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Scleroderma Renal Disease


Presentation

  • A patient with scleroderma renal disease and pulmonary hypertension who had a successful pregnancy with the use of angiotensin converting enzyme inhibitors is presented.[ncbi.nlm.nih.gov]
  • There is no evidence at present to support the use of angiotensin-converting enzyme inhibitors prophylactically. The mechanisms and significance of chronic renal impairment in scleroderma need to be better defined.[ncbi.nlm.nih.gov]
  • More than 700 full-color high-quality photographs as well as carefully chosen figures, algorithms, and tables to illustrate essential concepts, nuances of clinical presentation and technique, and decision making provide a visual grasp and better understanding[books.google.com]
  • Kidney function abnormalities or proteinuria were present in 173 (26%); 48% had no abnormalities. Most patients had other explanations for the abnormality.[jrheum.org]
  • The patient again presented in August 2010 with complaints of rapidly progressive shortness of breath with orthopnea, generalized swelling and reduced urine output of one week's duration.[sjkdt.org]
Orthopnea
  • Three days before presentation, she had been admitted to a local hospital because of orthopnea.[jstage.jst.go.jp]
  • The patient again presented in August 2010 with complaints of rapidly progressive shortness of breath with orthopnea, generalized swelling and reduced urine output of one week's duration.[sjkdt.org]
Pleural Effusion
  • Examination of the chest showed evidence of pleural effusion on the left side. His muscle power was 4/5 in the proximal upper and lower limbs, with neck flexor and truncal weakness.[sjkdt.org]
Hypertension
  • A patient with scleroderma renal disease and pulmonary hypertension who had a successful pregnancy with the use of angiotensin converting enzyme inhibitors is presented.[ncbi.nlm.nih.gov]
  • […] of essential (volatile) oils and without any antecedent factors. [9], [10] The patient in our report developed accelerated hypertension and acute renal insufficiency.[sjkdt.org]
  • A Atchley and others studied the effects of hypertension, endocarditis, and circulatory diseases on the kidney and few years ago we never heard of kidney disease and now you are speaking of patients in the hundreds of thou spawned successive generations[books.google.com]
  • – a sudden onset of malignant hypertension and renal failure that occurs in a minority of scleroderma patients.[ucsfmed.wordpress.com]
  • […] hemolytic anemia; or (b) slowly progressive chronic form with proteinuria, hypertension and azotemia Scleroderma renal crisis: malignant hypertension and oligo-anuric acute renal failure; occurs in 2 - 5%, particularly those with diffuse cutaneous systemic[pathologyoutlines.com]
Proximal Muscle Weakness
  • The patient being reported is a 21-year-old male who presented in the Immunology Outpatient Department in February 2010 with complaints of arthritis involving small as well as large joints, low-grade fever, on and off, puffy fingers and proximal muscle[sjkdt.org]
Neurologic Manifestation
  • After system… PMC Free PDF PMC Free Full Text FREE Publisher Full Text Polyarteritis Nodosa Neurologic Manifestations.[unboundmedicine.com]

Workup

Creatinine Increased
  • Creatinine test As kidney function declines, your creatinine increases. This protein is also related to muscle mass. Parathyroid hormone (PTH) test The kidney and the parathyroid glands interact through the regulation of calcium and phosphorus.[healthline.com]
Pleural Effusion
  • Examination of the chest showed evidence of pleural effusion on the left side. His muscle power was 4/5 in the proximal upper and lower limbs, with neck flexor and truncal weakness.[sjkdt.org]

Treatment

  • 545 Dialysis ultrafiltration and hemofiltration 555 Use of drugs in uremia and dialysis 567 Donor and recipient selection in renal transplantation 587 Immunosuppression and treatment of rejection in renal transplantation 595 XIII 606 Renal tubular and[books.google.com]
  • The patient was diagnosed as a case of MCTD and advised treatment. However, he refused any treatment and took some alternative medicine for six months.[sjkdt.org]
  • For both, completely effective treatment is not available, mainly as a result of the lack of knowledge of its pathogenesis.[academy.theisn.org]
  • What is the treatment? Treatments for scleroderma vary according to the problems you have. Various drugs (as well as warm gloves) may help Raynaud’s syndrome, for instance.[edren.org]
  • Because SRC most commonly stems from the progression of an existing diffuse disease state, the treatment of both is required, although these treatments are not inherently identical. Treatment Angiotensin-converting enzyme inhibitors (ACEIs).[consultant360.com]

Prognosis

  • The patient was discharged on his request and the poor prognosis was explained.[sjkdt.org]
  • However, it can help to define prognosis and guide treatment in atypical forms. The prognosis of SRC has dramatically improved with the introduction of angiotensin-converting enzyme inhibitors (ACEi).[ncbi.nlm.nih.gov]
  • Nevertheless, the prognosis is still not good. Survival at one and five years is only 76% and 65%, respectively, for SRC patients treated with ACE inhibitors.[the-rheumatologist.org]
  • Blood tests to look for certain antibodies (for instance anti-Scl-70 antibodies, anti-centromere antibodies) may help, and can help predict prognosis.[edren.org]
  • No medications appear to be truly effective in patients with aggressive disease and long-term prognosis remains disappointing [ 9 ].[clinmedjournals.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.[jrheum.org]
  • Definition / general Connective tissue disorder of unknown etiology with multiple organ system involvement, symptoms due to excessive collagen deposition and vascular disease Also called scleroderma Clinical features 75% women, usually ages 30 , rare[pathologyoutlines.com]
  • Prophylactic use of ACE inhibitors does not seem to decrease the risk of SRC (observed in other studies) so it may be the changing etiology of SSc over time. Disclosure: M. Turk, None; J. E. Pope, None.[acrabstracts.org]
  • It has been suggested that the RAAS may be less activated, leading to modest blood pressure (BP) elevation, 2 and that vascular insult and vasoconstriction are the culprits, rather than a renin-mediation etiology.[consultant360.com]
  • […] frequently by patients with gastritis gastric ulcer Common Causes of Acute Abdominal Pain Upper abdominal pain Acute cholecystitis Acute pancreatitis Every now and againI get a very sharp pain just above and to the right of my belly button What is the etiology[banksuenden.eu]

Epidemiology

  • Pathogenesis – thought to involve small interlobular arteries in the kidneys which develop intimal proliferation and spasm à vasculopathy that reduces renal blood flow à activation or RAAS à hypertension and worsened renal dysfunction Epidemiology and[ucsfmed.wordpress.com]
  • The serum creatinine was 831μmol/l equivalent of an estimated glomerular filtration rate 2 ml/min/1.73 m 2 according to chronic kidney disease epidemiology; the urea was 38 mg/dl. Proteinuria was 0.8 g/24 hours.[omicsonline.org]
  • Epidemiology of the Rheumatic Diseases. Oxford, U.K.: Oxford University Press; 1993:192. ... 2. Lawrence RC, Helmick CG, Arnett FC, et al. Estimates of the prevalence of arthritis and selected musculoskeletal disorders in the United States.[aafp.org]
  • Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers. Curr. Opin. Rheumatol. 24, 165–170 (2012). 4. Steen, V. D., Syzd, A., Johnson, J. P., Greenberg, A. & Medsger, T. A. Jr.[nature.com]
  • Epidemiology of systemic sclerosis: incidence, prevalence, survival, risk factors, malignancy, and environmental triggers . Curr. Opin. Rheumatol. 24 , 165–170 (2012). 4. Steen, V. D. , Syzd, A. , Johnson, J. P. , Greenberg, A. & Medsger, T. A. Jr.[nature.com]
Sex distribution
Age distribution

Pathophysiology

  • From basic science and pathophysiology to clinical best practices, Brenner & Rector’s The Kidney is your go-to resource for any stage of your career.[books.google.com]
  • Pathology The pathophysiology is very similar to malignant nephrosclerosis and is characterized by pronounced constriction of arcuate and interlobular arterioles resulting in focal cortical ischemia.[radiopaedia.org]
  • According 2013 American Society for Apheresis guidelines pathophysiology of SSc lends little support to the use of Therapeutic Plasma Exchange [ 10 ].[clinmedjournals.org]
  • "Systematic review: pathophysiology and management of gastrointestinal dysmotility in systemic sclerosis (scleroderma)". Aliment. Pharmacol. Ther. 23 (6): 691–712. doi : 10.1111/j.1365-2036.2006.02804.x.[en.wikipedia.org]
  • Pathology Although the pathophysiology underlying SRC is not completely understood, it is accepted as being related to intimal thickening of the renal interlobular and arcuate arteries as a result of endothelial cell injury and abnormal oxidative stress[consultant360.com]

Prevention

  • Doctors recommend that you get the following vaccinations: pneumococcal vaccine hepatitis B vaccine influenza vaccine H1N1 (swine flu) vaccine You can’t always prevent CKD.[healthline.com]
  • Interventions designed for Raynaud’s—protecting fingers and toes from trauma and cold temperatures, and staying warm—may prevent damage.[pharmacytimes.com]
  • Prevention No preventive measures have been validated in the literature to support predictable, reliable improvement in outcomes.[consultant360.com]
  • There is limited evidence for treatments to prevent this damage or stop it progressing. Blocking a substance in the blood called endothelin has helped treat some aspects of scleroderma.[clinicaltrials.gov]
  • To prevent death and serious organ damage TTP requires immediate treatment with plasma exchange (PLEX) – see Figure 5 for a diagram.[unckidneycenter.org]

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