Systemic sclerosis – diagnostic and therapeutic recommendations of the Polish Dermatological Society. Part 2: treatment

Krasowska, Dorota; Rudnicka, Lidia; Dańczak‐Pazdrowska, Aleksandra; Chodorowska, Grażyna; Woźniacka, Anna; Lis‐Święty, Anna; Czuwara, Joanna; Maj, Joanna; Majewski, Sławomir; Sysa-Jędrzejowska, Anna; Wojas‐Pelc, Anna

doi:10.5114/dr.2017.71831

Cited by 7 publications

(5 citation statements)

References 39 publications

(161 reference statements)

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“…Due to ILD, skin or joint involvement, 3 of the 6 patients with calcium deposits had a significant reduction in deposits 6 months after the first treatment cycle. It continued to improve gradually over the following months [21]. There is also a report by Hurabielle et al, in which the researchers describe a case of a woman with deposits in the wrist area.…”

Section: -Intravenous Immunoglobulin (Ivig)mentioning

confidence: 78%

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Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 4. Treatment of patients with systemic sclerosis characteristics and recommendations concerning treatment of skin involvement, Raynaud’s phenomenon, calcinosis

Bultrowicz,

Kopeć-Mędrek,

Gumkowska-Sroka

et al. 2023

Rheumatology Forum

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Systemic sclerosis (SSc) is a systemic connective tissue disease marked by diffuse microangiopathy and excessively immune-stimulated fibroblast activity, leading to fibrosis of the skin and internal organs. In the literature, the first report of the disease dates back to 1753 and is attributed to the physician Carlo Curzio of Naples, who described the case of a 17-year-old girl who developed sclerosis of the skin all over her body. The disease is a rare condition. It is estimated that 1 in 10 000 people in Poland suffer from SSc. Women predominate among the patients, with a 3-4-fold prevalence compared to men. Typically, the disease has its onset between 30 and 50 years of age. Early detection and treatment of organ complications are key to improving quality of life and reducing mortality in patients with SSc. Given the significant variability in the clinical course, an individualised approach to patients and multidisciplinary collaboration appear to be justified, both in the diagnostic and treatment phases. The treatment is based on the organ-specific therapeutic strategy, which involves tailoring the pharmacotherapy to the clinical presentation, disease stage, and organ complications. Treatment of patients should include, in addition to pharmacology, education of the patient and family and, if necessary, surgical treatment or other necessary interventions.

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Section: -Intravenous Immunoglobulin (Ivig)mentioning

confidence: 78%

“…Adverse effects of prostanoids include facial erythema, diarrhoea, headache, drop in blood pressure and skin exanthema. Prostanoids administered orally have proven to be of limited efficacy [21].…”

Section: Vascular Treatmentmentioning

confidence: 99%

Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 4. Treatment of patients with systemic sclerosis characteristics and recommendations concerning treatment of skin involvement, Raynaud’s phenomenon, calcinosis

Bultrowicz,

Kopeć-Mędrek,

Gumkowska-Sroka

et al. 2023

Rheumatology Forum

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“…This is an important consideration as, due to the heterogeneous course of SSc, many different medications are used depending on the nature and degree of internal organ involvement: MTX is the drug of choice in skin and joint manifestations whereas CP is more effective in lung fibrosis and renal involvement. Although our patients' care was chosen according to expert recommendations [68], it is possible that these treatments could affect the findings in our SSc cohort.…”

Section: Discussionmentioning

confidence: 99%

Interplay between IL-17 family members and angiogenic cytokines in subjects with systemic sclerosis.

et al. 2021

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IntroductionSystemic sclerosis (SSc) is a chronic, autoimmune connective tissue disease characterized by immune system activation, vasculopathy, and collagen accumulation. Despite progressive fibrosis of the vasculature, compensatory angiogenesis is impaired. The cause of the shift towards anti-angiogenesis observed in SSc is unknown. The IL-17 cytokine family participates in the pathogenesis of SSc and angiogenesis.Material and methodsOur study aimed to evaluate levels and find relationships between the levels of proangiogenic cytokines and cytokines from the IL-17 family in 42 SSc subjects and 20 healthy controls. VEGF, PlGF, HGF, TGFβ1, GM-CSF, IL-17A, IL-17B, IL-17E and IL-17F were quantified in the sera of all participants by ELISA sandwich kits.ResultsSignificantly higher mean concentrations of PlGF compared to controls - mean value (19.3 pg/ml in the SSc group vs. 11.4 pg/ml in the control group; p<0.001) and of HGF (1931 pg/ml in the SSc group vs. 1483 pg/ml in controls; p<0.05). Mean serum TGFβ1 level was also significantly lower in the SSc group (781 pg/ml) than controls (35991 pg/ml; p<0.001). Among the IL-17 family, significantly higher mean concentrations of IL-17B (67.0 pg/ml vs. 2.6 pg/ml in controls; p<0,001), IL-17E (8.0 pg/ml vs 0.64 pg/ml in controls; p<0.001) and IL-17F (0.42 pg/ml vs. 0.0 pg/ml in controls; p< 0.01) were detected. Serum concentrations of HGF and PlGF correlated with the concentrations of IL17A, IL-17B, and IL-17E.ConclusionsIn conclusion, our findings indicate that selected cytokines from the IL17 family participate in the pathogenesis of SSc and are responsible for the vascular abnormalities associated with this disorder.

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“…W przypadku zaburzonej integralności śródbłonka efekt naczyniorozszerzający substancji P będzie znacznie zmniejszony [50]. Za relaksację mięśni gładkich odpowiedzialne są wewnątrz-Dermatology Review/Przegląd Dermatologiczny 2019/1 alternative and supportive form of treating RP [53]. In SSc patients with secondary microcirculatory disorders and intolerance and contraindication for prostanoids, Walecka et al used sulodexide administered IV twice a day in the dose of 600 LSU with good therapeutic results.…”

Section: Zmiany Naczyniowe W Przebiegu Chorób Autoimmunologicznych Tkunclassified

“…Kolejną opcję terapeutyczną stanowią inhibitory fosfodiesterazy 5, w tym sildenafil, iloprost i alprostadyl. Wśród alternatywnych i wspomagających form terapii RP wymienia się również sulodeksyd [53]. U chorych z zaburzeniami w mikrokrążeniu w przebiegu SSs w przypadku nietoleracji lub przeciwwskazań do terapii prostanoidami Walecka i wsp.…”

Section: Zmiany Naczyniowe W Przebiegu Chorób Autoimmunologicznych Tkunclassified

Sulodexide – unrealized potential in dermatology

Krasowska¹,

Polkowska-Pruszyńska²,

Michalak-Stoma³

2019

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Sulodexide is a highly purified mixture of glycosaminoglycans composed of fast mobility heparin (80%) and dermatan sulfate (20%). Glycosaminoglycans constitute a group of heteropolysaccharides consisting of repeating disaccharide units, which contain amino sugar combined with uronic acid or a hexose. Glycosaminoglycans build glycocalyx covering endothelium of blood vessels, and thus, play an important role in vascular homeostasis. Multi-directional activity of sulodexide including antithrombotic, fibrinolytic, anti-aggregation, anti-inflammatory, anti-proliferative, and anti-oxidative qualities, as well as a high safety profile have allowed using the drug in numerous entities. Advantageous results of sulodexide therapy are observed in diseases involving endothelial damage, prothrombotic or inflammatory states. Thanks to its pleiotropic effects, sulodexide can be also applied in treatment of skin diseases. streszczenie Sulodeksyd to wysoce oczyszczona mieszanina glikozaminoglikanów złożona w 80% z heparyny szybko przemieszczającej się w polu elektroforezy oraz w 20% z siarczanu dermatanu. Glikozaminoglikany są grupą heteropolisacharydów składających się z powtarzających się jednostek disacharydowych, zawierających aminocukier połączony z kwasem uronowym lub heksozą. Glikozaminoglikany stanowią budulec glikokaliksu pokrywającego śródbłonek naczyń krwionośnych i odgrywają istotną rolę w homeostazie naczyniowej. Wielokierunkowe działanie sulodeksydu obejmujące właściwości przeciwzakrzepowe, fibrynolityczne, antyagregacyjne, przeciwzapalne, antyproliferacyjne i antyoksydacyjne oraz wysoki profil bezpieczeństwa umożliwiły zastosowanie leku w wielu jednostkach chorobowych. Korzystne efekty terapii sulodeksydem obserwuje się w chorobach przebiegających z uszkodzeniem śródbłonka, ze stanami prozakrzepowymi i zapalnymi. Dzięki właściwościom plejotropowym sulodeksyd znalazł również zastosowanie w terapii chorób skóry.

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Systemic sclerosis – diagnostic and therapeutic recommendations of the Polish Dermatological Society. Part 2: treatment

Cited by 7 publications

References 39 publications

Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 4. Treatment of patients with systemic sclerosis characteristics and recommendations concerning treatment of skin involvement, Raynaud’s phenomenon, calcinosis

Everything you always wanted to know about systemic sclerosis but were afraid to ask: Part 4. Treatment of patients with systemic sclerosis characteristics and recommendations concerning treatment of skin involvement, Raynaud’s phenomenon, calcinosis

Interplay between IL-17 family members and angiogenic cytokines in subjects with systemic sclerosis.

Sulodexide – unrealized potential in dermatology

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