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Puffy fingers improves predictiveness of very early systemic sclerosis diagnosis

Publish date: August 20, 2013

Tara Haelle

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Balancing liberal criteria can be difficult

It is appreciated among rheumatologists that although

Raynaud’s phenomenon is common in the general population, it occurs more

commonly in patients with connective tissue disorders and in particular in those

with scleroderma. Recently, preliminary criteria have been proposed by the European

League Against Rheumatism (EULAR) Scleroderma Trials and Research Group to

identify very early systemic sclerosis. Three red-flag features were identified

as those which would raise suspicion for early scleroderma: Raynaud’s phenomenon,

puffy fingers, and a positive ANA. In Dr. Minier’s study, puffy fingers emerged

as an important feature suggesting a higher likelihood of systemic sclerosis.

Dr. Robert Spiera

It has already been appreciated that patients with Raynaud’s phenomenon who have a positive ANA have a higher likelihood of developing a

connective tissue disorder. In this cohort study, the presence of puffy fingers

in the past or at the time of evaluation markedly increased a positive

predictive value that scleroderma would be present or develop. Perhaps even

more important was their observation that among patients with Raynaud’s phenomenon

without detectable ANA (in whom the likelihood of a connective tissue disorder

evolving was traditionally felt to be low), if puffy fingers were present,

as many as 20% ultimately developed features suggestive of systemic sclerosis.

Rheumatologists have typically recognized the presence

of puffy fingers as potentially an important clinical finding, indicating a

likelihood of the patient having an inflammatory arthritis, connective tissue

disorder, or scleroderma. Of course there is a differential diagnosis of

puffy hands, including thyroid disease, edema, or other metabolic

abnormalities. Nevertheless, in the context of Raynaud’s phenomenon and

detectable ANA, vigilance for evolution toward a scleroderma spectrum disorder

is warranted.

Ultimately, the balance of disease criteria can be

difficult. More liberal diagnostic criteria allow the recognition and diagnosis

of the disorder in patients earlier in the disease course. That must be

balanced against the downside of establishing a diagnosis of a potentially dangerous

and debilitating rheumatic disease in a patient in whom it might not more fully

evolve. Ultimately, the very diagnosis itself could be a major cause of anxiety

and, indeed, morbidity to those patients.

The finding, however, of the importance of puffy

fingers as a predictive factor in establishing a diagnosis of systemic

sclerosis is important, and would be particularly of value if recognized in the

general medical community in terms of identifying patients worthy of further

investigation or rheumatologic referral.

Dr. Robert Spiera is a professor of clinical medicine at

Weill Cornell Medical College and director of the Scleroderma, Vasculitis and Myositis Center at the Hospital for Special Surgery in New York.

FROM ANNALS OF THE RHEUMATIC DISEASES

The presence of puffy fingers in patients with Raynaud’s phenomenon who are antinuclear antibody positive was further validated as an important sign of possible early systemic sclerosis in a recent study.

The findings support the value of using the European League Against Rheumatism Scleroderma Trial and Research Group’s (EUSTAR’s) new criteria of ANA positivity, Raynaud’s phenomenon, and puffy fingers as three red flags that raise suspicion for very early systemic sclerosis, reported Dr. Tünde Minier of the University of Pécs in Hungary and her colleagues (Ann. Rheum. Dis. 2013 Aug. 12 [doi: 10.1136/annrheumdis-2013-203716]). They calculated that the positive predictive value of ANA positivity for developing systemic sclerosis in patients with Raynaud’s phenomenon increased from 33.9% to 88.5% when combined with the presence of puffy fingers.

Dr. Tünde Minier

The researchers examined 469 patients with Raynaud’s phenomenon who were enrolled in the multicenter, prospective, observational Very Early Diagnosis of Systemic Sclerosis (VEDOSS) study at 33 EUSTAR centers throughout and outside Europe. About a third of the patients were ANA negative (32.2%), and 67.8% were ANA positive. Among the ANA-positive participants, 53.6% had a systemic sclerosis pattern on nailfold capillaroscopy, compared with only 13.4% of the ANA-negative patients (P less than .001).

Just over half of the ANA-negative patients lacked a systemic sclerosis pattern on nailfold capillaroscopy, systemic sclerosis–related specific clinical symptoms, or an erythrocyte sedimentation rate of 25 mm/hr or greater, and so were diagnosed with primary Raynaud’s.

The examinations of the patients also included assessments for digital ulcers, digital pitting scars, telangiectases, calcinosis, tendon friction rubs, esophageal symptoms, and symptoms consistent with median nerve compression syndrome.

The most common clinical features in the ANA-positive patients were previous or current puffy fingers, found in 38.5% of the ANA-positive patients versus 23.3% of the ANA-negative patients, and esophageal symptoms, identified in 35.2% of the ANA-positive patients and 18.4% of the ANA-negative patients.

"Almost 90% of ANA-positive Raynaud’s phenomenon patients with previous or current finger edematous skin changes (puffy fingers) already had a nailfold capillaroscopy systemic sclerosis pattern and/or systemic sclerosis–specific autoantibodies," Dr. Minier’s team wrote. Specifically, 73.3% of ANA-positive patients with Raynaud’s and puffy fingers had a nailfold capillaroscopy systemic sclerosis pattern, compared with only 41.2% of ANA-positive patients without puffy fingers. Overall, 88.5% of ANA-positive patients with Raynaud’s and puffy fingers met the criteria for very early systemic sclerosis.

The ANA-positive patients with puffy fingers were also more likely to have other symptoms than were the ANA-positive patients without puffy fingers. Sclerodactyly was identified in 17.8% of the ANA-positive patients with puffy fingers, compared with 6.2% of the ANA-positive patients without current or previous puffy fingers (P = .002). Telangiectases also appeared on 17.3% of the ANA-positive patients with puffy fingers, compared with 9.2% of those without puffy fingers (P = .033). Similarly, 42.1% of ANA-positive patients with puffy fingers had esophageal symptoms, compared with 30.9% of those without puffy fingers (P = .043).

The researchers also noted that even puffy fingers in ANA-negative patients may require more careful follow-up, because 17% of the ANA-negative patients with current or previous puffy fingers had a nailfold capillaroscopy systemic sclerosis pattern and 20% of the ANA-negative patients with puffy fingers had sclerodactyly with other systemic sclerosis symptoms.

The authors noted that one limitation of their study is the inability to generalize their findings to the broader population of Raynaud’s phenomenon patients seen by general physicians, since a higher percentage of the patients enrolled in this cohort meet very early systemic sclerosis classification.

"Patients identified with the very early diagnostic criteria may also have scleroderma with limited cutaneous involvement or even undifferentiated connective tissue disease," a limitation that longer-term follow-up data may clarify with a comparison of the ACR 1980 classification criteria and the new ACR-EULAR criteria.

The study did not use external funding. The authors had no disclosures.

* This story was updated 8/20/2013.

Puffy fingers improves predictiveness of very early systemic sclerosis diagnosis

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