Elashoff, Robert M.; Fürst, Daniel E.; Vasunilashorn, Sarinnapha; McNitt-Gray, Michael F.; Brown, Marianne; Roth, Michael D.; Tashkin, Donald P.

doi:10.1378/chest.07-2444

Cited by 210 publications

(194 citation statements)

References 35 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%

“…13,14 All randomized subjects underwent HRCT scanning at baseline; repeat HRCT imaging was performed in 102 subjects after completion of 1 year of therapy. Baseline scans were determined to be acceptable for clinical interpretation in 156 of 158 cases (99%), and 98 paired follow-up scans were considered acceptable for assessment.…”

Section: Hrct Scanningmentioning

confidence: 99%

“…Using a scoring system described previously, scan interpretation was performed at the radiology core with the readers blinded to the clinical information. 13,14 …”

Section: Hrct Scanningmentioning

confidence: 99%

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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%

Section: Hrct Scanningmentioning

confidence: 99%

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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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“…The classic UIP pattern with bibasilar reticulation, traction bronchiectasis and honeycombing is also observed in patients with scleroderma but less commonly than NSIP. Honeycombing is seen more frequently in patients with limited SSc than in those with diffuse SSc [30]. A HRCT is required to make these radiographic distinctions.…”

Section: High Resolution Ctmentioning

confidence: 99%

“…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]. However, data from the Scleroderma Lung Study suggest that BAL granulocytosis does not add any additional prognostic information to HRCT and pulmonary function measures and is not a predictor of treatment response [36,37].…”

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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Cyclophosphamide for connective tissue disease-associated interstitial lung disease

Barnes

Holland

Westall

et al. 2018

Cochrane Database of Systematic Reviews

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This review, which is based on studies of varying methodological quality, demonstrates that overall, in this population, small benefit may be derived from the use of cyclophosphamide in terms of mean difference in % FVC when compared with placebo, but not of the difference in % DLCO, or when compared with mycophenolate. Modest clinical improvement in dyspnoea may be noted with the use of cyclophosphamide. Clinical practice guidelines should advise clinicians to consider individual patient characteristics and to expect only modest benefit at best in preserving FVC. Clinicians should carefully monitor for adverse effects during treatment and in the years thereafter.Further studies are required to examine the use of cyclophosphamide; they should be adequately powered to compare outcomes within different subgroups, specifically, stratified for extent of pulmonary infiltrates on high-resolution computed tomography (HRCT) and skin involvement in SSc. Studies on other forms of connective tissue disease are needed. Researchers may consider comparing cyclophosphamide (a potent immunosuppressant) versus antifibrotic agents, or comparing both versus placebo, in particular, for those with evidence of rapidly progressive fibrotic disease, who may benefit the most.

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High-Resolution CT Scan Findings in Patients With Symptomatic Scleroderma-Related Interstitial Lung Disease

Cited by 210 publications

References 35 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

Cyclophosphamide for connective tissue disease-associated interstitial lung disease

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