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Unbound Medicine.
(Scleroderma Renal Disease)
1,759 results
  • Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review. [Review]
    Clin Rev Allergy Immunol. 2021 Jan 15 [Online ahead of print]Zhao M, Wu J, … Lu Q
  • There are two major clinical subsets of scleroderma: (i) systemic sclerosis (SSc) is a complex systemic autoimmune disorder characterized by inflammation, vasculopathy, and excessive fibrosis of the skin and multiple internal organs and (ii) localized scleroderma (LoS), also known as morphea, is confined to the skin and/or subcutaneous tissues resulting in collagen deposition and subsequent fibro…
  • Systemic sclerosis: Update for oral health care providers. [Review]
    Spec Care Dentist. 2020 Sep; 40(5):418-430.Alhendi FJ, Werth VP, … Stoopler ET
  • Systemic sclerosis (SSc), also known as scleroderma, is an autoimmune disease of unknown origin characterized by an uncontrolled inflammatory process resulting in fibrosis of the skin, internal organs and vasculopathy. Manifestations of SSc are heterogenous and can include pulmonary, cardiac, neural, renal, muscular, cutaneous and orofacial complications. Recent scientific advances have led to a …
  • BIOMARKERS OF DISEASE ACTIVITY IN SYSTEMIC SCLEROSIS. [Journal Article]
    Wiad Lek. 2020; 73(10):2300-2305.Pawlik KK, Bohdziewicz A, … Rudnicka L
  • Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy and uncontrolled cutaneous and internal organs fibrosis. Diagnosis of SSc in an early phase can be difficult because of a lack of typical symptoms. The delay in diagnosis and treatment of SSc may lead to uncontrolled progression of the disease, thus identification of possible early indicators of skin and organ involve…
  • Scleroderma renal crisis: Case reports and update on critical issues. [Review]
    Eur J Rheumatol. 2020 Nov 19 [Online ahead of print]Zanatta E, Codullo V, Allanore Y
  • To date, scleroderma renal crisis (SRC) remains a life-threatening complication in patients affected by systemic sclerosis (SSc), with high morbidity and mortality. In the last few years, some studies have tried to more precisely identify predictors of SRC and clarify the role of previous drug exposure-in particular, angiotensin-converting enzyme (ACE) inhibitors and corticosteroids-in patients w…
  • Hematological Manifestations among Patients with Rheumatic Diseases. [Review]
    Acta Haematol. 2020 Nov 20 [Online ahead of print]Klein A, Molad Y
  • CONCLUSIONS: The aim of this review was to discuss cytopenias caused by systemic lupus erythematosus and antirheumatic drugs, Felty's syndrome in rheumatoid arthritis, and autoimmune hemolytic anemia, thrombosis, and thrombotic microangiopathies related to rheumatological conditions such as catastrophic antiphospholipid syndrome and scleroderma renal crisis. Key Message: The differential diagnosis of various hematological disorders should include rheumatic autoimmune diseases among other causes of blood cell and hemostasis abnormalities. It is crucial that hematologists be aware of these presentations so that they are diagnosed and treated in a timely manner.
  • Genomics of Human Fibrotic Diseases: Disordered Wound Healing Response. [Review]
    Int J Mol Sci. 2020 Nov 14; 21(22)Stone RC, Chen V, … Tomic-Canic M
  • Fibrotic disease, which is implicated in almost half of all deaths worldwide, is the result of an uncontrolled wound healing response to injury in which tissue is replaced by deposition of excess extracellular matrix, leading to fibrosis and loss of organ function. A plethora of genome-wide association studies, microarrays, exome sequencing studies, DNA methylation arrays, next-generation sequenc…
  • Anti-PM/Scl Antibody-positive Systemic Sclerosis Complicated by Multiple Organ Involvement. [Journal Article]
    Intern Med. 2020 Nov 02 [Online ahead of print]Shimizu T, Saito C, … Yamagata K
  • A 40-year-old Japanese woman developed malignant-phase hypertension complicated by thrombotic microangiopathy, progressing to end-stage renal disease. Five years later, she was diagnosed with pulmonary arterial hypertension and interstitial pneumonia. Despite a lack of overt skin sclerosis, nucleolar staining in our indirect immunofluorescence analysis and nailfold capillaroscopy facilitated the …
  • Systemic sclerosis in pregnancy. [Review]
    Obstet Med. 2020 Sep; 13(3):105-111.Clark KE, Etomi O, Ong VH
  • Systemic sclerosis is a rare multisystem connective tissue disease. It predominantly affects women and poses a significant risk to mother and baby during pregnancy if not managed appropriately. The commonest manifestations are skin fibrosis and Raynaud's phenomenon. Subgroups of women have an increased risk of organ involvement, especially interstitial lung disease, pulmonary arterial hypertensio…
  • Hypertensive Emergency in a Woman with Systemic Sclerosis. [Case Reports]
    High Blood Press Cardiovasc Prev. 2020 Dec; 27(6):597-599.Sauza-Sosa JC, Zenteno-Langle R, Zamora-Medina MDC
  • Systemic sclerosis (SSc) is a rare autoimmune disease that causes fibrosis in the skin and subcutaneous tissue, involving other organs such as the heart, lungs, kidneys, and gastrointestinal tract. Additionally, it can cause pulmonary arterial hypertension. Scleroderma renal crisis (SRC) is one of the most dreadful complications of SSc. SRC is a medical emergency that can present as a clinical pi…
  • The role of the renin-angiotensin system in skin physiology and pathophysiology. [Journal Article]
    Exp Dermatol. 2020 Sep; 29(9):891-901.Silva IMS, Assersen KB, … Steckelings UM
  • Since its first description around the year 2000, the local renin-angiotensin system (RAS) in skin has been subject of an increasing number of studies with many additions over the last two to three years. A focus of research has been investigations on the role of cutaneous angiotensin receptors and locally synthesised angiotensin II in wound healing, in dermatoses associated with skin fibrosis an…
  • [Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity]. [Journal Article]
    Ter Arkh. 2020 Jun 05; 92(5):4-14.Nasonov EL, Reshetnyak TM, Alekberova ZS
  • Uncontrolled hypercoagulation and inflammation (thromboinflammation), which are both independent and closely related and amplifying each other pathological processes, form the basis for pathogenesis of a wide range of diseases and complications, including immuno-inflammatory (autoimmune) rheumatic diseases, with the development of potentially fatal injuries of internal organs. Thrombotic microang…
  • Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma. [Review]
    Arthritis Rheumatol. 2020 09; 72(9):1415-1426.Simms RW
  • You are consulted to evaluate a 56-year-old woman with known Raynaud's phenomenon, finger swelling of several; months' duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles…
  • Renal disorders in rheumatologic diseases: the spectrum is changing (Part 1: connective tissue diseases). [Review]
    J Nephrol. 2020 Jun 11 [Online ahead of print]Ponticelli C, Doria A, Moroni G
  • The kidney is frequently involved by autoimmune rheumatic diseases. The renal manifestations may be variable, ranging from asymptomatic proteinuria and microscopic haematuria to nephrotic syndrome and rapidly progressive glomerulonephritis or vasculitis. In a number of cases the kidney involvement is related to the treatment of the original disease and may represent a major cause of morbidity and…
  • Renal Involvement in Systemic Sclerosis: An Update. [Review]
    Kidney Blood Press Res. 2020; 45(4):532-548.Chrabaszcz M, Małyszko J, … Rudnicka L
  • CONCLUSIONS: Renal disease occurring in patients with systemic sclerosis may have a variable clinicopathological picture. The most specific renal condition associated with systemic sclerosis is scleroderma renal crisis, characterized by acute onset of renal failure and severe hypertension. Although the management of scleroderma renal crisis was revolutionized by the introduction of angiotensin-converting enzyme inhibitors, there is still a significant proportion of patients with poor outcomes. Therefore, research on establishing disease markers (clinical, ultrasonographical and serological) and clear diagnostic criteria, which could limit the risk of developing scleroderma renal crisis and facilitate diagnosis of this complication, is ongoing. Other forms of renal involvement in systemic sclerosis include vasculitis, an isolated reduced glomerular filtration rate in systemic sclerosis, antiphospholipid-associated nephropathy, high intrarenal arterial stiffness and proteinuria. Key Messages: Scleroderma renal crisis is the most specific and life-threatening renal presentation of systemic sclerosis, albeit with declining prevalence. In patients with scleroderma renal crisis, it is mandatory to control blood pressure early with increasing doses of angiotensin-converting enzyme inhibitors, along with other antihypertensive drugs if necessary. There is a strong association between renal involvement and patients' outcomes in systemic sclerosis; consequently, it becomes mandatory to find markers that may be used to identify patients with an especially high risk of scleroderma renal crisis.
  • Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy. [Journal Article]
    Dermatol Ther. 2020 07; 33(4):e13641.Luo G, Wan Y, … Deng X
  • Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by increased peripheral blood eosinophils and diffuse fasciitis, generalized morphea (GM) is a subtype of localized scleroderma, and IgA nephropathy is a chronic glomerulonephritis caused by abnormal deposition of IgA in the mesangial area of the glomeruli. We describe a 49-year-old male patient with hard skin, cutaneou…
  • Reduced RVSWI Is Associated With Increased Mortality in Connective Tissue Disease Associated Pulmonary Arterial Hypertension. [Journal Article]
    Front Cardiovasc Med. 2020; 7:77.Clapham KR, Highland KB, … Fares WH
  • CONCLUSIONS: Right ventricular dysfunction as measured by right ventricular stroke work index is associated with decreased survival in patients with connective tissue disease associated pulmonary arterial hypertension despite similar pulmonary vascular resistance. These findings are suggestive of intrinsic right ventricular function in connective tissue disease associated pulmonary arterial hypertension that has a negative impact on the long-term survival of these individuals.
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