Nail Fold Capillary Changes Are Associated with Pulmonary, but Not with Cardiac Involvement in Juvenile Dermatomyositis

Zoltán Barth^1,2,3, Thomas Schwartz^2,4, Berit Flatø^5,6, Akos Koller^7,8, May Brit Samersaw-Lund⁹, Ivar Sjaastad^10,11 and Helga Sanner⁶, ¹Bjorknes College, Oslo, Norway, ²Oslo University Hospital and University of Oslo, Institute for Experimental Medical Research, Oslo, Norway, ³Medical School, University of Pecs, Institute for Translational Medicine, Pecs, Hungary, ⁴Institute for Experimental Medical Research, Institute for Clinical Medicine, University of Oslo, Oslo, Norway, ⁵Institute for Clinical Medicine, University of Oslo, Oslo, Norway, Oslo, Norway, ⁶Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, Oslo, Norway, ⁷University of Physical Education, Institute of Natural Sciences, Budapest, Hungary, ⁸Medical School, University of Pecs, Department of Neurosurgery, Pecs, Hungary, ⁹Oslo University Hospital, Rikshospitalet, Department of Respiratory Medicine, Oslo, Norway, ¹⁰Department of Cardiology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, ¹¹Institute for Experimental Medical Research, Oslo University Hospital and University of Oslo, Oslo, Norway, Oslo, Norway

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Capillaroscopy, juvenile dermatomyositis and pediatric rheumatology, Pulmonary Involvement

Session Information

Date: Monday, November 6, 2017

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster II: Lupus and Related Disorders, Myositis, Scleroderma and Vasculitis

Session Type: ACR Poster Session B

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

Background/Purpose: Juvenile dermatomyositis (JDM) is a rare autoimmune disease of unknown origin, characterised primarily by cutaneous rashes and symmetrical weakness of the proximal skeletal muscles. Other organs can also be affected with characteristic changes in the involved tissues, in which local inflammation and impaired microcirculation probably have pathogenic role. Microvascular abnormalities are easily assessable in the nail folds by capillaroscopic examination (Nail Fold Capillaroscopy, NFC), and might mirror microvascular involvement in other organs. We aimed to examine the association between alterations in nailfold capillary network and pulmonary and cardiac involvement in JDM patients with medium- to long-term disease duration.

Methods: Fifty-nine JDM patients were examined after disease onset median 16.8 (2-38) years. Nailfold capillary density (NCD) was assessed by NFC and patients were divided into ‘Low NCD’ (<6.0 cap/mm) and ‘Normal NCD’ (≥6.0 cap/mm) groups. Pulmonary involvement was assessed by pulmonary function test (PFT; forced vital capacity, FVC; total lung capacity, TLC; diffusing capacity for carbon monoxide, DLCO) and high resolution CT imaging (interstitial lung disease, ILD; airway disease). PFT parameters are presented as predictive values. Additionally, the patients were also grouped by PFT values: a ‘low’ value was defined as less than the 5^th percentile of the predicted value. Cardiac measures included echocardiography and conventional, as well as Holter ECG measures. Systolic cardiac function was assessed by long axis strain (LAS), diastolic function was measured by early diastolic tissue velocity (e’). Arrhythmias and heart rate variability were assessed by ECG.

Results: The low NCD group presented lower FVC and TLC without difference in FVC/DLCO ratio. Moreover, patients with low NCD presented more frequently low DLCO and abnormalities in PFT/HRCT than those patients with normal NCD. There were no significant association between NCD and cardiac measures.

Conclusion: Nail fold capillary alterations in JDM patients after median 16.8 years disease duration was associated with pulmonary involvement, and might be a useful tool to detect patients at risk for pulmonary complications. Further studies are needed to confirm the applicability of NCD as a biomarker for lung involvement in JDM after medium to long term disease duration.

Disclosure: Z. Barth, None; T. Schwartz, None; B. Flatø, None; A. Koller, None; M. B. Samersaw-Lund, None; I. Sjaastad, None; H. Sanner, None.

To cite this abstract in AMA style:

Barth Z, Schwartz T, Flatø B, Koller A, Samersaw-Lund MB, Sjaastad I, Sanner H. Nail Fold Capillary Changes Are Associated with Pulmonary, but Not with Cardiac Involvement in Juvenile Dermatomyositis [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/nail-fold-capillary-changes-are-associated-with-pulmonary-but-not-with-cardiac-involvement-in-juvenile-dermatomyositis/. Accessed .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/nail-fold-capillary-changes-are-associated-with-pulmonary-but-not-with-cardiac-involvement-in-juvenile-dermatomyositis/