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From Raynaud's Phenomenon to Very Early Diagnosis of Systemic Sclerosis- The VEDOSS approach
From Raynaud's Phenomenon to Very Early Diagnosis of Systemic Sclerosis- The VEDOSS approach
La riabilitazione multidisciplinare del malato reumatico
panoramica sulla sclerosi sistemic
The race against time to disclose lung inflammation in interstitial lung disease in systemic sclerosis: is PET scan the winning solution?
The race against time to disclose lung inflammation in interstitial lung disease in systemic sclerosis: is PET scan the winning solution
Mixed Connective Tissue Disease, a Roundabout to Rheumatic Diseases?
revisione circa la reale natura come entità separata della connettivite mist
The Measurement of the Endothelial Glycocalyx as a New Biomarker of Endothelial Derangement in Systemic Sclerosis: A Challenge for the Future
The role of the Endothelial Glycocalyx as a New Biomarker of Endothelial Derangement in Systemic Sclerosi
Very Early Systemic Sclerosis and Pre-systemic Sclerosis: Definition, Recognition, Clinical Relevance and Future Directions
PURPOSE OF REVIEW:
The approach to systemic sclerosis (SSc) has changed over the years with an increasing focus on the very early diagnosis of the disease. The terminology identifying patients in the early phase of SSc has been significantly confusing in the last three decades. The purpose of this article is to analyze how the concept of "very early SSc" has evolved over the years, which is the role of an early diagnosis and how early treat patients.
RECENT FINDINGS:
Several attempts have been made over time, to create more sensitive and specific classification criteria to include the largest number of SSc patients, also in the earliest phase. An algorythm for the very early diagnosis of SSc was identified, diagnostic preliminary criteria proposed, and new 2013 ACR/EULAR SSc classification criteria published, including new items and adding emphasis to the vasculopathic manifestations. True biomarkers that could predict the disease evolution are still missing. Treat or not to treat patients in the earliest phases still remain a dilemma. For the moment, the only feasible clinical strategy in very early SSc remains a tight follow up program to detect in "real time" the early internal organ involvement which may allow an aggressive therapeutic agenda
Very early and early diagnosis of systemic sclerosis
Systemic sclerosis (SSc) is easily diagnosed when the disease is evolved to skin fibrosis with obliterative vasculopathy and internal organs involvement. In the very early/early phase of SSc, the diagnosis remains very difficult because of the lack of validated diagnostic criteria. Actually, the American College of Rheumatology (ACR) and LeRoy classification criteria are not sufficiently sensitive to enable very early/early diagnosis of the disease, limiting the possibility of a precocious treatment. Therefore, in many cases, treatment is delayed for several years following the onset of Raynaud‘s phenomenon (RP) and even after the onset of the first non-RP symptom. For this reason, the very early/early diagnosis of SSc is today of pivotal importance. Recently, RP, puffy fingers turning into sclerodactyly and antinuclear antibody (ANA) positivity are considered the three red flags for the suspicion of a very early SSc. Further signs such as positivity of other specific autoantibodies and/or videocapillaroscopy pattern may allow very early diagnosis of SSc. This may allow follow-up of the patient and start the appropriate therapeutic regimen to arrest the disease progression when an organ involvement is detected. The time gap between the onset of signs and the diagnosis can be considered the ‘window of opportunity‘ where the disease may be stopped before skin and internal organs are irreversibly damaged. Therefore, the main topic discussed in this chapter will be ‘why we need to distinguish between a very early and an early diagnosis of SSc‘
Immunosuppression for interstitial lung disease in systemic sclerosis
The efficacy of immunosuppressors in the treatment of systemic sclerosis-interstitial lung disease is still matter of controversy. In this review we will analyse the evidence that immunosuppressors, despite not being able to reverse fibrotic changes, may help in slowing disease progression. Induction treatment with cyclophosphamide should be started as soon as possible in patients at risk for progression. Mycophenolate mofetil and rituximab have to be considered in patients who are unable to tolerate cyclophosphamide. After remission, maintenance treatment with mycophenolate mofetil or azathioprine should be started in order to preserve the benefits achieved during the induction treatment
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