Title:Nailfold Capillaroscopy – Its Role in Diagnosis and Differential Diagnosis of Microvascular Damage in Systemic Sclerosis
Volume: 9
Issue: 4
Author(s): Sevdalina Lambova, W. Hermann and Ulf Muller-Ladner
Affiliation:
Keywords:
Capillaroscopy, systemic sclerosis.
Abstract: In the nailfold area, specific diagnostic microvascular abnormalities are easily recognized via capillaroscopic
examination in systemic sclerosis (SSc). They are termed “scleroderma” type capillaroscopic pattern, which includes
presence of dilated, giant capillaries, haemorrhages, avascular areas, and neoangiogenic capillaries and are observed in the
majority of SSc patients (in more than 90%). LeRoy and Medsger (2001) proposed criteria for early diagnosis of SSc with
inclusion of the abnormal capillaroscopic changes and suggested to prediagnose SSc prior to the development of other
manifestations of the disease. It is a new era in the diagnosis of SSc. At present, an international multicenter project is performed.
It aims validation of criteria for very early diagnosis of SSc (project VEDOSS (Very Early Diagnosis of Systemic
Sclerosis) and is organized by European League Against Rheumatism (EULAR) Scleroderma Trials and Reasearch. Very
recently the first results of the VEDOSS project were processed and new EULAR/ACR (American College of Rheumatology)
classification criteria have been validated and published (2013), in which the characteristic capillaroscopic
changes have been included.
Our observations confirm the high frequency of the specific capillaroscopic changes of the fingers in SSc, which have
been found in 97.2% of the cases from the studied patient population. We have performed for the first time capillaroscopic
examinations of the toes in SSc. Interestingly,“scleroderma type” capillaroscopic pattern was also found at the toes in a
high proportion of patients - 66.7%, but it is significantly less frequent as compared with fingers (97.2%, p<0.05). In our
opinion, the examination of the toes of SSc patients should be considered as it suggests an additional opportunity for evaluation
of the microvascular changes in these patients although the observed changes are in a lower proportion of cases.
Thus, capillaroscopic examination is a cornerstone for the very early diagnosis of SSc. Patients with clinical symptoms of
peripheral vasospasm (Raynaud’s phenomenon (RP)) in association with puffy fingers and/or sclerodactyly should be
carefully examined. Hence, appearance of “scleroderma” type capillaroscopic changes in RP patients should be interpreted
in the clinical context, because some of the components of this pattern may be observed in several other connective
tissue diseases such as mixed connective tissue disease, undifferentiated connective tissue disease that are termed
“scleroderma-like” capillaroscopic changes. Capillaroscopic examination is an obligatory screening method in these cases,
but the pathologic capillaroscopic changes are not specific and their interpretation is in clinical context.
