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Scleroderma – Pathogenesis, Clinical features and Treatment

Scleroderma is a generalized condition characterized by excessive deposition of collagen in the skin and multiple internal organs, alterations in the vasculature and immunologic abnormalities. The pathogenesis is still very unclear and is only a hypothesis. It is hypothesised that, an unknown etiological agent may trigger the disease in a genetically predisposed individual, which may lead to
molecular and cellular alterations. Etiological agents could be viruses, chemicals, presence of HLA-B8, DR5, DR3 and
DR52 or even a phenotypic change in the cells. The cells altered are the fibroblasts, endothelial cells and the immune cells. Altered fibroblasts produce excessive collagenin the skin and multiple organs, altered endothelial cells may cause obliteration of arteries andarterioles and altered immune cells may have a dysregulated production of cytokines and abnormal production of auto-antibodies. These auto-antibodies may combine with self-antigens, forming immune complexes and along with cytokines may further damage blood vessels and induce fibroblasts to produce collagen. This condition presents mostly in adults of age 30-50 and has a female predilection of 3-6:1. Scleroderma usually begins with symptoms on the skin of the face, hands and trunk. One of the first signs of the disease, although not specific for scleroderma, is the Raynaud’s phenomenon. In Raynaud’s phenomenon, there is narrowing or vasoconstriction of the small arteries supplying the skin of the extremities. When the affected person is exposed to cold temperatures or has an emotional distress, there is vasospasm and lesser blood supply to the extremities leading to the skin becoming pale yellow or white and cold and numb. The skin develops a hard texture, cannot be wrinkled or picked up, because it becomes indurated or fixed to the underlying connective tissue. When the skin of the face is involved, the
face becomes taut, expressionless and is mask-like. Fibrosis of fingers may cause tightness and stiffness of skin on the digits leading to claw like fingers. There may be ulceration under the finger tips, due to vascular obliteration and ischemia. Other organs may slowly get involved with fibrosis manifesting in the kidneys, lungs and the gastrointestinal tract. Progressive fibrosis, may lead to multiple organ failure. A milder form of systemic sclerosis is called localized scleroderma or morphea. There may be one or many, well defined areas of elevated linear patches on the skin of the face and trunk which may look like scars. They are hence called, coup de sabre which means “strike of a sword” since they look like scars due to a blow of a sword! This localised condition is however, not life threatening unlike its systemic counterpart. Peri-oral tissues become hard as a result
of collagen deposition and this may result in microstomia, limitation of the opening
of the mouth. Furrows radiate from the mouth to produce a “purse-string” appearance. Intra-orally, the tongue becomes stiff, hypomobile and board like, leading to difficulty in eating and speaking. The oesophagus looses its elasticity and
becomes firm, leading to difficulty in swallowing. Multiple areas of gingival recession are found and the patient also develops xerostomia due to fibrosis of salivary glands. Dental radiographs may reveal a diffuse widening of the periodontal ligament space. This has been confirmed by many studies, and is in fact enough to establish a tentative diagnosis of scleroderma, although prudence is to be practised as there are other conditions like osteosarcoma and periodontal disease which may have this feature. 10-20 % of the patients may show, resorption of the posterior ramus, coronoid process and condyle. Under the microscope, the primary feature is the vast and diffuse deposition of collagen in the stroma of the organ involved. Unfortunately, there is no specific satisfactory treatment for scleroderma. Sometimes, the disease may stabilize on its own or patients may succumb to multiple organ failure due to fibrosis. Drugs like pencillamine have been administered to reduce collage deposition and cortico-steroids are of little benefit. Most of the management strategies have been to give symptomatic relief. Overall, the prognosis for the systemic condition is poor, although it is better for patients with the localised cutaneous form of the disease.

46 thoughts on “Scleroderma – Pathogenesis, Clinical features and Treatment

  1. Great video. I suggest you should add pictures of the conditions mentioned to reinforce the information.

  2. Good video… but i think diseases such as these could do without the sound effects… u might wanna follow up on the sound osmosis uses… cause if someone who has it, is watching the video, it might be rather offensive to that person.
    nevertheless good job

  3. In the kidneys affected by scleroderma, scleroderma renal crisis can cripple your kidneys to irreversible failure. Fibrosis from this disease can damage the kidneys first through hyperplastic arteriosclerosis, and if it is not controlled, this can lead to the kiss of death for your kidneys through fibrinoid necrosis, and at that point, you will need to get your kidneys dialyzed or transplanted, or you will face death. If there is no irreversible damage in the renals, the doctor will often prescribe antihypertensive meds, and especially angiotensin-converting enzyme inhibitors – or ACE inhibitors, to keep any kidney damage that is reversible from worsening into irreversible damage.

  4. Great video , thank you for sharing. We were wondering if we could have your permission to use your video for our organization for educational purpose only? We are from China and we are the Chinese non profit organization for scleroderma.Our organization is a NGO established by the scleroderma patients. It’s the only organization that serves the scleroderma patients in China.
    Our organization strives to deliver the knowledge and up to date information for scleroderma, provide a platform for scleroderma patients to share their stories and treatments. We also work together with other rare disease organizations to help patients and at the same time to push our government to pay more attention to the rare diseases. We work hard to improve the life quality of the patients and help them to achieve equal opportunity to live. 
    We sincerely hope that we can work together to raise the awareness, promote the drug research and share the therapeutic method to help our patients live better.
    If you have any questions and concerns, please email us: [email protected]
    Looking forward to your response.

  5. Only Ayurvedic treatment is effective for scleroderna. For vascular obtraction there are not specific medicine in modern system.con.no.what's App.8652016172,9867279966,9869141564.

  6. I'm loving this channel. It's amazing, such good quality contents in a super appealing form! I'm an italian student,i always make lots of diagrams and stuff to make informations easy to remember and i love when my notes are also graphically nice! So yeah, this is super helpful and yours is my dream job i guess 😛

  7. Scleroderma is what happens when the body accumulates too much acid and that leads to a tissue damage. To reverse it all you need to do is alkalize the body by drinking Kangen water and repair the damage by drinking Reliv nutrition. Not sure why people choose pain, suffering and death, when simple solution exist

  8. 1 nattokinase reduces fibrin build up. # 2 chanca piedra herb breaks down calcium build up. #3 vitamin K2, magnesuim and vitamin E. Removes calcium out the arteries and to the bones where it should go. #4 chlorophyll and Nac removes heavy metals and toxins out the body so your liver can work properly and clean the blood. #5 serrapeptase will help with inflammation and pain. #6 is the most important. You must go alkaline vegan ( fruit and veggies only). You must stop using and eating all toxins. No sugar, no bread no white flour, no dairy , no eat, no meat, no soy. #7 chinese medicine has reversed this find a good ole chinese doctor or go to Dr. Robert morse office in florida. Find him on youtube. In the beginning there was the garden where sickness and disease did not exist. If you want to heal go back to the garden. Love you all

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