Performance of the St George’s Respiratory Questionnaire in patients with connective tissue disease‐associated interstitial lung disease

Suzuki, Atsushi; Kondoh, Yasuhiro; Swigris, Jeffrey J.; Ando, Masahiko; Kimura, Tomoki; Kataoka, Kensuke; Yamano, Yasuhiko; Furukawa, Taiki; Numata, Mari; Sakamoto, Koji; Hasegawa, Yoshinori

doi:10.1111/resp.13293

Cited by 17 publications

(9 citation statements)

References 44 publications

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“…This 50-item patient-reported questionnaire was specifically developed for respiratory diseases, [22] is frequently used in patients with ILD, and has recently been validated in connective tissue disease-associated ILD. [23,24] The SGRQ includes three domains relating to symptoms, activity, and impact, as well as a total score that ranges from 0-100 with a higher score indicating worse quality of life. Non-elective respiratory-related hospitalisations, non-respiratoryrelated hospitalisations, and cumulative number of days admitted to hospital within 6 months of each frailty assessment were identified from the medical record.…”

Section: Outcome Assessmentsmentioning

confidence: 99%

Functional ageing in fibrotic interstitial lung disease: the impact of frailty on adverse health outcomes

et al. 2019

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BackgroundAccelerated biological and functional ageing is common in fibrotic interstitial lung disease (ILD); however, their impact on adverse health outcomes has not been evaluated in this population.MethodsPatients were prospectively recruited from a specialised ILD clinic. Functional ageing was determined by frailty index and biological age by measurement of absolute telomere length (aTL) from patients' peripheral blood leukocytes. Adverse health outcomes included health-related quality of life (St George's Respiratory Questionnaire), number and length of respiratory and non-respiratory hospitalisations, medication tolerability and time to death or lung transplantation. Multivariable models were used to determine the risks and rates of adverse health outcomes associated with the frailty index and aTL.Results540 patients with fibrotic ILD, including 100 with idiopathic pulmonary fibrosis (IPF), provided 749 frailty index assessments, with 189 patients providing blood samples. The frailty index was strongly associated with quality of life, rate of hospitalisation, time to hospital discharge and mortality, including adjustment for age, sex, disease severity and IPF diagnosis. Mortality prognostication was improved by the addition of the frailty index to commonly used clinical parameters and previously validated composite indices. Conversely, aTL was not associated with most adverse health outcomes. The effect of chronological age on outcomes was mediated primarily by the frailty index, and to a lesser extent by aTL.ConclusionsFunctional ageing is associated with adverse health outcomes in patients with fibrotic ILD, indicating the need for consideration of the individual functional age into clinical decision-making.

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Section: Outcome Assessmentsmentioning

confidence: 99%

Functional ageing in fibrotic interstitial lung disease: the impact of frailty on adverse health outcomes

et al. 2019

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“…We used the 6MWD to assess the patient activity tolerance 17 and St. George's Respiratory Questionnaire (SGRQ) to evaluate quality of life. 18 Pulmonary function tests (PFTs) and HRCT were performed at our hospital at three-six-month intervals, according to the changes of symptoms. The HRCT scores of the patients were calculated based on the Ichikado criterion by the same two radiologists.…”

Section: Methodsmentioning

confidence: 99%

Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients

2019

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Objectives: This study reports a low dose combination therapy of cyclophosphamide (CYC) and pirfenidone (PFD) and the efficiency and safety of the therapy in refractory connective tissue disease associated interstitial lung disease (CTD-ILD) patients. Patients and methods: The study included seven CTD-ILD patients (2 males, 5 females; mean age 48.8 years; range, 32 to 63 years) treated between January 2016 and December 2017 in our clinic. At enrolment, all patients had shown no improvement in their symptoms (dyspnea or cough) after at least one month of high dose steroids treatment. Patients who had received adjusted immunosuppressive agents other than steroids or anti-fibrotic medications within the three months before enrolment were excluded. We changed the treatment to a low dose combination of CYC 0.4 g/m 2 monthly and PFD 300 mg twice per day and quickly reduced the steroids. All the patients were followed-up for 12 months. Results: Two patients had anti-synthetase syndrome, two had Sjögren syndrome, two had scleroderma and one had mixed connective tissue disease. The baseline forced vital capacity (FVC) was 39-81% and the six-minute walk distance (6MWD) was 202 m-324 m. Within 12 months followup, the median improvement in the FVC was 13.4% (range, 0-35.9%), the median improvement of carbon monoxide diffusing capacity was 6.3% (range, 1.7-16%) and the median improvement of 6MWD was 52.7% (range, 34.4-86.3%). All the patients were self-sufficient, and their dyspnea, chest high-resolution computed tomography scores, and quality of life improved simultaneously. Exceeding our expectations, no adverse events associated with CYC or PFD were observed during the follow-up period. Conclusion: Our study provided preliminary while promising clinical evidence for combination therapy of CYC-PFD for CTD-ILD. A low dose combination of CYC and PFD was unexpectedly well tolerated, with satisfactory effects in refractory CTD-ILD patients. Well-designed controlled studies are needed to further establish the safety and efficacy of this approach.

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“…Clinically significant improvement/deterioration in lung physiology (composite categorical change) was defined based on previous reports: ≥10% relative increase/decline in forced vital capacity (FVC) or ≥5% to <10% relative increase/decline in FVC and ≥15% relative increase/decline in DL CO . The minimal clinically important difference (MCID) was utilized as the threshold: 28 m improvement/deterioration in 6‐min walk distance (6MWD), ≥1 point increase/decrease in MMRC and ≥7 point decrease/increase in total SGRG score . In 6MWD and MMRC, the significant change for idiopathic pulmonary fibrosis (IPF) patients was utilized because of lack of evidence for CTD‐ILD.…”

Section: Methodsmentioning

confidence: 99%

“…25 The minimal clinically important difference (MCID) was utilized as the threshold: 28 m improvement/deterioration in 6-min walk distance (6MWD), ≥1 point increase/decrease in MMRC and ≥7 point decrease/increase in total SGRG score. [26][27][28] In 6MWD and MMRC, the significant change for idiopathic pulmonary fibrosis (IPF) patients was utilized because of lack of evidence for CTD-ILD.…”

Section: Definition Of Significant Changementioning

confidence: 99%

Multidimensional improvement in connective tissue disease‐associated interstitial lung disease: Two courses of pulse dose methylprednisolone followed by low‐dose prednisone and tacrolimus

et al. 2018

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Performance of the St George’s Respiratory Questionnaire in patients with connective tissue disease‐associated interstitial lung disease

Abstract: These data support the validity and reliability of SGRQ as a sensitive measure for capturing HRQoL in patients with CTD-ILD.

Cited by 17 publications

References 44 publications

Functional ageing in fibrotic interstitial lung disease: the impact of frailty on adverse health outcomes

Functional ageing in fibrotic interstitial lung disease: the impact of frailty on adverse health outcomes

Efficacy of Combination Therapy With Pirfenidone and Low-Dose Cyclophosphamide for Refractory Interstitial Lung Disease Associated With Connective Tissue Disease: A Case-Series of Seven Patients

Multidimensional improvement in connective tissue disease‐associated interstitial lung disease: Two courses of pulse dose methylprednisolone followed by low‐dose prednisone and tacrolimus

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