EMQs in Clinical Medicine.pdf - Peshawar Medical College

Rheumatological conditions – answers 129
tight skin gives rise to the ‘beak’ nose and small mouth aperture (microstomia).
Limited scleroderma is associated with the presence of anticentromere
antibodies. The term ‘CREST syndrome’ was the previous
name for limited cutaneous scleroderma because it summarized the signs
of calcinosis, Raynaud’s phenomenon, oesophageal dysmotility, sclerodactyly
and telangiectasia that can be found in affected patients.
Diffuse scleroderma is characterized by oedema and extensive sclerosis
which can involve most of the body including major organs, e.g. heart,
lungs, kidneys. Diffuse scleroderma is associated with the presence of
anti-topoisomerase and anti-RNA antibodies.
A
B
K
A 51-year-old woman complains of weakness in her shoulders and
thighs. She has a significantly raised CK level.
Polymyositis is an autoimmune condition characterized by a nonsuppurative
inflammation of striated muscle that gives rise to a
symmetrical proximal myopathy. Unlike other causes of pain and weakness
(e.g. polymyalgia rheumatica) CK is characteristically elevated.
Electromyography (EMG) shows fibrillation potentials in this
condition.
Dermatomyositis describes polymyositis associated with a heliotrope
(purple) rash on the cheeks and other areas of skin exposed to sunlight.
Dermatomyositis is associated with an increased risk of malignancy.
Treatment is with steroids and other immunosuppressive agents.
A 25-year-old man has been referred to rheumatology clinic with
multiple painful stiff joints and uveitis. He also complains of ulcers on
his penis and mouth.
Behçet’s disease is an inflammatory disorder of unknown aetiology characterized
by signs of orogenital ulceration and eye lesions, e.g. uveitis,
retinal vein occlusion. A positive skin pathergy test is pathognomonic.
A positive result involves the development of a pustule on the skin
within 24 h of being pricked at that exact point with a sterile needle.
Erythema nodosum is a recognized cutaneous manifestation of Behçet’s
disease.
Neurological manifestations of Behçet’s disease include meningoencephalitis,
dementia and cerebral vein occlusion.
Behçet’s disease is managed with immunosuppressants, e.g. corticosteroids,
ciclosporin.
A 45-year-old woman presents with dryness in the eyes and mouth.
Her Schirmer’s test is positive.
Primary Sjögren’s syndrome (SS) is characterized by symptoms of dry
eyes (xerophthalmia) and dry mouth (xerostomia). Secondary SS
describes the above symptoms in the presence of other autoimmune disease
processes, e.g. rheumatoid arthritis.