Systemic Scleroderma


Manifestation

SSc, younger-age onset

SSc

Anticentromere antibodies (ACA)

Positive mainly in limited cutaneous form

In general higher incidence

Pulmonary hypertension, muscle weakness, involvement of the heart and kidneys

More frequently in diffuse cutaneous SSc

In general higher incidence

Peripheral vasculopathy

Frequent

Less frequent

General prognosis

Better

Markedly worse


Modified according to Manno et al. [5] and Hügle et al. [4]



Prognosis of the disease is usually given by severity of pulmonary involvement; the interstitial process usually gradually leads to development of global respiratory insufficiency with fatal consequences. Pulmonary arterial hypertension, on the other hand, is characterized by rapid progress with a very poor diagnosis. The age over 75 years is in late-onset SSc associated with a substantially worse prognosis than in patients older than 60 years [9]. In the cohort from the EUSTAR/EULAR database, mortality due to SSc was higher in the late-onset group, but the survival time from diagnosis was longer compared with the younger patients. The disease is manifested by a far more severe involvement of individual organs, and the condition is often complicated by various comorbidities; therefore, diagnosis must be established as soon as possible. In the patients, special attention should be paid to the incidence of malignant processes, including solid tumors in men with extensive skin involvement; pulmonary fibrosis as a predisposition to lung cancer, breast cancer in women, and chronic reflux esophagitis; and Barrett’s esophagus as a predisposition to esophageal carcinoma.

SSc treatment must be always comprehensive. The basic process cannot be usually controlled by pharmacological treatment as the effect of most preparations can be observed only in the cutaneous form of the disease. In the edematous phase of the disease, patients receive corticosteroids with methotrexate and in active alveolitis corticosteroids on a long-term basis and repeated intravenous pulses of cyclophosphamide [10]. Other procedures are organ specific and have only a symptomatic effect. In late-onset SSc treatment, the basic therapeutic procedures do not differ substantially from treatment of younger patients, only it is complicated by frequent visceral organ involvement and neurological comorbidities and the associated risk of serious drug interactions. The future of a complex SSc treatment lies in biological preparations that will be targeted at individual phases of the immunopathological process. The results of clinical trials focused on the current biologic drugs used to treat rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis are not unequivocal.

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Jul 16, 2017 | Posted by in MUSCULOSKELETAL MEDICINE | Comments Off on Systemic Scleroderma

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