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1.
J Eur Acad Dermatol Venereol ; 38(7): 1251-1280, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38456584

RESUMO

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this consensus provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes.


Assuntos
Consenso , Esclerodermia Localizada , Escleroderma Sistêmico , Humanos , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/terapia , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Diagnóstico Diferencial
2.
Dermatologie (Heidelb) ; 75(3): 181-196, 2024 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-38366245

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is a very heterogeneous, chronic, rare, but socioeconomically important disease with a severe disease course and severe impairment of the quality of life of affected patients. OBJECTIVES: Overview of the current state of research on the pathogenesis, diagnosis and therapy of SSc. METHODS: A literature search was performed. RESULTS: The pathogenesis of SSc is not fully understood. ACR/EULAR criteria allow the diagnosis of early forms of SSc. Classification into limited cutaneous SSc and diffuse cutaneous SSc is of prognostic and therapeutic relevance. New organ-specific treatment options for SSc have led to improved quality of life and prognosis.


Assuntos
Esclerodermia Difusa , Escleroderma Sistêmico , Humanos , Qualidade de Vida , Escleroderma Sistêmico/diagnóstico , Prognóstico , Progressão da Doença
4.
Z Rheumatol ; 79(10): 1057-1066, 2020 Dec.
Artigo em Alemão | MEDLINE | ID: mdl-32040755

RESUMO

BACKGROUND: Raynaud's phenomenon and the frequently ensuing digital ulcerations represent an early and very distressing symptom in patients with systemic sclerosis (scleroderma, SSc) causing significant limitations in the ability to work and quality of life. The use of vasoactive drugs (especially intravenous prostacyclin derivatives) is recommended to reduce the risk of hypoxic tissue damage up to the loss of fingers. METHODS: In order to obtain information about the current state of treatment of patients with prostacyclin derivatives in routine clinical life in Germany, a survey was conducted among the centers affiliated to the German Network for Systemic Scleroderma (DNSS). In addition, a separate patient survey was conducted by the schleroderma self-help group (Sklerodermie Selbsthilfe e. V.), which only covered the symptoms Raynaud's syndrome, digital ulcers and the use of intravenous prostacyclin derivatives. RESULTS: Of the 433 patients surveyed 56% stated that they had already been treated with prostacyclin derivatives (iloprost/alprostadil) because of their illness and symptoms. A total of 61% received the treatment for severe Raynaud's phenomenon and 39% for digital ulcerations. Most respondents not only experienced an improvement in Raynaud's phenomenon and digital ulcers but also a significant improvement of limitations in everyday life. They also needed significantly less outside help and absenteeism from work was much lower. CONCLUSION: Patients consistently reported a positive effect of treatment with prostacyclin derivatives on Raynaud's phenomenon, acral ulcerations, pain and daily restrictions and felt well and safely cared for during inpatient treatment. These positive effects in the patients' perceptions provide crucial information supporting and confirming the current European and international treatment recommendations.


Assuntos
Epoprostenol , Doença de Raynaud , Escleroderma Sistêmico , Epoprostenol/análogos & derivados , Epoprostenol/uso terapêutico , Dedos/irrigação sanguínea , Alemanha , Humanos , Pacientes Internados , Qualidade de Vida , Doença de Raynaud/diagnóstico , Doença de Raynaud/tratamento farmacológico , Doença de Raynaud/epidemiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/tratamento farmacológico , Pele/irrigação sanguínea
6.
Arthritis Care Res (Hoboken) ; 72(8): 1041-1048, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-31150152

RESUMO

OBJECTIVE: To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. METHODS: The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). RESULTS: Overall, 37.4% of patients (1,590 of 4,252) received PT at the end of a yearly follow-up. The most frequently used type of PT was lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms of PT simultaneously. The prescription of PT was associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients]; P < 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receiving PT than patients without these symptoms, with corresponding ORs ranging from 1.96 (95% confidence interval [95% CI] 1.69-2.28) for joint contractures to 3.83 (95% CI 2.89-5.08) for arthritis. When comparing the type of PT prescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receiving PT could be observed (P = 0.001). CONCLUSION: To our knowledge, this is the first study reporting the use of PT in patients with systemic sclerosis (SSc) in a large cohort. Although SSc is characterized by considerable disability and restriction of motion, <40% of patients received PT.


Assuntos
Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Modalidades de Fisioterapia/estatística & dados numéricos , Escleroderma Sistêmico/terapia , Índice de Gravidade de Doença , Distribuição de Qui-Quadrado , Estudos de Coortes , Avaliação da Deficiência , Feminino , Alemanha , Humanos , Masculino , Pessoa de Meia-Idade , Razão de Chances , Sistema de Registros , Escleroderma Sistêmico/patologia
7.
Z Rheumatol ; 79(3): 294-303, 2020 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-31754786

RESUMO

BACKGROUND: Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies. MATERIAL AND METHODS: This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords "SSc" and "treatment". RESULTS: The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy. CONCLUSION: Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.


Assuntos
Transplante de Células-Tronco Hematopoéticas , Imunossupressores/uso terapêutico , Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Doenças do Tecido Conjuntivo , Ciclofosfamida , Humanos , Doenças Pulmonares Intersticiais/imunologia , Doenças Pulmonares Intersticiais/terapia , Escleroderma Sistêmico/imunologia , Escleroderma Sistêmico/terapia
8.
Hautarzt ; 69(11): 882-884, 2018 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-30374906
9.
Hautarzt ; 69(11): 892-900, 2018 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-30255259

RESUMO

Localized scleroderma (LoS) is a very heterogeneous connective tissue disease characterized by progressive sclerosis of the skin with possible involvement of extracutaneous structures. Both children and adults can be affected but show different frequencies for the individual subtypes of the disease. The clinical heterogeneity has already caused several modifications of existing classification criteria. Patients suffering from LoS can essentially be subdivided into five different subsets, which are defined by the horizontal and vertical extent of the tissue involvement. The quality of life of these patients is significantly impaired depending on the extent of the cutaneous and subcutaneous involvement. A causal treatment does not yet exist; however, patients should be treated with the currently available medications for progressive subtypes during the early phase of inflammation to reduce or avoid severe, cosmetic and functional impairments. Lichen sclerosus (LS) usually affects the genital as well as extragenital skin and both children and adults can be affected. This article focuses on the extragenital LS, which occurs more frequently in adults. The cause of the disease as well as causal treatment strategies are still lacking. Currently, treatment is adapted to the therapeutic strategies for LoS.


Assuntos
Líquen Escleroso e Atrófico , Esclerodermia Localizada , Administração Cutânea , Adulto , Criança , Humanos , Líquen Escleroso e Atrófico/diagnóstico , Líquen Escleroso e Atrófico/terapia , Qualidade de Vida , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/terapia , Pele
11.
Scand J Rheumatol ; 47(1): 62-70, 2018 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-28990485

RESUMO

OBJECTIVES: Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. METHODS: We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. RESULTS: Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5 years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). CONCLUSIONS: We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).


Assuntos
Medição de Risco/métodos , Escleroderma Sistêmico/diagnóstico , Adulto , Estudos Transversais , Progressão da Doença , Feminino , Humanos , Masculino , Prognóstico
12.
J Eur Acad Dermatol Venereol ; 31(10): 1581-1594, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28786499

RESUMO

The term 'sclerosing diseases of the skin' comprises specific dermatological entities which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this guideline provides clinicians with an overview of the diagnosis and treatment of scleromyxedema, scleredema (of Buschke) and nephrogenic systemic sclerosis (nephrogenic fibrosing dermopathy).


Assuntos
Dermopatia Fibrosante Nefrogênica/diagnóstico , Dermopatia Fibrosante Nefrogênica/terapia , Escleredema do Adulto/diagnóstico , Escleredema do Adulto/terapia , Escleromixedema/diagnóstico , Escleromixedema/terapia , Diagnóstico Diferencial , Humanos , Dermopatia Fibrosante Nefrogênica/patologia , Escleredema do Adulto/patologia , Escleromixedema/patologia
13.
J Eur Acad Dermatol Venereol ; 31(9): 1401-1424, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28792092

RESUMO

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present guideline focuses on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, current strategies in the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 1 of this guideline provides clinicians with an overview of the diagnosis and treatment of localized scleroderma (morphea), and systemic sclerosis including overlap syndromes of systemic sclerosis with diseases of the rheumatological spectrum.


Assuntos
Esclerodermia Localizada , Escleroderma Sistêmico , Doenças do Tecido Conjuntivo Indiferenciado , Humanos , Diagnóstico Diferencial , Europa (Continente) , Exame Físico , Prognóstico , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/patologia , Esclerodermia Localizada/terapia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia , Doenças do Tecido Conjuntivo Indiferenciado/diagnóstico , Doenças do Tecido Conjuntivo Indiferenciado/patologia , Doenças do Tecido Conjuntivo Indiferenciado/terapia
14.
Clin Exp Allergy ; 47(11): 1445-1455, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28696503

RESUMO

BACKGROUND: Subcutaneous allergen immunotherapy with grass pollen allergoids has been proven to be effective and safe in the treatment of patients with allergic rhinoconjunctivitis. Based on the extensive cross-reactivity among Pooideae species, it has been suggested that grass pollen extracts could be prepared from a single species, rather than from a multiple species mixture. OBJECTIVE: To find the optimal dose of a Phleum pratense (P. pratense) allergoid preparation and compare its efficacy and safety to a 6-grass pollen allergoid preparation. METHODS: In this double-blind, placebo-controlled study (EudraCT: 2011-000674-58), three doses of P. pratense allergoid (1800 therapeutic units (TU), standard-dose 6000 TU and 18 000 TU) were compared with placebo and the marketed 6-grass pollen allergoid (6000 TU). In a pre-seasonal dosing regimen, 102 patients were randomized to five treatment groups and received nine subcutaneous injections. The primary efficacy endpoint was the change in weal size (late-phase reaction [LPR]) in response to the intracutaneous testing (ICT) before and after treatment, comparing the active allergoids to placebo. Secondary outcomes were the change in Total Nasal Symptom Score (TNSS) assessed in the allergen exposure chamber (AEC), the changes in P. pratense-serum-specific IgG4 and the incidence of adverse events (AEs). RESULTS: All three doses of the P. pratense and the 6-grass pollen allergoid preparations were significantly superior to placebo for the primary outcome, whereas there were no significant differences in the change in TNSS. Compared to the standard-dose, the high-dose of P. pratense did not produce any additional significant benefit, but showed a slight increase in AEs. Yet this increase in AEs was lower than for the 6-grass pollen preparation. CONCLUSIONS & CLINICAL RELEVANCE: The standard-dose of the new P. pratense allergoid was comparable to the marketed 6-grass pollen preparation at equal dose for the parameters measured.


Assuntos
Alérgenos/imunologia , Relação Dose-Resposta Imunológica , Phleum/efeitos adversos , Extratos Vegetais/imunologia , Pólen/imunologia , Rinite Alérgica Sazonal/imunologia , Rinite Alérgica Sazonal/terapia , Adolescente , Adulto , Idoso , Alergoides , Dessensibilização Imunológica/efeitos adversos , Dessensibilização Imunológica/métodos , Feminino , Alemanha , Humanos , Imunoglobulina E/imunologia , Imunoglobulina G/imunologia , Masculino , Pessoa de Meia-Idade , Extratos Vegetais/administração & dosagem , Rinite Alérgica Sazonal/diagnóstico , Imunoterapia Sublingual , Resultado do Tratamento , Adulto Jovem
16.
Hautarzt ; 66(8): 599-603, 2015 Aug.
Artigo em Alemão | MEDLINE | ID: mdl-26115973

RESUMO

Systemic sclerosis (SSc) is a rare, chronic inflammatory autoimmune disease with unknown etiology, which leads to deposition of collagen and extracellular matrix proteins in the skin and affected internal organs. The diagnosis of SSc is based on clinical, serological, and paraclinical examinations. In 2013 new criteria for the classification of systemic sclerosis, which also take early forms of SSc into consideration, were developed. A complete clinical and paraclinical examination is important for the oligosymptomatic early stages and the subsequent disease course of SSc in order to diagnose and timely treat a developing organ involvement.


Assuntos
Doenças Autoimunes/classificação , Doenças Autoimunes/diagnóstico , Dermatologia/normas , Guias de Prática Clínica como Assunto , Escleroderma Sistêmico/classificação , Escleroderma Sistêmico/diagnóstico , Diagnóstico Diferencial , Alemanha , Humanos , Avaliação de Sintomas/normas
17.
18.
Z Rheumatol ; 74(2): 100-3, 2015 Mar.
Artigo em Alemão | MEDLINE | ID: mdl-25805510

RESUMO

Large data bases and the projects arising from them have led to a much improved understanding of systemic sclerosis over the last decade. Serology has developed further so that more autoantibodies are available for routine testing. Capillary microscopy has become standard and relevant progress has also been made in therapy. Many diagnostic terms found in medical documentation do not adequately reflect this progress. The nomenclature is inconsistent and, therefore, confusing. The international classification of diseases (ICD) nomenclature is, from our point of view, also in need of improvement. This article aims to reestablish a common German language standard for systemic sclerosis, which reflects current knowledge and is suitable for implementation in the clinical routine.


Assuntos
Classificação Internacional de Doenças/normas , Reumatologia/normas , Escleroderma Sistêmico/classificação , Escleroderma Sistêmico/diagnóstico , Terminologia como Assunto , Tradução , Alemanha , Guias de Prática Clínica como Assunto
19.
Hautarzt ; 65(11): 944-8, 2014 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-25336296

RESUMO

Digital ulcers (DU's) are one of the main symptoms of systemic scleroderma and occur in approximately 60% of all scleroderma patients. Due to possible complications such as infections, gangrene or amputation, they require regular medical attention and a good wound treatment by doctors and nursing staff. A definition of DU's has not yet been established. In 2009 the European League Against Rheumatism (EULAR) published guidelines for the treatment of DU's. An improvement of the healing of active ulcers has been described with Iloprost. Bosentan significantly reduced the frequency of occurrence of new DU's. In some small studies PDE-5 inhibitors appear helpful. Further studies with other therapeutic approaches will follow in the next few years.


Assuntos
Iloprosta/uso terapêutico , Inibidores da Fosfodiesterase 5/uso terapêutico , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/terapia , Úlcera Cutânea/etiologia , Úlcera Cutânea/terapia , Sulfonamidas/uso terapêutico , Bosentana , Dedos/patologia , Dermatoses da Mão/diagnóstico , Dermatoses da Mão/etiologia , Dermatoses da Mão/terapia , Humanos , Escleroderma Sistêmico/diagnóstico , Úlcera Cutânea/diagnóstico , Cicatrização/efeitos dos fármacos
20.
Dtsch Med Wochenschr ; 139(20): 1064-9, 2014 May.
Artigo em Alemão | MEDLINE | ID: mdl-24801303

RESUMO

Raynaud's phenomenon (RP) is characterized by repeated vasospastic attacks of the distal extremities induced by cold, humidity, vibrations or emotional stress. It typically presents a triphasic colour change from white (palor; vasoconstriction) to blue (cyanosis) and red (reactive hyperaemia). The symptoms are based on a primary RP in 90 %. Secondary RP is a symptom of an underlying disease. RP has to be distinguished from other colour changes of the distal extremities like acrocyanosis, erythromelalgia, perniosis and Chilblain-Lupus. Patients history, clinical examination, ANA, ESR/CRP and nailfold capillaroscopy are essential for the early diagnosis of an underlying disease. The initiation of angiologic tests is important in patients with digital ulcers, necrosis or gangrene. Important differential diagnoses in secondary RP are autoimmune rheumatic diseases like systemic sclerosis and systemic lupus erythematodes as well as vascular diseases like arterial occlusions and compression syndromes or concomitant medication (i. e. beta-blocker).


Assuntos
Arteriopatias Oclusivas/diagnóstico , Diabetes Mellitus Tipo 1/diagnóstico , Exame Físico/métodos , Doença de Raynaud/diagnóstico , Doenças Reumáticas/diagnóstico , Diagnóstico Diferencial , Humanos
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