Anniek M van Roon1,2, Cato C Huisman3,4, Arie M van Roon1,2, Dan Zhang3,4, Alja J Stel3,4, Andries J Smit3,4, Hendrika Bootsma3,4, Douwe J Mulder3,4. 1. From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. a.m.van.roon01@umcg.nl. 2. A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen. a.m.van.roon01@umcg.nl. 3. From the Department of Internal Medicine, Division of Vascular Medicine, and the Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen; Department of Rheumatology, Medical Center Leeuwarden, Leeuwarden, the Netherlands. 4. A.M. van Roon, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; C.C. Huisman, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; A.M. van Roon, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; D. Zhang, MD, Department of Rheumatology, Medical Center Leeuwarden; A.J. Stel, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; A.J. Smit, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen; H. Bootsma, MD, PhD, Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen; D.J. Mulder, MD, PhD, Department of Internal Medicine, Division of Vascular Medicine, University of Groningen, University Medical Center Groningen.
Abstract
OBJECTIVE: To assess the presence of a systemic sclerosis (SSc) pattern on nailfold capillary microscopy (NCM) in patients with Raynaud phenomenon (RP) and to explore its association with abnormal pulmonary function tests (PFT). METHODS: NCM patterns were assessed in 759 consecutive patients with RP. Patterns were classified as normal (n = 354), nonspecific (n = 159), or SSc pattern (n = 246). Abnormal PFT was defined as forced vital or diffusion capacity < 70%. Patients were classified as primary RP (n = 245), or secondary: no definite diagnosis (n = 391), SSc (n = 40), primary Sjögren syndrome (pSS; n = 30), systemic lupus erythematosus (SLE; n = 30), mixed connective tissue disease (MCTD; n = 7), rheumatoid arthritis (RA; n = 15). RESULTS: An SSc pattern on NCM was frequently observed in most patients with a definite diagnosis: SSc (88%), pSS (33%), SLE (17%), MCTD (71%), and RA (13%). In patients without definite diagnosis, 17% had a normal NCM pattern, 35% nonspecific, and 48% SSc pattern. Abnormal PFT was more frequent in patients with an SSc pattern (35.9% vs 19.5%, p = 0.002), even when corrected for SSc diagnosis (p = 0.003). Absence of an SSc pattern had high negative predictive value (88%); positive predictive values were low. CONCLUSION: SSc pattern on NCM is common in patients with RP, and in those with connective tissue diseases other than SSc. It is associated with a higher prevalence of abnormal PFT, independent of the presence of an SSc diagnosis. Although these data need validation in a prospective setting, they underline the importance of NCM in RP and putative value to stratify the risk of pulmonary involvement in early stages of disease.
OBJECTIVE: To assess the presence of a systemic sclerosis (SSc) pattern on nailfold capillary microscopy (NCM) in patients with Raynaud phenomenon (RP) and to explore its association with abnormal pulmonary function tests (PFT). METHODS: NCM patterns were assessed in 759 consecutive patients with RP. Patterns were classified as normal (n = 354), nonspecific (n = 159), or SSc pattern (n = 246). Abnormal PFT was defined as forced vital or diffusion capacity < 70%. Patients were classified as primary RP (n = 245), or secondary: no definite diagnosis (n = 391), SSc (n = 40), primary Sjögren syndrome (pSS; n = 30), systemic lupus erythematosus (SLE; n = 30), mixed connective tissue disease (MCTD; n = 7), rheumatoid arthritis (RA; n = 15). RESULTS: An SSc pattern on NCM was frequently observed in most patients with a definite diagnosis: SSc (88%), pSS (33%), SLE (17%), MCTD (71%), and RA (13%). In patients without definite diagnosis, 17% had a normal NCM pattern, 35% nonspecific, and 48% SSc pattern. Abnormal PFT was more frequent in patients with an SSc pattern (35.9% vs 19.5%, p = 0.002), even when corrected for SSc diagnosis (p = 0.003). Absence of an SSc pattern had high negative predictive value (88%); positive predictive values were low. CONCLUSION: SSc pattern on NCM is common in patients with RP, and in those with connective tissue diseases other than SSc. It is associated with a higher prevalence of abnormal PFT, independent of the presence of an SSc diagnosis. Although these data need validation in a prospective setting, they underline the importance of NCM in RP and putative value to stratify the risk of pulmonary involvement in early stages of disease.
Authors: Panagiota Anyfanti; Elena Angeloudi; Athanasia Dara; Alexandra Arvanitaki; Eleni Bekiari; George D Kitas; Theodoros Dimitroulas Journal: Life (Basel) Date: 2022-07-31