Relationship Between High-Resolution Computer Tomography and FVC% Predicted for Classification of Pulmonary Hypertension in Systemic Sclerosis

Sara Jaafar¹, Paul Cronin ², Dharshan Vummidi ¹, Scott Visovatti ¹, Amber Young ³, Suiyuan Huang ¹, Vallerie McLaughlin ⁴ and Dinesh Khanna ⁵, ¹University of Michigan, Ann Arbor, MI, ²Unviersity of Michigan, Ann Arbor, MI, ³University of Michigan, Ann Arbot, ⁴Univeristy of Michigan, Ann Arbor, MI, ⁵Department of Medicine, University of Michigan, Ann Arbor, Michigan, USA, Ann Arbor

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: FVC and HRCT, Pulmonary hypertension, Scleroderma

Session Information

Date: Sunday, November 10, 2019

Title: Systemic Sclerosis & Related Disorders – Clinical Poster I

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in scleroderma-spectrum disorders (SSc). FVC% has been used to differentiate Group 1 (PAH) vs. Group 3 (PH due to chronic lung diseases) in different trials and cohorts. The objective of the current analysis was to assess the relationship between the degree of lung disease on high-resolution computed tomography (HRCT, gold standard) vs. FVC% in identifying those with PH due to Group 1 vs. Group 3 PH.

Methods: In this single center retrospective analysis, 268 patients with SSc who had right heart catheterizations (RHCs) at a tertiary center in US were included. HRCTs were reviewed by 2 thoracic radiologists who assessed the degree of total lung involvement in increments of 10% to up to 30% or > 30% lung involvement, and if there was concomitant emphysema. If emphysema was present, it was classified as mild, moderate, or severe. Chronic lung disease was defined as HRCT showing > 20% total lung involvement due to ILD; or if the total lung involvement due to ILD was 10-20% but the patient had concomitant moderate-to-severe emphysema. Each HRCT was categorized based on FVC% performed closest to the RHC into < 70% or ≥ 70%. Sensitivities and specificities were calculated .

Results: Of 268 RHCs, 57 had Group 1 and 36 had Group 3 PH based on the updated hemodynamic definition of pulmonary hypertension¹, as proposed by the 6th World Symposium on Pulmonary Hypertension². In 75 of 93 patients with Group 1 or 3 PH, we had available HRCT data and 54 were reviewed by thoracic radiologists. Of 75 HRCTs, 34 (45%) patients had moderate-to-severe lung disease (based on the definition above), 20 had mild disease, and 21 did not have any ILD and/or significant emphysema. When we included all HRCTs, FVC% had a sensitivity of 74% (95% CI (59%, 88%)) and specificity of 37% ((95% CI (22%, 51%)) Table 1). When we excluded those with normal HRCTs, FVC% had a sensitivity of 74% (95% CI (59%, 88%)) and specificity of 50% (95% CI (28%, 72%)) when compared to FVC% (Table 2).

Conclusion: FVC% misclassifies a large number of patients into Group 1 vs. Group 3 PH as it may be influenced by other SSc-related disease processes. Future studies should incorporate the degree of HRCT involvement to differentiate between the 2 groups.

Table 1 ACR

Table 1: Relationship between HRCTs and FVC% in the Whole Cohort

Table 2 ACR

Table 2: Relationship between HRCTs and FVC% in those with ILD Involvement on HRCT

Disclosure: S. Jaafar, None; P. Cronin, None; D. Vummidi, None; S. Visovatti, None; A. Young, None; S. Huang, None; V. McLaughlin, Acceleron, 2, 5, Actelion, 2, 5, Arena, 2, 5, Bayer, 2, 5, Caremark, 5, United Therapeutics, 5, Gilead, 2, Sonovie, 2; D. Khanna, Acceleron, 5, 8, Acceleron Pharma, 5, Actelion, 5, 8, Actelion Pharmaceuticals, 5, Astra Zeneca, 5, Bayer, 2, 5, 8, Behring, 5, Blade, 5, Blade Therapeutics, 5, 8, Blade therapeutics, 5, BMS, 2, 5, 8, Boehringer Ingelham, 5, Boehringer Ingelheim, 5, 8, Boehringer-Ingelheim, 5, Bristol Myers Squibb, 2, 5, Bristol-Myers Squibb, 2, 5, Celegene, 5, Celgene, 5, 8, ChemomAB, 5, ChemomAb, 5, CiviBioPharma, Inc., 3, Corbus, 5, Corobus, 5, Corpus, 5, CSL Behring, 5, 8, Curizon, 5, Curzion, 5, Cytori, 5, 8, Eicos, 4, Eicos Sciences, 4, Eicos Sciences, Inc, 4, Eicos Sciences, Inc/ CiviBioPharma, Inc, 1, 4, Eicos Sciences, Inc/ CiviBioPharma, Inc., 1, Eicos, Inc, 4, Eicos, Inc., 5, 8, Eicos, INC., 4, Galapagos, 5, Genentech, 5, Genentech/Roche, 5, GlaxoSmithKline, 5, GSK, 5, Horizon, 2, 5, Mitsubishi Tanabe Pharma, 5, Mitsubishi Tanabe Pharma Dev America, 5, Mitsubishi Tanabe Pharma Development America, 5, Mitsubishi Tanabi, 5, NIH K24 and R01, 2, NIH / NIAMS K24 AR-063120, 2, NIH/NIAMS R01& K24, 2, Pfizer, 2, 5, Sanofi, 5, Sanofi Aventis, 5, Sanofi-Aventis, 5, 8, Sanofi-Aventis/Genzyme, 5, UCB, 5, UCB Pharma, 5.

To cite this abstract in AMA style:

Jaafar S, Cronin P, Vummidi D, Visovatti S, Young A, Huang S, McLaughlin V, Khanna D. Relationship Between High-Resolution Computer Tomography and FVC% Predicted for Classification of Pulmonary Hypertension in Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/relationship-between-high-resolution-computer-tomography-and-fvc-predicted-for-classification-of-pulmonary-hypertension-in-systemic-sclerosis/. Accessed .

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