Page 372 - Medicine and Surgery

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368 Chapter 8: Musculoskeletal system

Aetiology Microscopy
Genetic factors are suggested by variations in inci- Extensive deposition of collagen is seen in the skin, vis-

dence between ethnic groups and familial clustering. cera, small and medium-sized arteries, arterioles and
Infectious and non-infectious environmental agents capillaries. In the wall of blood vessels concentric prolif-

have also been implicated. A scleroderma like disor- eration and thickening of the intima and fibrosis of the
der is seen following exposure to silica, vinyl chlo- adventitia is seen.
ride, petroleum-based solvents, rape seed oil and
bleomycin. Investigations
AswithsystemiclupuserythematosustheESRisraised

with a normal CRP.
Pathophysiology
Autoantibodies include anticentromere, antitopo-
Immune system activation with the production of au-
isomerase-1 (Scl-70), anti-RNA polymerase, and
toantibodies including antiendothelial cell antibodies
rheumatoid factor is positive in 30%.
andupregulationofcelladhesionmoleculescausesdam-
Full blood count may show anaemia of chronic disease
age to blood vessels. This results in the release of acti-
or haemolytic anaemia.
vating factors, changes in vascular permeability and the
X-ray changes: In the hands there is a loss of the tufts
proliferation of active fibroblasts, which produce exces-
of the terminal phalanges and soft tissue calcification.
sive collagen.
There is basal fibrosis in the lungs with reticulonodu-
lar shadowing. Contrast imaging of the GI tract may
Clinical features demonstrate oesophageal dilation, poor motility and
Anumber of patterns of scleroderma and systemic scle- diverticulae of large bowel.
rosis are recognised:
Management
There is no curative treatment, education is essential and
Localised scleroderma
symptomatic treatments initiated.
Morphoea are patches of sclerotic skin on the trunk

Raynaud’s phenomenon is treated by avoiding cold,
andlimbs,whichmaybelocalisedormoregeneralised.
and vasodilators such as nifedipine, ACE inhibitors or
Linear scleroderma describes a dermatomal distribu-

in severe cases prostaglandin infusions.
tion of skin and subcutaneous sclerosis.
Oesophageal symptoms are treated with H 2 antag-

onists or proton pump inhibitors, and oesophageal
Systemic sclerosis
stricturesmayrequirerepeateddilatation.Malabsorp-
Limited cutaneous systemic sclerosis begins with

tion may require changes in diet.
Raynaud’s phenomenon prior to the development Hypertension resulting from renal involvement needs
of skin sclerosis restricted to the hands (digital ul-
control with ACE inhibitors or calcium channel an-
cers, ischaemia and necrosis), face (beaked nose, small
tagonists.
mouth), feet and forearms. Previously this form of The role of immunosuppressive drugs remains un-
systemic sclerosis was referred to as CREST syndrome
clear.
(calcinosis,Raynaud’sdisease,esophagealdysmotility, Bosentan (an endothelin receptor antagonist) is used
sclerodactyly and telangiectasia).
in the treatment of pulmonary hypertension sec-
Diffusecutaneoussystemicsclerosisreferstoextensive

ondary to systemic sclerosis and has been shown to
skinsclerosisalongwithmultisysteminvolvement(see
reduce the formation of multiple digital ulcers.
Fig. 8.4).
Systemic sclerosis sine scleroderma is a rare form of Prognosis

the illness in which there is systemic disease in the Localised forms are milder and do not progress to sys-
absence of skin sclerosis. temicinvolvement.Notreatmenthasbeenshowntoalter
Overlap syndromes have combinations of the features the long-term progression of scleroderma. Diffuse dis-

of systemic sclerosis, systemic lupus erythematosus, ease with severe visceral involvement carries the worst
dermatomyositis or rheumatoid arthritis. prognosis.