Scleroderma Year 4 Internal Medicine Notes OmpathStudy

Scleroderma Year 4 · Internal Medicine Collection: 4TH YEAR ALL NOTES / IMED / I MED BEAUTIFUL NOTES / RHEUMATOLOGY -- Scleroderma Spectrum Disease\n435 medicine teamwork\n\n Lecture objectives: \n⇨ Background\n⇨ Scleroderma\n⇨ Sjogren’s Syndrome\n⇨ Inflammatory Myopathies\n\n Done By​: ​Noura AlTawil, Kayan Kaaki\nRevised By​:​ Omar Alsulaiman, Ahmed Alyahya\nReferences: Doctors’ Slides, Kumar, Master The Boards\n\n [ ​Important​ ​ ​ ​Notes ​ ​ ​Extra​ ​ ​Editing file​ ] \n\n Scleroderma Spectrum Diseases\n● Scleroderma Spectrum diseases are: a group of ​heterogeneous diseases that have a ​predominant feature and\nshare other ​common​ features.\n● They are rare, difficult to treat & associated with significant morbidity and mortality. Sjogren’s Syndrome (SS) 1- Scleroderma or Systemic Sclerosis (SSc): \n❖ SSc is ​characterized​ by ​skin thickening​,​ vasculopathy​ (Raynaud’s Phenomenon) and ​autoantibody production​.\n● Scleroderma is a disease that has 3 different components and it's important to know that because when\nyou're going to manage the patient you're gonna manage him symptom by symptom or organ by organ.\n\n ○ They have interstitial lung disease, pulmonary hypertension, arthritis.\n❖ Types are classified​ based on​ cutaneous involvement​ into: ​(​Starts in the finger then spread proximally and the face is\ninvolved in both types) \nDiffuse (30% of cases) Limited (70% of cases) \n(It starts with the fingers and/or toes and once you (It starts with the fingers and/or toes and the\nhave involvement proximal to the elbow or to the involvement is distal to the elbows and knees)\nknees it is diffuse) \n- Associated with more​ internal organ - Often ​more indolent​ ​(​Always associated with\ninvolvement hand thickening)\n- Worse​ prognosis. - Has a higher risk of ​pulmonary\nhypertension \nAnti-topoisomerase / RNA polymerase III antibodies Anti-centromere antibodies \nClinical features: Clinical features: ​Usually starts with Raynaud’s\n● The skin changes develop more rapidly. Phenomenon many years before any skin changes\n● Early involvement of other organ: happen.\n● GIT involvement ● The skin is thickened, bound down to\n● Renal involvement underlying structure and the finger taper\n● Lung disease (sclerodactyly).\n​(mentioned below) ● Characteristic​ facial appearance: ‘beaking’\nof the nose, radial furrowing of the lips and\nlimitation of mouth opening.\n● Known as CREST Syndrome​ ( ​C​alcinosis,\nR​aynaud's,​ E​sophageal dysmotility,\nS​clerodactyly, ​T​elangiectasia) \n\n ​Autoantibodies​:\nScl-70 Associated with ​diffuse subset​, Interstitial Lung Disease (ILD), Reduced risk of\n(anti-topoisomerase) Pulmonary Arterial Hypertension (PAH).\nMost specific test \nAnti-centromere Associated with ​limited subset​, PAH, and Digital Ulcer (DU).​ ​(Extremely specific for\nCREST Syndrome) \nRNA polymerase III Associated with SRC, malignancy associated SSc and mortality. \nScl-PM Associated with ​myositis ​overlap. \nANA positive in 85% to 90%, but ​non​specific.\n\n 2013 Criteria for the Classification of Systemic Sclerosis.\n\n - First item in criteria whether the sub-type is diffused or limited the score remains 9, but you have to eliminate any other causes that might\nimitate scleroderma such as Nephrogenic Systemic Fibrosis (allergic reaction to Gadolinium with MRI in patients with renal failure) or Graft\nVersus Host Disease (Post bone marrow transplantation they have severe GI and skin manifestations)\n\n​Organ involvement in SSc:​ ​(​when you treat, you will treat each organ one by one)\n● SSc is a disease that is difficult to evaluate, treat, and monitor.\n● It’s very​ heterogeneous​, usually diagnosed late and there is ​no​ ​single drug that treats everything.\n● Pathogenesis in each organ involved is ​not the same​: could be Neurovascular, Fibroproliferative, or\nInflammatory.\n● A strategy should be adopted to evaluate each manifestation and organ involved on a regular basis. \n\n​Skin involvement:\n- Skin​ is the​ largest​ and ​most important ​organ involved in SSc.\n- Skin involvement has been considered a ​reflection of ​internal organ involvement.\n- It predicts severe disease and mortality. ​the more skin involved the higher the chance that internal organs are involved\nthe more severe the disease\n- It always starts in the fingers and toes and extends ​proximally.​ Contracture of the fingers and disability are\npreventable with stretching exercise. Patients are advised to use emollients and creams ​(and vaseline on wet\nskin)​ at all times.\n- Skin loosening occurs 5 years after the onset of the disease.\n- The diagnosis of scleroderma is primarily based upon the presence of characteristic skin changes.\n- Treatment ​is usually initiated when active skin inflammation is apparent or progressive skin thickening. ​(So once\nyou stop the inflammation with immunosuppressive agent fibroblast will be deactivated and there won't be collagen deposition) \n1.Methotrexate (if no ILD ​ ​Because it