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Systemic sclerosis: clinical features and management
اسکلروز سیستمیک: ویژگی های بالینی و مدیریت-2018 Systemic sclerosis (SSc) differs from other multisystem connective tis
sue/autoimmune diseases in that its clinical features result mainly from
a combination of fibrosis and vascular abnormality (rather than from
inflammation). This has major implications for management. SSc is
associated with high morbidity and mortality, and is often very painful
and disabling. There are two major subtypes, defined on the basis of
the extent of skin involvement: limited (often previously referred to
as CREST) and diffuse cutaneous. The two subtypes have very
different natural histories, autoantibody associations and prognoses,
and require different approaches to management, at least in their
early stages. The two most characteristic features of SSc are Ray
naud’s phenomenon (which can be very severe) and skin thickening
(ʻsclerodermaʼ). Although both cause troublesome, often disabling
symptoms, it is the internal organ involvement of the disease that
can be life-threatening. This article discusses recent advances in
early diagnosis, clinical features and the approach to investigation
and management. It is an exciting time for clinicians with an interest
in SSc, because following on from the development of new treatments
for several organ-based complications (e.g. pulmonary arterial hyper
tension, digital ulceration), several promising ʻdisease-modifyingʼ ther
apies (including antifibrotics) are currently being studied in clinical
trials.
Keywords: MRCP; pulmonary fibrosis; pulmonary hypertension; Raynaud’s phenomenon; scleroderma; scleroderma renal crisis; sys temic sclerosis |
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