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Scleroderma: Clinical And Physical Presentations, Prognosis And Treatment As A Progressive Systemic Slcerosis Disease

Updated on February 13, 2014

Rheumatoid Nodule


Scleroderma Overview

The clinically significant presentations include systemic manifestations, bones and joints, the alimentary canal and renal involvement. In this Hub, a special kind of scleroderma known as the mixed connective tissue disease would be briefly touched.

Systemic manifestations: The pattern of involvement reported in Indian series (especially) is acrosclerosis (53%), arthralgia (83%), arthritis (20%), upper GI lesions (66%), lower GI lesion (33%), lungs (20%) and Kidneys (25%). Progressive systemic sclerosis overlaps with other connective tissue disorders such as systemic lupus erythematosus (14%), rheumatoid lupus erythematosus (14%), rheumatoid arthritis (8.4%) and mixed connective tissue disease (2.8%).

Bones and Joints: Mobility is restricted due to fibrosis of soft tissue. Primary articular involvement can occur in many. Creptus can be palpated over major joints and tendons and 50% develop deformities. Long standing cases show erosion and absorption of the terminal phalanges.

Gastrointestinal Tract: Affection of the GI tract can be demonstrated by investigations even in the asymptomatic cases. Oesophagus and intestines are affected most frequently. Common symptom is dysphagia, which is initially due to esophageal spasm and later fibrosis. Barium swallow reveals dilated esophagus resembling achalasia cardia. The small and large intestines show dilatation, impaired peristalsis and malabsorption.

Involvement of the lungs results in progressive interstitial fibrosis and alveolocapillary block syndromes. Cystic changes are seen occasionally; secondary pulmonary hypertension and cor pulmonale develop in long standing cases. Heart shows enlargement, myocardial dysfunction and rarely conduction defects. Malignant hypertension is more common.

Renal involvement: Secondary hypertension and renal failure may follow. Nervous system involvement manifests as cranial or peripheral neuropathy at times.

Laboratory Investigations: Erythrocyte sedimentation rate is moderately raised but it may be normal in early cases and in cases where only the skin is affected. During the active phase, IgG may be elevated. Muscle enzymes are elevated when myopathy occurs. Rheumatoid factor is positive in 20 – 25% and antinuclear factor (ANF) in about 40% cases in low titers.

Differential diagnosis: Progressive systemic sclerosis must be distinguished from other collagen disorders such as dermatomyositis and mixed connective tissue disease. Thickening and tightness of the skin may occur in other disorders like eosinophilic fasciitis scleroderma adultorum Bushke, myxedema and acromegaly.

Scleroderma Manifestations


Sclerodema adultorum Bushke

This condition is characterized by painless edematous induration with an abrupt onset over a short period of time occurring in young subjects. Face, scalp, trunk and proximal parts of the extremities are widely affected. Though the exact cause is not clear, some cases are seen to follow streptococcal infection. There is accumulation of mucopolysacchrarides in the dermis and in the underlying muscles. The condition resolves spontaneously over a period of 6- 12 months even without specific treatment. Systemic manifestations of PSS are not present.

In eosinophils fascilitis which starts in the form of painful and tender swellings over the extremities, there is fasciitis, myositis, eosinophilia and hypergammaglobulinemia. The skin is indurated and tight. Biopsy reveals perivascular infiltration of eosinophils, histiocytes lymphocytes and plasma cells in the skin, fat and even underlying muscles. These lesions are self-limiting. Myxedema and acromegaly are associated with other endocrine abnormalities.

Course and prognosis: Progressive systemic sclerosis is a slowly progressive disease. Widespread skin involvement, lesions of the kidneys, lungs and heart and onset earlier in life are unfavourable factors. More than 70% survive 5 years or more.

Treatment: Several drugs like para-aminobenzoic acid (PABA) have been tried, but no drug is available which will arrest the progression of the disease. The use of corticosteroids is controversial. The renal lesions may even be aggravated by corticosteroids. Non-steroidal anti-inflammatory drugs like indomethacin or ibuprofen may help in relieving arthralgia and arthritis. D. Penicillamine may help in the early stages on account of its ability to inhibit cross-linking of collage. Blood flow in the digital arteries can be improved by the infusion of low molecular weight dextran. This may prevent ischemic ulcers and help in their recovery. Intractable Raynaud’s syndrome is relieved by cervical sympathectomy.

Mixed Connective Tissue Disease

In this condition, the features of scleroderma, systemic lupus and polymyositis overlap. Women suffer four times more frequently than men. Onset is in the third and fourth decades. Prognosis varies in individual cases. In general, prognosis is good and the condition responds to small doses of corticosteroids.

© 2014 Funom Theophilus Makama


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