ArtsAutosBooksBusinessEducationEntertainmentFamilyFashionFoodGamesGenderHealthHolidaysHomeHubPagesPersonal FinancePetsPoliticsReligionSportsTechnologyTravel

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

Updated on February 13, 2014

Rheumatoid Nodule

Source

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

Source

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

Comments

    0 of 8192 characters used
    Post Comment

    No comments yet.

    working

    This website uses cookies

    As a user in the EEA, your approval is needed on a few things. To provide a better website experience, hubpages.com uses cookies (and other similar technologies) and may collect, process, and share personal data. Please choose which areas of our service you consent to our doing so.

    For more information on managing or withdrawing consents and how we handle data, visit our Privacy Policy at: https://hubpages.com/privacy-policy#gdpr

    Show Details
    Necessary
    HubPages Device IDThis is used to identify particular browsers or devices when the access the service, and is used for security reasons.
    LoginThis is necessary to sign in to the HubPages Service.
    Google RecaptchaThis is used to prevent bots and spam. (Privacy Policy)
    AkismetThis is used to detect comment spam. (Privacy Policy)
    HubPages Google AnalyticsThis is used to provide data on traffic to our website, all personally identifyable data is anonymized. (Privacy Policy)
    HubPages Traffic PixelThis is used to collect data on traffic to articles and other pages on our site. Unless you are signed in to a HubPages account, all personally identifiable information is anonymized.
    Amazon Web ServicesThis is a cloud services platform that we used to host our service. (Privacy Policy)
    CloudflareThis is a cloud CDN service that we use to efficiently deliver files required for our service to operate such as javascript, cascading style sheets, images, and videos. (Privacy Policy)
    Google Hosted LibrariesJavascript software libraries such as jQuery are loaded at endpoints on the googleapis.com or gstatic.com domains, for performance and efficiency reasons. (Privacy Policy)
    Features
    Google Custom SearchThis is feature allows you to search the site. (Privacy Policy)
    Google MapsSome articles have Google Maps embedded in them. (Privacy Policy)
    Google ChartsThis is used to display charts and graphs on articles and the author center. (Privacy Policy)
    Google AdSense Host APIThis service allows you to sign up for or associate a Google AdSense account with HubPages, so that you can earn money from ads on your articles. No data is shared unless you engage with this feature. (Privacy Policy)
    Google YouTubeSome articles have YouTube videos embedded in them. (Privacy Policy)
    VimeoSome articles have Vimeo videos embedded in them. (Privacy Policy)
    PaypalThis is used for a registered author who enrolls in the HubPages Earnings program and requests to be paid via PayPal. No data is shared with Paypal unless you engage with this feature. (Privacy Policy)
    Facebook LoginYou can use this to streamline signing up for, or signing in to your Hubpages account. No data is shared with Facebook unless you engage with this feature. (Privacy Policy)
    MavenThis supports the Maven widget and search functionality. (Privacy Policy)
    Marketing
    Google AdSenseThis is an ad network. (Privacy Policy)
    Google DoubleClickGoogle provides ad serving technology and runs an ad network. (Privacy Policy)
    Index ExchangeThis is an ad network. (Privacy Policy)
    SovrnThis is an ad network. (Privacy Policy)
    Facebook AdsThis is an ad network. (Privacy Policy)
    Amazon Unified Ad MarketplaceThis is an ad network. (Privacy Policy)
    AppNexusThis is an ad network. (Privacy Policy)
    OpenxThis is an ad network. (Privacy Policy)
    Rubicon ProjectThis is an ad network. (Privacy Policy)
    TripleLiftThis is an ad network. (Privacy Policy)
    Say MediaWe partner with Say Media to deliver ad campaigns on our sites. (Privacy Policy)
    Remarketing PixelsWe may use remarketing pixels from advertising networks such as Google AdWords, Bing Ads, and Facebook in order to advertise the HubPages Service to people that have visited our sites.
    Conversion Tracking PixelsWe may use conversion tracking pixels from advertising networks such as Google AdWords, Bing Ads, and Facebook in order to identify when an advertisement has successfully resulted in the desired action, such as signing up for the HubPages Service or publishing an article on the HubPages Service.
    Statistics
    Author Google AnalyticsThis is used to provide traffic data and reports to the authors of articles on the HubPages Service. (Privacy Policy)
    ComscoreComScore is a media measurement and analytics company providing marketing data and analytics to enterprises, media and advertising agencies, and publishers. Non-consent will result in ComScore only processing obfuscated personal data. (Privacy Policy)
    Amazon Tracking PixelSome articles display amazon products as part of the Amazon Affiliate program, this pixel provides traffic statistics for those products (Privacy Policy)