Scleroderma
Dotaz
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Clinics in dermatology, ISSN 0738-081X vol. 12, no. 2, April-June 1994
193-332 s. : il., tab. ; 28 cm
- MeSH
- dítě MeSH
- dospělí MeSH
- klinické lékařství MeSH
- lokalizovaná sklerodermie diagnóza klasifikace terapie MeSH
- nemoci kůže a pojivové tkáně terapie MeSH
- Raynaudova nemoc farmakoterapie MeSH
- systémová sklerodermie diagnóza klasifikace terapie MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- Publikační typ
- sborníky MeSH
- Konspekt
- Patologie. Klinická medicína
- NLK Obory
- dermatovenerologie
- revmatologie
Clinics in dermatology, ISSN 0738-081X vol. 12, no. 3, July-September 1994
333-479 s. : il., tab. ; 28 cm
- MeSH
- atrofie klasifikace MeSH
- Borrelia burgdorferi imunologie MeSH
- genetické nemoci vrozené MeSH
- klinické lékařství MeSH
- lokalizovaná sklerodermie genetika imunologie patofyziologie MeSH
- myalgický syndrom s eozinofilií MeSH
- systémová sklerodermie genetika imunologie patofyziologie MeSH
- Publikační typ
- sborníky MeSH
- Konspekt
- Patologie. Klinická medicína
- NLK Obory
- dermatovenerologie
- revmatologie
1 online zdroj
- MeSH
- systémová sklerodermie * MeSH
- Publikační typ
- periodika MeSH
- Konspekt
- Patologie. Klinická medicína
- NLK Obory
- dermatovenerologie
- MeSH
- lidé MeSH
- senioři MeSH
- srdeční selhání MeSH
- systémová sklerodermie komplikace MeSH
- Check Tag
- lidé MeSH
- mužské pohlaví MeSH
- senioři MeSH
- Publikační typ
- kazuistiky MeSH
255 s. : il.
Dear Editor, Scleroderma associated with neoplasia is rare, with only a small number of cases reported. We describe 4 patients with paraneoplastic scleroderma who were treated at the I. Department of Dermatovenereology, St. Anna Hospital, during the period between 2004 and 2014. The patients were diagnosed with cholangiogenic carcinoma, endometrial carcinoma, prostatic adenocarcinoma, and adenoma of the suprarenal gland. In the case of concurrent scleroderma and tumor, four situations may occur: they can develop independently of each other; scleroderma may be induced by the tumor; the tumor can develop in the scleroderma; or the tumor can be induced by immunosuppressive therapy. Sclerotization of the skin was described in association with lung cancer, carcinoid, plasma cell dyscrasia, cancer of the ovary, cervix, breast, esophagus, stomach, nasopharynx, melanoma, and sarcoma (1,2,5,7,10). Symptoms may be induced by substances secreted by the tumor (hormones, cytokines, etc.) (9). Tumorous cells further induce cytotoxic and autoantibody response. Scleroderma is characterized by immunological dysregulation, vasculopathy, and hyperproduction of the extracellular matrix by activated fibroblasts. Endothelial, inflammatory, and mesenchymal cells produce cytokines, chemokines, and growth factors e.g. Interleukin-1 (IL1), Interleukin-6 (IL6), tumor necrosis factor alpha (TNF α), collagen alpha 1, connective tissue growth factor (CTGF) (3), and basic fibroblast growth factor (bFGF). This factor is also produced by lung cancer cells (4). The clinical picture of scleroderma and paraneoplastic scleroderma is similar. Diffuse thickening of the skin and/or sclerodermatous plaques can be seen. The histological picture is consistent with scleroderma. Capillaroscopy changes, antinuclear antibodies (ANA), sclerodactyly, and Raynaud phenomenon suggest the diagnosis of systemic scleroderma (SS) (4). Our patients did not fulfill enough of the criteria for SS. Both diffuse and localized scleroderma was seen in 3 patients and generalized localized scleroderma in one case. All patients had a histological picture consistent with scleroderma, negative ANA and ENA antibodies (Table 1, Figure 1). A 66-year-old woman presented with a 10 months history of sclerodermatous plaques on her neck, trunk, and upper and lower extremities. The skin on her breasts and cheeks was diffusely indurated. Examination showed thrombocytopenia, elevated transaminases, Cancer antigen 19-9 (Ca 19-9), thyroid stimulating hormone (TSH), and anti-thyroid peroxidase antibodies, dysmotility of the lower part of esophagus, hepatosplenomegaly, cholecystolithiasis, and benign polyps of colon. She was given prednisone 40 mg/day but did not return for follow up. After 6 months she was diagnosed with cholangiogenic carcinoma with metastatic disease and died shortly afterwards. A 74-year-old woman had localized scleroderma on the trunk for three years. She was treated with procaine penicillin for positive borrelia Immunoglobulin M (IgM) antibodies. Her condition worsened suddenly with confluent scleroderma plaques on her trunk, extremities, and genital region, and vasoneurosis on her lower extremities; she was started on prednisone 35 mg/day. Examination revealed endometrial cancer. The patient underwent a hysterectomy, adnexectomy, and radiotherapy with curative effect. Scleroderma patches softened with residual hyperpigmentation, and prednisone was stopped two years later. A 80-year-old man had a month-long history of diffuse thickening and toughening of the skin on the forearms and lower legs and scleroderma patches on the thighs and shins. Examination revealed prostate adenocarcinoma, and therapy with antiandrogen bicalutamide and prednisone 15 mg/day was started. Two years after the diagnosis he continues with bicalutamide treatment, prednisone 5 mg q.a.d. and has residual toughening of the skin on his lower legs. A 62-year-old woman with seronegative rheumatoid arthritis presented with diffusely tough skin on her extremities and trunk, present for 2 months. Examination revealed cervicitis with a benign endometric polyp, cholecystolithiasis, borderline pulmonary hypertension, and a hormonally inactive suprarenal adenoma. She was given prednisone 40 mg/day and penicillamine with effect. In the 3rd year of therapy she has residual induration of her lower legs and a scleroderma plaque in the lumbar region. She is monitored for her suprarenal adenoma. Two patients had scleroderma at the same time as a malignant tumor; in one patient the localized scleroderma worsened rapidly at the time of the tumor diagnosis, and in one patient a clinically silent adenoma was found. Adrenal tissue can secrete molecules such as serotonine or bFGF involved in fibroplasia (3,6). One patient died of a metastatic disease, two patients after the successful treatment of the tumor, and the patient with suprarenal adenoma experienced softening of the skin and regression of scleroderma. Although paraneoplastic scleroderma is often classified as a pseudoscleroderma, we regard neoplasia as a distinct triggering impulse for scleroderma. Recently, an association between RNA polymerase I/III antibodies in systemic scleroderma and cancer was suggested (8). Such studies may confirm the true link between scleroderma and malignancy. These patients are characterized by older age, sudden onset, diffuse thickening of the skin, and/or generalized morphea with a concurrent neoplastic process. In the case of a successful tumor treatment, skin changes regress.
- MeSH
- lidé středního věku MeSH
- lidé MeSH
- lokalizovaná sklerodermie komplikace diagnóza MeSH
- paraneoplastické syndromy komplikace diagnóza MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- senioři nad 80 let MeSH
- senioři MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- kazuistiky MeSH
Systémová sklerodermie (SSc) jako chronické multisystémové onemocnění je spojeno se závažným funkčním postižením a významným dopadem na kvalitu života pacientů. K hodnocení dopadu tohoto onemocnění na funkční omezení pacientů v běžném životě bylo vyvinuto několik specifických nástrojů, včetně Scleroderma Health Assessment Questionaire (SHAQ), Cochin Hand Functional Scale (CHFS), Mouth Handicap in Systemic Sclerosis (MHISS) a UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0). Cílem této práce bylo vytvoření českých verzí těchto nástrojů a jejich lingvistická validace. Originální verze dotazníků byly přeloženy třemi nezávislými bilingválními revmatology a tyto překlady byly konsenzuálně diskutovány a sjednoceny autorskou skupinou. Poté se šest bilingválních nelékařů vyjádřilo k lingvistické a obsahové stránce dotazníků a jejich připomínky byly expertním panelem zapracovány. Tato verze byla postoupena dvěma nezávislým zaslepeným překladatelům k přeložení zpět do anglického jazyka. Konsenzuálně pak byly vytvořeny prefinální české verze dotazníků, které vyplnilo 55 pacientů se SSc. K hodnocení srozumitelnosti a výstižnosti jednotlivých otázek dotazníků byl použit dotazník zpětné vazby. Všichni pacienti se SSc označili dotazníky jako zcela jasné a srozumitelné. Výsledkem této práce jsou české verze dotazníků SHAQ, CHFS, MHISS a UCLA SCTC GIT 2.0. Hodnoty dosažených skóre pacientů se SSc se numericky blížily výsledkům jiných publikací. Tyto dotazníky mohou být vhodnými nástroji používanými v klinickém výzkumu i v rutinní klinické praxi.
Significant impact on a patient's quality of life. Several specific tools have been developed to assess the impact of this disease on the patient's functional disability in everyday life, including Scleroderma Health Assessment Questionaire (SHAQ), Cochin Hand Functional Scale (CHFS), Mouth Handicap in Systemic Sclerosis (MHISS) a UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 (UCLA SCTC GIT 2.0). The aim of this work was to perform the Czech translation and linguistic validation of the above-mentioned Patient-reported outcome measures. The original versions of the questionnaires were translated by three independent bilingual rheumatologists and consensually discussed and synthesized by the authors of this paper. In the next step, six bilingual non-physicians commented on the linguistic and content aspects of the questionnaires, and their comments were taken into account by an expert panel. Subsequently, two independent blinded translators carried out a back-translation. Consensually, pre-final Czech versions of the questionnaires were created, which were afterward filled out by 55 patients with SSc. A feedback questionnaire was used to assess the comprehensibility and conciseness of each question. All patients with SSc considered questionnaires clear and understandable. This work has resulted in the development of the Czech versions of the SHAQ, CHFS, MHISS, and UCLA SCTC GIT 2.0. questionnaires. Patients with SSc achieved scores that were numerically similar to the results of previously published studies. These questionnaires may be appropriate tools to be used in clinical research and routine clinical practice.
- Klíčová slova
- Scleroderma Health Assesment Questionaire, Cochin Hand Functional Scale, Mouth Handicap in Systematic Sclerosis,
- MeSH
- kvalita života MeSH
- lidé středního věku MeSH
- lidé MeSH
- průzkumy a dotazníky MeSH
- reprodukovatelnost výsledků MeSH
- systémová sklerodermie * patofyziologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- práce podpořená grantem MeSH
- MeSH
- dermatomyozitida MeSH
- kožní manifestace MeSH
- nemoci pojiva MeSH
- systémová sklerodermie MeSH
- Publikační typ
- přehledy MeSH
