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Diffuse Scleroderma


Presentation

  • The Scl-86 antigen is present in freshly prepared rabbit thymus, spleen, and liver nuclei as well as in nuclei from various cultured cell lines, but is not detectable in extractable nuclear antigen from rabbit thymus.[ncbi.nlm.nih.gov]
  • Acase of diffuse scleroderma in a 56-year-old woman who received paclitaxel for the treatment of a metastatic ovarian cancer is presented.[ncbi.nlm.nih.gov]
  • Kidney function abnormalities or proteinuria were present in 173 (26%); 48% had no abnormalities. Most patients had other explanations for the abnormality.[ncbi.nlm.nih.gov]
  • In patients presenting with early diffuse scleroderma prognosis is variable. Our goal was to determine possible baseline predictors of future good outcomes.[ncbi.nlm.nih.gov]
  • CASE REPORT 153 Downloads 22 Citations Abstract A case of diffuse scleroderma in a 56-year-old woman who received paclitaxel for the treatment of a metastatic ovarian cancer is presented.[link.springer.com]
Weight Loss
  • loss, pruritis Skin thickening - no or minimal progression Skin thickening - rapid progression, usually reaching peak involvement within 2 years Organs affected - Reynaud's, fingertip ulceration, oesophageal symptoms Organs affected - risk of renal ([barts.wikia.com]
  • Arthralgias, arthritis, and muscle weakness may occur in as many as a quarter of patients, and a small number may experience dyspnea, weight loss and dysphagia as well.[emedicine.com]
  • It can also lead to tiredness, weight loss and loss of appetite. Cardiac Shortness of breath due to heart problems.[sclerodermasociety.co.uk]
  • loss, fatigue, and joint pain and stiffness symptoms come on suddenly and get worse quickly over the first few years, but then the condition normally settles and the skin may gradually improve In some cases of systemic sclerosis, organs such as the heart[nhs.uk]
Anemia
  • Anemia preceded allograft SRC in two cases, pericardial effusion in one, and skin tightening in two others.[doi.org]
  • Anemia: Low red blood cell counts (anemia) can contribute to many symptoms including fatigue, shortness of breath, or dizziness. This can occur because of low iron or vitamin levels, a chronic inflammatory state, or for other reasons.[hss.edu]
  • GAVE can be a cause of upper gastrointestinal bleeding or iron deficiency anemia in patients with scleroderma.[en.wikipedia.org]
  • It is usually heralded by sudden, severe hypertension with features of thrombotic microangiopathic hemolytic anemia.[merckmanuals.com]
  • On the one hand, studies of identical twins indicate that scleroderma is not inherited: it does not pass from parent to child like sickle cell anemia or color blindness, both of which are found on a single gene with defective changes.[center4research.org]
Swelling of the Finger
  • There can be swelling of the fingers, hands, forearms and face and sometimes the feet and lower legs. This is followed by a skin thickening and tightness that can limit body movement.[drugs.com]
Dyspnea
  • Arthralgias, arthritis, and muscle weakness may occur in as many as a quarter of patients, and a small number may experience dyspnea, weight loss and dysphagia as well.[emedicine.com]
  • Respiratory/lungs Progressive shortness of breath (dyspnea) and pain in the chest wall (precordial chest pain) and a persistent dry cough. Renal Hypertension or the kidneys not working well.[sclerodermasociety.co.uk]
  • […] or cardiac systems indicate a more severe prognosis one of the highest mortalities among systemic autoimmune diseases limited scleroderma is more benign Presentation Symptoms skin diffuse pruritus gastrointestinal acid reflux respiratory progressive dyspnea[medbullets.com]
  • GI disturbances (eg, heartburn, dysphagia) or respiratory complaints (eg, dyspnea) are occasionally the first manifestations. Swelling of the skin is usually symmetric and progresses to induration.[merckmanuals.com]
Rales
  • […] affecting the fingers ( sclerodactyly ) shiny with loss of “wrinkles” from skin folds limited mobility due to skin tightening digital ulceration edema not responsive to diuresis hyper and hypopigmentation telangiectasias on skin and mucosa respiratory dry rales[medbullets.com]
Constipation
  • There may be constipation alternating with diarrhea leading to malnutrition.[sclerodermasociety.co.uk]
  • Breathing problems may result from scarring in the lungs and can include: Dry cough Shortness of breath Wheezing Increased risk for lung cancer Digestive tract problems may include: Difficulty swallowing Esophageal reflux or heartburn Bloating after meals Constipation[nlm.nih.gov]
  • […] of systemic sclerosis Gastrointestinal symptoms Upper GIT Gastroesophageal reflux / heart burn Dyspepsia Dysphagia Early satiety Micro-aspiration (accelerates lung disease) Lower GIT Bloating, distention Nausea, vomiting Pain Alternating diarrhoea / constipation[dermnetnz.org]
  • Constipation and diarrhoea may alternate. Pseudo-obstruction is treated initially by bowel rest and antibiotics. Laparotomy may be needed. Surgery may be required for rectal prolapse.[patient.co.uk]
Diarrhea
  • There may be constipation alternating with diarrhea leading to malnutrition.[sclerodermasociety.co.uk]
  • […] may result from scarring in the lungs and can include: Dry cough Shortness of breath Wheezing Increased risk for lung cancer Digestive tract problems may include: Difficulty swallowing Esophageal reflux or heartburn Bloating after meals Constipation Diarrhea[nlm.nih.gov]
  • Shortness of breath Heartburn Diarrhea Weight Loss How Is Scleroderma Diagnosed? Your doctor will check you and ask about your health history. He’ll likely take an X-ray, do some blood tests, or take a small sample of skin (called a biopsy ).[webmd.com]
  • […] feet Pain, stiffness, and swelling of fingers and joints Wrist pain Breathing problems may result from scarring in the lungs and can include: Dry cough Shortness of breath Wheezing Digestive tract problems may include: Bloating after meals Constipation Diarrhea[medicalmarijuana.com]
Hypertension
  • Noninvasive assessment of PAs can be performed accurately in most patients independent of clinical signs of pulmonary hypertension.[ncbi.nlm.nih.gov]
  • A case of severe hypertension and acute renal failure developing in a 56 year old woman affected by diffuse scleroderma is reported.[ncbi.nlm.nih.gov]
  • […] symptoms generally diminished Skin thickening - stable or slow progression Skin thickening - Stable or slow regression Organs affected - Raynaud's, ulcers of digit tips, calinosis, oesophageal stricture, small bowel malabsorption, pulmonary arterial hypertension[barts.wikia.com]
  • Vascular complications of systemic sclerosis include Raynaud phenomena, finger and toe ulcers, and pulmonary hypertension.[grantome.com]
  • […] in Systemic Sclerosis and Treatment With Ambrisentan Completed NCT01051960 Phase 4 Ambrisentan 7 A Clinical Trial of Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated With Systemic Sclerosis Completed NCT01042158 Phase 4 tadalafil[malacards.org]
Chest Pain
  • Respiratory/lungs Progressive shortness of breath (dyspnea) and pain in the chest wall (precordial chest pain) and a persistent dry cough. Renal Hypertension or the kidneys not working well.[sclerodermasociety.co.uk]
  • Early symptoms of pulmonary hypertension include shortness of breath, chest pain, and fatigue. How common is scleroderma? Approximately 250 persons per million American adults are affected by scleroderma.[my.clevelandclinic.org]
  • pain Palpitations Renal disease Scleroderma renal crisis: Proteinuria High blood pressure Renal failure.[dermnetnz.org]
  • Problems can include chest pain, abnormal heart rhythms and heart failure. Kidneys. Kidney damage can lead to high blood pressure, headache, seizures and too little urine being made. Other symptoms.[drugs.com]
Arthritis
  • Abstract We report a case of subcorneal pustular dermatosis (SPD) in a patient with rheumatoid arthritis (RA) and diffuse scleroderma.[ncbi.nlm.nih.gov]
  • OBJECTIVE: Scoring poorly on the health assessment questionnaire (HAQ) has recently been shown to be a strong predictor of morbidity and mortality in rheumatoid arthritis (RA), while a good HAQ score is predictive of a better outcome.[ncbi.nlm.nih.gov]
  • Provo st and his expert contributors correlate dermatology and internal medi cine by describing the dermatologic manifestations of systemic disease, from rheumatoid arthritis to Raynaud's disease to periocarditis.[books.google.com]
  • PubMed, Arthritis Rheumatol, 08/03/2015. (Also see Skin Fibrosis ) Treatments and Clinical Trials Scleroderma Treatments and Clinical Trials. ISN.[sclero.org]
  • Funding and Disclosures Supported by grants from the National Institutes of Health (FR-0056), the Arthritis Foundation, the Health Research and Services Foundation, the Mary Hillman Jennings Foundation, and the RGK Foundation, Austin. Tex.[nejm.org]
Arthralgia
  • The Raynaud phenomenon, swelling of the extremities, and diffuse arthralgias often precede the onset... FULL TEXT[doi.org]
  • Musculoskeletal Joint pain can be either arthralgia or myalgia. The terms arthralgia and myalgia are often used in combination or interchangeably. Arthralgia is defined as pain in a joint whereas myalgia is generalised muscle pain.[sclerodermasociety.co.uk]
  • Arthralgias, arthritis, and muscle weakness may occur in as many as a quarter of patients, and a small number may experience dyspnea, weight loss and dysphagia as well.[emedicine.com]
  • […] mortalities among systemic autoimmune diseases limited scleroderma is more benign Presentation Symptoms skin diffuse pruritus gastrointestinal acid reflux respiratory progressive dyspnea dry cough due to restrictive lung disease musculoskeletal myalgias arthralgias[medbullets.com]
Joint Swelling
  • People with diffuse scleroderma often experience: Tiredness Loss of appetite and weight Joint swelling and/or pain Skin changes, such as swelling, appearing shiny, and feeling tight and itchy.[diseases.emedtv.com]
  • Symptoms common with diffuse scleroderma include: Tiredness Loss of appetite or loss of weight Joint swelling Joint pain Skin can swell, appear shiny, and feel tight and itchy. The damage of diffuse scleroderma occurs over a period of a few years.[verywell.com]
  • People with diffuse disease are often tired, lose appetite and weight, and have joint swelling or pain. Skin changes can cause the skin to swell, appear shiny, and feel tight and itchy.[sclerodermaontario.ca]
Tendon Friction Rub
  • Tendon friction rubs - palpable/audible over the flexor/extensor tendons of the hands, knees and ankles. GI features GI symptoms are extremely common in SSc, occurring in the majority of cases and are often one of the earlier manifestations [ 7 ].[patient.co.uk]
  • Tendonitis is more frequent with fibrosis of the tendon sheaths, give rise to characteristic tendon friction rubs.[arthritis.co.za]
Flexion Contracture
  • Flexion contractures may develop in the fingers, wrists, and elbows. Friction rubs may develop over the joints, tendon sheaths, and large bursae. Esophageal dysfunction is the most frequent visceral disturbance and occurs in most patients.[merckmanuals.com]
Localized Scleroderma
  • The two main types are systemic scleroderma and localized scleroderma. The milder forms are most often seen in women between the ages of 30 and 50; the more severe forms usually affect men, blacks, and older persons.[medical-dictionary.thefreedictionary.com]
  • Localized Scleroderma The changes, which occur in localized scleroderma, are usually found in only a few places on the skin or muscles, and rarely spread elsewhere. Generally, localized scleroderma is relatively mild.[scleroderma.org]
  • There are two types of localized scleroderma: morphea and linear. Morphea Reddish patches of skin that thicken into firm oval-shaped areas are distinctive of the morphea type of localized scleroderma.[verywell.com]
  • Treatment of Localized Scleroderma There is no one treatment for localized scleroderma.[center4research.org]
Hyperpigmentation
  • Skin can breakdown and discharge chalky material (calcium) Salt and pepper dyspigmentation ( hyperpigmentation and hypopigmentation ) Pruritus Rarer cutaneous features Morphoea Most frequently plaque, nodular or linear More common in limited cutaneous[dermnetnz.org]
  • During the subsequent 6 months, the patient developed lower limb oedema, which progressed to the hands and face, coupled with generalized pain and hyperpigmentation of skin.[doi.org]
  • 'Salt and pepper' appearance of skin, due to areas of hypopigmentation and hyperpigmentation. Dry or itchy skin; reduced hair over affected skin areas. Musculoskeletal features Joint pain and swelling. Myalgia (due to inflammatory myopathy).[patient.co.uk]
Skin Ulcer
  • Ulcerations on the fingertips and toes Pain and stiffness in the joints Persistent cough Shortness of breath Heartburn ( acid reflux ) Difficulty swallowing Digestive and gastrointestinal problems Constipation Weight loss Fatigue Hair loss In addition[my.clevelandclinic.org]
  • There also can be: Skin ulcers Skin that is lighter or darker than usual Loss of hair Abnormal skin dryness, including vaginal dryness Calcium deposits in the skin (subcutaneous calcinosis) Small red spots caused by localized swelling of tiny blood vessels[drugs.com]
Pruritus
  • Skin can breakdown and discharge chalky material (calcium) Salt and pepper dyspigmentation ( hyperpigmentation and hypopigmentation ) Pruritus Rarer cutaneous features Morphoea Most frequently plaque, nodular or linear More common in limited cutaneous[dermnetnz.org]
  • […] scleroderma is rapidly progressive involvement of pulmonary, renal, or cardiac systems indicate a more severe prognosis one of the highest mortalities among systemic autoimmune diseases limited scleroderma is more benign Presentation Symptoms skin diffuse pruritus[medbullets.com]
Alopecia
  • Her past medical history includes autoimmune thyroid disease and alopecia areata. Physical exam reveals sclerodactyly, and tight, hardened skin with limited mobility in her fingers. Her physician sends her for additional autoimmune workup.[medbullets.com]
Facial Pain
  • Neurologic/psychiatric Facial pain and decreased sensation. Pins and needles sensation (paresthesias) and weakness in the hand. Headaches, depression, and anxiety. It can also lead to tiredness, weight loss and loss of appetite.[sclerodermasociety.co.uk]
  • Other symptoms can include dry eyes and mouth, sudden episodes of severe facial pain (trigeminal neuralgia), and impotence.[drugs.com]

Workup

Pericardial Effusion
  • Anemia preceded allograft SRC in two cases, pericardial effusion in one, and skin tightening in two others.[doi.org]
  • Other risk factors for SRC include anaemia, HRT, pericardial effusion, cardiac insufficiency, high skin score and large joint contractures, the presence of antibodies to RNA polymerases (ARA; see below) and new cardiac events [ 1, 17, 18 ].[doi.org]
  • Pericarditis or pericardial effusion ; these may cause cardiac impairment or congestive cardiac failure. Arrhythmias and conduction defects (including bradyarrhythmias and tachyarrhythmias). Endocarditis.[patient.co.uk]
Esophageal Motility Disorder
  • Carla Maradey-Romero, Scott Gabbard and Ronnie Fass, Treatment of Esophageal Motility Disorders Based on the Chicago Classification, Current Treatment Options in Gastroenterology, 10.1007/s11938-014-0032-9, 12, 4, (441-455), (2014).[doi.org]
Soft Tissue Calcification
  • Scleroderma, also known as systemic sclerosis, is an autoimmune connective tissue disorder characterized by multisystem fibrosis and soft tissue calcification.[radiopaedia.org]
Myocardial Fibrosis
  • Diffuse myocardial fibrosis or cardiac conduction abnormalities occur. Intimal hyperplasia of interlobular and arcuate arteries can develop within the kidneys, causing renal ischemia and hypertension.[merckmanuals.com]
  • Myocardial fibrosis. Left ventricular (LV) systolic dysfunction, LV diastolic dysfunction. Pericarditis or pericardial effusion ; these may cause cardiac impairment or congestive cardiac failure.[patient.co.uk]

Treatment

  • Our findings do not provide evidence that MTX is significantly effective in the treatment of early diffuse SSc.[ncbi.nlm.nih.gov]
  • CONCLUSION: MMF was associated with a modest improvement in mRSS and was well tolerated in the treatment of dcSSc.[ncbi.nlm.nih.gov]
  • Acase of diffuse scleroderma in a 56-year-old woman who received paclitaxel for the treatment of a metastatic ovarian cancer is presented.[ncbi.nlm.nih.gov]
  • Latest research provides new treatment option and offers hope for Systemic Sclerosis using patients’ own Fat-Derived Stem Cells.[thebahamasweekly.com]
  • OBJECTIVE: To assess the safety and efficacy of anti-thymocyte globulin (ATG) followed by mycophenolate mofetil (MMF) in the treatment of diffuse scleroderma.[ncbi.nlm.nih.gov]

Prognosis

  • In patients presenting with early diffuse scleroderma prognosis is variable. Our goal was to determine possible baseline predictors of future good outcomes.[ncbi.nlm.nih.gov]
  • There are lots of questions re prognosis life expectancy etc going round in my head right now. my arms legs mouth are affected currently and my upper chest is just beginning to feel taut (3 days now) i have some acid reflux but that is it . would like[healthunlocked.com]
  • In some of the few studies that have looked at the characteristics of patients with sine scleroderma, it is mostly associated with limited forms of scleroderma rather than diffuse forms, and is generally considered to have a good prognosis.[sclerodermainfo.org]
  • […] trial was small, its results were unmatched in terms of symptom reversal and provided a benchmark for the safe and effective use of minocycline as a relatively benign, inexpensive treatment for a significantly disabling and painful disease with poor prognosis[roadback.org]
  • Treatment and prognosis No cure for scleroderma exists. Treatment aims to reduce systemic involvement and target particular symptomatic organ systems.[radiopaedia.org]

Etiology

  • CONCLUSION: Patients with diffuse scleroderma without renal crisis rarely have significant increases in serum creatinine or proteinuria that cannot be explained by other etiologies.[ncbi.nlm.nih.gov]
  • Etiology and pathogenesis of scleroderma. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley and Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017:chap 83. Varga J.[nlm.nih.gov]
  • ) to blue (hypoxia) to red (re-perfusion) E sophageal dysmotility S clerodactyly T elangiectasia Epidemiology demographics female male African Americans Caucasian 30-50 years of age but can affect all ages risk factors exposure to potential triggers Etiology[medbullets.com]
  • Scleroderma's etiology is unknown, although previous studies have provided a few hints.[nature.com]

Epidemiology

  • Jørn Olsen and Olga Basso, Reproductive Epidemiology, Handbook of Epidemiology, 10.1007/978-3-540-26577-1_27, (1043-1109), (2005).[doi.org]
  • Linked Articles Clinical and epidemiological research Britta Maurer Nicole Graf Suzana Jordan Carina Mihai Otylia Kowal-Bielecka Yannick Allanore Oliver Distler on behalf of EUSTAR coauthors Marco Matucci Cerinic Serena Guiducci Ulrich Walker Giovanni[ard.bmj.com]
  • […] blood flow to skin from either cold temperatures or stress, which causes vasospasms colors of affected area, commonly the digits, change from white (ischemia) to blue (hypoxia) to red (re-perfusion) E sophageal dysmotility S clerodactyly T elangiectasia Epidemiology[medbullets.com]
  • Catto, Epidemiology of Bladder Cancer: A Systematic Review and Contemporary Update of Risk Factors in 2018, European Urology, 10.1016/j.eururo.2018.09.001, (2018). John G. Hanly, Qiuju Li, Li Su, Murray B.[doi.org]
  • This article reviews the epidemiology, pathogenesis, risk factors, clinical features, and treatment of SRC, with an emphasis on recent insights and developments.[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • Z CHEN, Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma), Alimentary Pharmacology & Therapeutics, 23, 6, (691-712), (2006). I. MARIE, P. DUCROTTE, P. DENIS, M. F. HELLOT and H.[doi.org]
  • Systemic scleroderma: clinical and pathophysiologic aspects. Journal of the American Academy of Dermatology 18: 457–481, 1988 PubMed CrossRef Google Scholar Mascaro G, Cardario G, Bordin G, Tarditi M, Ferraris G, et al.[doi.org]
  • Tyrosine kinases are involved in transforming growth factor-beta and platelet-derived growth factor, which play a central role in the pathophysiology of systemic sclerosis.[medlink.com]
  • Fenves, Recent Developments in the Classification, Evaluation, Pathophysiology, and Management of Scleroderma Renal Crisis, Current Rheumatology Reports, 10.1007/s11926-015-0551-y, 18, 1, (2015).[doi.org]
  • Newer therapies based on the pathophysiologic mechanisms underlying the development and perpetuation of SRC are needed.[doi.org]

Prevention

  • This report emphasizes the possibility that the correction of malignant hypertension may prevent progressive renal falure in some patients affected by diffuse scleroderma.[ncbi.nlm.nih.gov]
  • […] of Raynaud's Phenomenon Unknown status NCT00577304 Phase 3 Nitroglycerin;Topical AmphiMatrix 22 Study of a Topical Formulation for the Treatment and Prevention of Raynaud's Phenomenon Symptoms Unknown status NCT00934427 Phase 3 0.9% nitroglycerin in[malacards.org]
  • For those at risk, avoidance of GBC exposure, whenever possible, is absolutely essential to prevent this potentially devastating complication.[doi.org]
  • […] subset of patients with diffuse cutaneous systemic sclerosis (defined as widespread skin involvement) and internal organ involvement and has a mortality of 30% to 50% at 5 years. 1 Conventional-dose immunosuppressive therapy has limited efficacy in preventing[doi.org]
  • Mouth care and proper brushing and flossing, as well as periodic dental checks, are necessary to prevent cavities and periodontal disease.[medical-dictionary.thefreedictionary.com]

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