Abnormal Nailfold Capillaroscopy Is Common in Patients with Connective Tissue Disease and Associated with Abnormal Pulmonary Function Tests

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.

Dr. van Roon, et al reply

“…We thank Dr. Lambova for her interesting comment 1 on our recent article published in The Journal 2 . We reported that a systemic sclerosis (SSc) or scleroderma-like capillaroscopic pattern is common in patients with Raynaud phenomenon, and can be frequently observed in patients with connective tissue diseases (CTD) other than SSc.…”

Scleroderma-like Pattern in Various Rheumatic Diseases

“…The recently published paper by van Roon, et al addresses an interesting question about the presence of "scleroderma" pattern in different connective tissue diseases and its association with abnormal pulmonary function tests 1 . The authors have detected it in 88% of cases with systemic sclerosis (SSc; 35/40), in 17% of patients with systemic lupus erythematosus (SLE; 5/30) and in 13% of patients with rheumatoid arthritis (RA; 2/15).…”

“…It appears early in the disease course, facilitates early diagnosis, and is accepted as a diagnostic criterion. Scleroderma-like pattern could be observed in a smaller proportion of patients with other rheumatic diseases (i.e., SLE, RA, and others) [1][2][3][4][5][6][7][8][9] .…”

Might Nailfold Capillaroscopy Be a “Proxy” for Lung Involvement in Connective Tissue Diseases?

“…In this issue of The Journal, Van Roon, et al assessed the presence of an SSc pattern on nailfold capillaroscopy in consecutive patients with RP at the tertiary referral center, Department of Internal Medicine, University Medical Center Groningen, the Netherlands, and examined its association with pulmonary function tests 2 years or more after presentation 17 . There were 759 patients with RP classified as having primary RP (n = 245) when their serology was negative and when they had a "normal" nailfold capillaroscopy and no clinical symptoms of CTD during followup (2 years or more following presentation), or otherwise as secondary RP (n = 514).…”

“…Van Roon, et al had the ambitious aim of assessing capillaroscopy as "proxy" for lung involvement in CTD 17 . The results of their study show that there are signals that both SSc pattern and CTD antibodies are supportive aids in managing patients with CTD.…”