New provisional criteria for the diagnosis of very early systemic sclerosis aim to close the “unacceptably wide” gap between the onset of early signs of the autoimmune connective-tissue disease and the time of disease diagnosis, according to Dr. Marco Matucci-Cerinic.
The current diagnostic standard is based on the signs and symptoms of overt disease “and does not adequately address the earliest disease predictors,” said Dr. Matucci-Cerinic, professor of rheumatology and medicine at the University of Florence (Italy). In addition to helping rheumatologists in practice, the proposed criteria could also be valuable to the EULAR/ACR systemic sclerosis reclassification project, they stated (Ann. Rheum. Dis. 2009;68:1377–80).
“Systemic sclerosis has the highest case-specific mortality of any of the connective tissue diseases, which is likely due to the fact that the disease is often well established by the time it is diagnosed,” Dr. Matucci-Cerinic said at the annual European Congress of Rheumatology in Copenhagen in June.
In an effort to bridge the gap, Dr. Matucci-Cerinic and colleagues in the VEDOSS (Very Early Diagnosis of Systemic Sclerosis) project of the EULAR Scleroderma Trials and Research (EUSTAR) group have developed evidence-based criteria for the diagnosis of very early disease.
The proposed definition requires three major criteria (including Raynaud's phenomenon, disease-specific antibodies, and pathognomonic microvascular alterations detected by nail fold videocapillaroscopy), or two major criteria and one additional criterion (including calcinosis, puffy fingers, digital ulcers, dysfunction of the esophageal sphincter, telangiectasia, and ground glass appearance on high-resolution chest CT), Dr. Matucci-Cerinic said.
Based on the criteria, patients with Raynaud's phenomena and hand edema (“puffy fingers”), which commonly present together in early disease, should be referred for capillaroscopy as well as serology to detect antinuclear, anticentromere, antitopoisomerase I, and extractable nuclear antigen antibodies.
An important component of the VEDOSS project is the European Union–wide call for primary care clinicians to refer all patients who exhibit two or more of the early symptoms to a rheumatologist or scleroderma center, he said.
To date, several drugs—including ACE inhibitors, calcium channel blockers, cyclophosphamide, methotrexate, and endothelin receptor antagonists—have improved the outcomes of patients with established disease, according to EULAR/EUSTAR systemic sclerosis treatment recommendations issued earlier this year (Ann. Rheum. Dis. 2009;68:620–8). The possibility that earlier, more aggressive treatment with these or other agents that are being evaluated in clinical trials might alter the progression of disease and potentially prevent irreversible organ damage warrants investigation, said Dr. Alan Tyndall of the University of Basel (Switzerland).