A Clinical, Radiographic, and Physiologic Scoring System for the Longitudinal Assessment of Patients with Idiopathic Pulmonary Fibrosis^1–3

Abstract

In order to develop a reproducible, quantifiable means of assessment of the clinical status of patients with idiopathic pulmonary fibrosis (IPF), a composite clinical-radiographic-physiologic (CRP) scoring system was devised, using 7 variables: dyspnea, chest radiograph, spirometry, lung volume, diffusion capacity, resting alveolar-arterial PO2, and exercise O2 saturation. To assess this scoring system, we examined the relationships between CRP scores and histopathologic findings, including a cellular pathology score composed of abnormalities deemed to be potentially reversible, a fibrotic pathology score based on abnormalities felt to be essentially irreversible, and an index of overall pathologic derangement (total pathology score), derived from the sum of the cellular and fibrotic scores. The initial CRP determination at the time of open lung biopsy correlated significantly with the total pathology score (r = 0.61, p less than 0.001). The CRP score determined after 6 months of corticosteroid therapy showed a significant correlation with the fibrotic pathology score present on open lung biopsy (r = 0.76, p less than 0.001). The change in CRP after 6 months of corticosteroid therapy tended to reflect the cellular histopathologic component of the open lung biopsy (r = -0.43, p less than 0.10). Moreover, in none of these relationships did any individual component of the CRP score correlate better with the respective histopathologic index than did the CRP score itself. These data suggest that this CRP score is useful for the estimation of the severity of underlying pathologic derangement and for the longitudinal quantitative assessment of clinical impairment in patients with IPF.

High-Dose Acetylcysteine in Idiopathic Pulmonary Fibrosis

“…The duration of the disease was more than three months, and bibasilar inspiratory crackles were present. In addition, the following functional abnormalities were present: a dyspnea score of at least 2 on a scale of 0 (minimum) to 20 (maximum), 28 vital capacity of no more than 80 percent of the predicted value or total lung capacity less than 90 percent of the predicted value, and singlebreath carbon monoxide diffusing capacity (D l co ) less than 80 percent of the predicted value. 29,30 Patients were excluded if the standard regimen with prednisone and azathioprine was contraindicated or not justified for them or if they presented with a known intolerance to acetylcysteine.…”

“…In addition, the severity scores according to high-resolution CT were assessed, 23,24 and chest radiographic scores were estimated for the clinical, radiologic, and physiological (CRP) score. 28 …”

“…29,30 Post hoc, we evaluated changes in vital capacity of more than 10 percent or 0.2 liter and changes in D l co of more than 15 percent or 1 mmol per minute per kilopascal as categorical variables, because these were recently shown to be related to the risk of death. [32][33][34] The following prespecified secondary end points were assessed for changes between baseline and 12 months: vital capacity and D l co as percentages of the predicted value and D l co :alveolar volume as absolute change and percentage of the predicted value; CRP score 28 ; dyspnea score 28 35 ; scores of ground-glass opacities and of fibrosis on high-resolution CT 24 (ranging from 0 [minimum] to 5 [maximum]); health status according to the St. George's Respiratory Questionnaire (total scores range from 0 to 100, with higher scores indicating a worse quality of life) 36 ; number of adverse effects and withdrawals; and mortality during the study and up to one month after withdrawal.…”

Mapping of topoisomerase II α epitopes recognized by autoantibodies in idiopathic pulmonary fibrosis

“…Eleven of the 36 patients were on immunosuppressive treatment: three patients were on steroids only, three were on cyclosporin A, one was on cyclophosphamide, one was on steroids and cyclosporin A, and three patients were on a combined regimen of steroids and cyclophosphamide. Radiological and physiological scores, diffusing lung CO capacity in a single breath corrected for alveolar ventilation (DLCOsb/AV percentage predicted) were measured as described by Watters et al [22]. Arterial partial pressure of oxygen (PaO 2 ) was measured both resting and after exercise.…”

“…Arterial partial pressure of oxygen (PaO 2 ) was measured both resting and after exercise. Patient lung involvement was globally quantified utilizing a composite clinical, radiological and physiological scoring system (CRP score) proposed by the same authors [22]. Lung histology was evaluated according to reported criteria [22].…”