Scleroderma, A Simple Guide To The Condition, Diagnosis, Treatment And Related Conditions

This book describes Scleroderma, Diagnosis and Treatment and Related DiseasesScleroderma is an autoimmune disease typically featured by the poor regulation of the immune system, vasculopathy and fibrosis. It is a connective tissue disorder featured primarily by the thickening and hardening of the skin produced by accumulation of collagen, and by injuries to the smallest arteries. There are 2 main types of scleroderma: localized and systemic.
In localized scleroderma, the disease involves mainly the skin and may have an effect on the muscles and bones. In systemic scleroderma, there is a participation of the internal organs, such as the digestive tract, heart, lungs, and kidneys, among others. The severity and prognosis of scleroderma are inconsistent. There are 2 subtypes of Scleroderma:1. Limited cutaneous Systemic Sclerosis (LcSSc)In LcSSc the skin thickening and hardening happens in the fingers, hands, face, forearms distal to elbow, and lower legs distal to the knees.
LcSSc is linked with anti-centomere antibodies, pulmonary arterial hypertension (PAH) with or without interstitial lung disease (ILD). CREST syndrome (Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, Telangiectasias) is linked with LcSSc.2. Diffuse cutaneous Systemic Sclerosis (DcSSc)In DcSSc the skin thickening and hardening happens proximal to the elbows and knees and may affect the trunk.
DcSSc normally have a faster start and progression and is at danger of progressing to the visceral organs, such as the kidneys, heart, lungs and gastrointestinal tract. Normal involvements are:a. Interstitial lung disease with or without PAH, cardiomyopathy, b. Diffuse gastrointestinal disease andc. Renal crisis. CauseThe precise cause of scleroderma is not fully known. There is some presence that genetic and environmental factors have a part in the genesis of scleroderma.
Silica and certain organic solvents are documented as danger factors. The result is a trigger of the immune system, causing blood vessel injury and damage to tissues that cause scar tissue formation and the collection of excess collagen. Genetic factors have at least a limited part. SymptomsScleroderma produces thick, hard patches of skin. Localized scleroderma is restricted to the skin and underlying tissueSystemic SclerosisSystemic Sclerosis you are so scaryYour disease attacked my whole bodyMost of all my skin is tight and thickenedMy fingers are all puffy and swollen.
My skin is so itchy and sometimes painfulMy fingers becomes blue when it becomes coolA condition called Raynauld's phenomenonThere is distension always in my abdomenSometimes I have heartburn and problem swallowingI feel as though my intestinal tract is not movingSometime my body is unable to absorb any nutritionAnd I end up with either diarrhea or constipationI tend to get overgrowth of bacteria in my GITI even soil my underwear before I could pass itMy heart enlarges and I develop pulmonary arterial hypertensionIs that why I am so breathless or is it due to lung infectionDoctor please be kind, keep me warmAnd give medicine to keep me strongDo not let my kidney fail or falterLet me spend some time with my son and daughter-An original poem by Kenneth KeeDiagnosis:1.
Physical signs of skin thickening, puffy fingers2. Auto antibodies-antinuclear antibody test is positive in majority of cases (90%)3. Nail capillaroscopy is a method to study micro circulation of nail bedTreatmentThere is no cure for SclerodermaTopical corticosteroids are the primary treatment. Stem cell transplantation and treatments directing on cytokine signaling, such as interleukin-6 (IL-6), have produced promising resultsTABLE OF CONTENTIntroductionC...