- Clinical Treatment Options in Scleroderma: Recommendations and Comprehensive Review. [Review]Clin Rev Allergy Immunol. 2021 Jan 15 [Online ahead of print]CR
- 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…
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- Systemic sclerosis: Update for oral health care providers. [Review]Spec Care Dentist. 2020 Sep; 40(5):418-430.SC
- 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 …
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- Serum defensin levels in patients with systemic sclerosis. [Journal Article]Adv Rheumatol. 2020 12 22; 60(1):54.AR
- CONCLUSIONS: Alpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease.
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- BIOMARKERS OF DISEASE ACTIVITY IN SYSTEMIC SCLEROSIS. [Journal Article]Wiad Lek. 2020; 73(10):2300-2305.WL
- 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]EJ
- 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…
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- Hematological Manifestations among Patients with Rheumatic Diseases. [Review]Acta Haematol. 2020 Nov 20 [Online ahead of print]AH
- 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.
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- Management of challenging radioiodine treatment protocols: a case series and review of the literature. [Journal Article]J Nucl Med Technol. 2020 Nov 20 [Online ahead of print]JN
- CONCLUSIONS: These cases highlight unique challenges that can be encountered during radioactive iodine administration and approaches that can overcome these challenges. We conclude that provider collaboration and treatment customization are critical to overcome patient-specific challenges.
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- Genomics of Human Fibrotic Diseases: Disordered Wound Healing Response. [Review]Int J Mol Sci. 2020 Nov 14; 21(22)IJ
- 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…
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- Anti-PM/Scl Antibody-positive Systemic Sclerosis Complicated by Multiple Organ Involvement. [Journal Article]Intern Med. 2020 Nov 02 [Online ahead of print]IM
- 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 …
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- Systemic sclerosis in pregnancy. [Review]Obstet Med. 2020 Sep; 13(3):105-111.OM
- 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…
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- Hydroxychloroquine and maintenance immunosuppression use in kidney transplant recipients: Analysis of linked US registry and claims data. [Journal Article]Clin Transplant. 2020 12; 34(12):e14118.CT
- Hydroxychloroquine (HCQ) is an antimalarial drug with immunomodulatory effects used to treat systemic lupus erythematosus (SLE) and scleroderma. The antiviral effects of HCQ have raised attention in the context of the COVID-19 pandemic, although safety is controversial. We examined linkages of national transplant registry data with pharmaceutical claims and Medicare billing claims to study HCQ us…
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- Scleroderma-associated thrombotic microangiopathy in overlap syndrome of systemic sclerosis and systemic lupus erythematosus: A case report and literature review. [Case Reports]Medicine (Baltimore). 2020 Oct 09; 99(41):e22582.M
- CONCLUSIONS: The occurrence of TMA leads to the deterioration of the prognosis of SSC-SLE overlap syndrome. The diagnosis of SSC-TMA in SSc-SLE overlap syndrome depends on clinical laboratory examination and renal histopathology.
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- Mycophenolate mofetil for systemic sclerosis: drug exposure exhibits considerable inter-individual variation-a prospective, observational study. [Journal Article]Arthritis Res Ther. 2020 10 06; 22(1):230.AR
- CONCLUSIONS: MMF-treated SSc patients exhibit considerable inter-individual variation in drug exposure, and lower MPA levels were primarily found in PPI users with poor prognostic factors. Body weight, renal function, sex, serology, gastrointestinal manifestations and/or measuring individual MPA exposure should be considered when using MMF for SSc.
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- Hypertensive Emergency in a Woman with Systemic Sclerosis. [Case Reports]High Blood Press Cardiovasc Prev. 2020 Dec; 27(6):597-599.HB
- 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…
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- Recognising the spectrum of scleromyositis: HEp-2 ANA patterns allow identification of a novel clinical subset with anti-SMN autoantibodies. [Journal Article]RMD Open. 2020 09; 6(2)RO
- CONCLUSIONS: SSc skin involvement is often absent in early seronegative scleromyositis. ANA positivity, Raynaud phenomenon, SSc-type capillaroscopy and/or lower oesophagal dysmotility may be clues for scleromyositis. Using HEp-2 IIF patterns, three novel clinicoserological subsets of scleromyositis emerged, notably (1) ANA-negative, (2) ANA-positive with a speckled pattern and (3) ANA-positive with nuclear dots and anti-SMN aAbs.
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- Reduced ventilatory efficiency during exercise predicts major vascular complications and mortality for interstitial lung disease in systemic sclerosis. [Journal Article]Clin Exp Rheumatol. 2020 May-Jun; 38 Suppl 125(3):85-91.CE
- CONCLUSIONS: V' E/V' CO2 slope is a prognostic marker of MVC and five-year mortality for ILD.
- Association of Anti-Topoisomerase I Antibodies of the IgM Isotype With Disease Progression in Anti-Topoisomerase I-Positive Systemic Sclerosis. [Journal Article]Arthritis Rheumatol. 2020 11; 72(11):1897-1904.AR
- CONCLUSIONS: In SSc patients who were anti-topo I IgG-positive, presence of anti-topo I IgM, which might be considered as a surrogate for an ongoing autoreactive B cell immune response, is associated with disease progression.
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- The Pathogenesis of Systemic Sclerosis: An Understanding Based on a Common Pathologic Cascade across Multiple Organs and Additional Organ-Specific Pathologies. [Review]J Clin Med. 2020 Aug 19; 9(9)JC
- Systemic sclerosis (SSc) is a multisystem autoimmune and vascular disease resulting in fibrosis of various organs with unknown etiology. Accumulating evidence suggests that a common pathologic cascade across multiple organs and additional organ-specific pathologies underpin SSc development. The common pathologic cascade starts with vascular injury due to autoimmune attacks and unknown environment…
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- The role of the renin-angiotensin system in skin physiology and pathophysiology. [Journal Article]Exp Dermatol. 2020 Sep; 29(9):891-901.ED
- 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…
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- Clinical features of Japanese systemic sclerosis (SSc) patients negative for SSc-related autoantibodies: A single-center retrospective study. [Journal Article]Int J Rheum Dis. 2020 Aug; 23(9):1219-1225.IJ
- CONCLUSIONS: Patients negative for ANA/SSc-related autoAbs form a clinically distinct subset among SSc patients.
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- Validity and reliability of the Patient Health Questionnaire-8 in Swedish for individuals with systemic sclerosis. [Journal Article]Rheumatol Int. 2020 Oct; 40(10):1675-1687.RI
- CONCLUSIONS: The PHQ-8 Swe has satisfactory content validity and sufficient reliability in patients with in majority limited SSc. It is more strongly associated with self-reported disability, pain, disease interferences with daily activities, fatigue, and quality of life than with disease severity, except for a moderate association with lung severity.
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- [Thrombotic microangiopathy in rheumatology: a link between thrombosis and autoimmunity]. [Journal Article]Ter Arkh. 2020 Jun 05; 92(5):4-14.TA
- 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…
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- Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma. [Review]Arthritis Rheumatol. 2020 09; 72(9):1415-1426.AR
- 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…
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- Renal disorders in rheumatologic diseases: the spectrum is changing (Part 1: connective tissue diseases). [Review]J Nephrol. 2020 Jun 11 [Online ahead of print]JN
- 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…
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- Renal Involvement in Systemic Sclerosis: An Update. [Review]Kidney Blood Press Res. 2020; 45(4):532-548.KB
- 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.
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- Systemic sclerosis and end-stage renal disease: study of patient characteristics, follow-up and outcomes in France. [Journal Article]J Nephrol. 2020 May 24 [Online ahead of print]JN
- CONCLUSIONS: The prognosis for SSc patients requiring RRT is still poor, with a significantly higher mortality and lower registration on kidney transplant waiting-list compared to matched controls.
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- Reduction of fat free mass index and phase angle is a risk factor for development digital ulcers in systemic sclerosis patients. [Journal Article]Clin Rheumatol. 2020 Dec; 39(12):3693-3700.CR
- CONCLUSIONS: In SSc patients, reduction of the FFMI and PhA represents after 12 months a risk factor for development of new digital ulcers and major vascular complication. Key Points • Fat free mass index represents a risk factor for development of digital ulcers • Phase angle represents a risk factor for development of digital ulcers • Body compositions in systemic sclerosis are a marker of activity disease.
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- Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy. [Journal Article]Dermatol Ther. 2020 07; 33(4):e13641.DT
- 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…
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- Reduced RVSWI Is Associated With Increased Mortality in Connective Tissue Disease Associated Pulmonary Arterial Hypertension. [Journal Article]Front Cardiovasc Med. 2020; 7:77.FC
- 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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- Clinical characteristics and outcomes of 566 Thais with systemic sclerosis: A cohort study. [Journal Article]Int J Rheum Dis. 2020 Jul; 23(7):945-957.IJ
- CONCLUSIONS: dcSSc is a common SSc subset among Thais. PF was an early complication in SSc and earlier PF detection was associated with a poorer prognosis. Elderly onset, high FC, severe skin tightness, and multiple organ involvements were associated with a greater mortality risk.
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