Abstract：
BACKGROUND:Scleromyxedema is a rare connective tissue disease that may affect numerous internal organs in addition to the skin. The disease is almost exclusively associated with monoclonal gammopathy. MATERIAL AND METHODS:This retrospective study summarizes the clinical characteristics of four patients with scleromyxedema. In all of the patients a systematic serological and apparative check-up was performed. RESULTS:The mean age of the four patients (three women and one man) was 51 years. In all cases, monoclonal gammopathy (3 cases of IgG lambda and 1 case of IgG kappa) was involved. In one patient, skin lesions were restricted to the upper part of the body and three patients had generalized disease. The internal organs of all patients were affected with fibrosis of the lungs, myositis and arthritis, peripheral polyneuropathy and hypomotility of the esophagus. The most effective forms of treatment in this patient collective were dexamethasone-pulse therapy, intravenous immunoglobulins and bortezomib. All patients had recurrences after finishing therapy. The mean observation period after the initial diagnosis of scleromyxedena was 6.25 years (range 2-11 years). CONCLUSION:Scleromyxedema is a rare multisystemic disease. The heterogeneous affection of internal organs necessitates a comprehensive check-up. The response to recently published treatment strategies is low and recurrences after finishing therapy are frequent.
journal_name
Z Rheumatoljournal_title
Zeitschrift fur Rheumatologieauthors
Kreuter A,Stücker M,Kolios AG,Altmeyer P,Möllenhoff Kdoi
10.1007/s00393-012-0997-5subject
Has Abstractpub_date
2012-08-01 00:00:00pages
504-14issue
6eissn
0340-1855issn
1435-1250journal_volume
71pub_type
杂志文章abstract：:The author describes the information gained from anamnesis which points to a psychogenetic cause of rheumatic complaints. These symptoms are part of the diagnostically important criteria which must be supplemented still further by means of a personal discussion with the patient. A great deal depends on the skill of th...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1993-07-01 00:00:00
abstract：:In recent years the great progress in knowledge on bone cell biology has allowed identification of molecular structures that can be targeted with pinpoint precision (druggable targets). Osteoclasts are regulated via the RANK-RANK-ligand (RANKL) signaling pathway and osteoblasts via the Wnt signaling pathway, both of w...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章,评审
doi：10.1007/s00393-016-0102-6
更新日期：2016-06-01 00:00:00
abstract：:Musculoskeletal ultrasonography (US) is an established and validated imaging technique in rheumatology. Ultrasonography is able to directly visualize soft tissue pathologies such as synovial tissue changes. Pathological findings in superficial cartilage, bone lesions and synovial tissue changes in the context of rheum...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章,评审
doi：10.1007/s00393-010-0730-1
更新日期：2010-12-01 00:00:00
abstract：:Rheumatoid arthritis (RA) is associated with a shortened life expectancy. Most premature deaths are caused by cardiovascular (CV) events; therefore it is of importance to consider the increased CV risk when treating RA patients. Traditional CV risk factors cannot fully explain the increased risk but the common underst...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-013-1198-6
更新日期：2013-05-01 00:00:00
abstract：:Various rheumatic symptoms can occur in association with malignancies and are termed the so-called paraneoplastic arthropathy. The clinical picture is often similar to primary inflammatory rheumatic diseases. At present there exist no epidemiological data on this disease entity. The case of a patient with tibial pain ...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-010-0734-x
更新日期：2011-06-01 00:00:00
abstract：UNLABELLED:We report on a 6-year-old Romanian girl with recently diagnosed hyper-IgD-syndrome. The leading symptom of this rare disease are periodic pyrexia, joint involvements (arthralgias/arthritis) and swollen lymph nodes. A permanent increase of alpha 1-acid glycoprotein fucosylation indicates persisting inflammati...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s003930050019
更新日期：1997-01-01 00:00:00
abstract：:In the last years some important therapies were introduced in the treatment of rheumatoid arthritis (RA), which represent a significant clinical advance. This concerns the introduction of TNF blockade with etanercept and inflixmab, the combination of DMARDs, leflunomide and the COX-2-specific inhibitors. In the year 2...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s003930170011
更新日期：2001-12-01 00:00:00
abstract：: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 th...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-014-1550-5
更新日期：2015-03-01 00:00:00
abstract：OBJECTIVE:The aim of the study was to determine which disease-related factors and non-disease features can explain the presence of systemic lupus erythematosus (SLE)-related fatigue in Turkish patients. METHODS:This cross-sectional study was carried out with 99 SLE patients and 71 healthy controls. To assess fatigue a...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-016-0185-0
更新日期：2017-12-01 00:00:00
abstract：BACKGROUND:The treatment strategies for osteoarthritis (OA) are well known from numerous studies. One of the challenges is long-term patient compliance to the recommended therapies without supervision. OBJECTIVE:To examine the ability of salutogenic concepts to improve rehabilitative management of OA. MATERIALS AND M...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章,评审
doi：10.1007/s00393-014-1557-y
更新日期：2015-09-01 00:00:00
abstract：:278 patients with ankylosing spondylitis have been studied in order to find a basis for prognosis by setting a mobility function index of the vertebral column. This index does not depend on manifestation age or on the blood sedimentation rate. It increases with progression of x-ray findings. A coincidence of impaired ...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1975-11-01 00:00:00
abstract：:409 unselected outpatients were asked standardized expert system questions on the medical history of joint complaints. An average number of 30.4 +/- 6.6 out of 60 questions with 346 different answers were asked in a mean time of 20.1 +/- 6.6 min. This time, required for the questions to be displayed on the computer sc...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1991-07-01 00:00:00
abstract：:Binding of gold to serum proteins of patients treated with Na-aurothiomalate (AM), gold keratinate (GK) and triethylphosphine gold (PG) was compared. In addition the pattern of distribution of gold in serum of healthy donors after in vitro incubation with these compounds and with tetrachlorogoldacid (GC) was studied. ...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1982-09-01 00:00:00
abstract：:A 28-year-old female patient had load-dependent pain of the left knee. MRI showed an osteochondral defect of the medial condyle based on extensive osteonecrosis of the femoral and tibial bone. An association with a prior steroid treatment after cervical injury is most likely. ...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-004-0556-9
更新日期：2004-06-01 00:00:00
abstract：:Methotrexate (MTX) is a safe and well-tolerated drug for the treatment of rheumatic diseases, even if the strictest safety standards are considered. Initial apprehension as to severe pulmonary or hepatic side-effects has not been confirmed. The risk of infection is not elevated compared with other disease-modifying an...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-010-0687-0
更新日期：2011-02-01 00:00:00
abstract：:The commission "Rehabilitation" of the German Society of Rheumatology compiled a data set for a routine report of the rehabilitation system for muskuloskeletal diseases. More than 250 rehabilitation hospitals offer inpatient rehabilitation for patients with musculoskeletal diseases. The prevalence of inpatient rehabil...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-005-0626-7
更新日期：2005-06-01 00:00:00
abstract：:In a twenty-nine year old female patient with progressive systemic sclerosis (scleroderma) (PSS) and an impressing involvement of the intestine (disturbance of the oesophageal motility, pseudo-obstruction, malabsorption) a spontaneous, asymptomatic pneumoperitoneum is observed. Perforation or pneumatosis cystoides int...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1986-03-01 00:00:00
abstract：:1436 young men were investigated clinically and radiologically for rheumatic sacro iliitis. In two men (1.4%) definite bilateral sacro iliitis was diagnosed. In 18 cases (12.5%) the signs were not sufficient to make a definite diagnosis. They were regarded as suspected iliitis. For comparison the cards of 6138 patient...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1977-09-01 00:00:00
abstract：:The development of synovitis was studied in 64 rabbits with experimentally produced allergic arthritis and in 30 rabbits with experimentally produced non-allergic arthritis. The results are compared with the morphological changes of the synovial membrane in rheumatoid arthritis, especially in early stages, known from ...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1982-01-01 00:00:00
abstract：:The photometric latex test (PLT) for the detection of rheumatoid factors (RF) has been correlated with the sensitized sheep cell test (SSC) and the latex slide test (LST). A total of 377 sera from patients with rheumatoid arthritis (RA) and 120 sera from control subjects were examined. When the PLT was carried out on ...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1978-03-01 00:00:00
abstract：:Fifty-seven patients with rheumatoid arthritis were examined clinically, radiologically, and by means of transcranial magnetic brain stimulation. With this method central motor conduction times of the corticospinal pathways can be measured. The aim of the study was to compare the different methods to detect a cervical...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1990-09-01 00:00:00
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journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章,评审
doi：10.1007/s00393-018-0534-2
更新日期：2018-12-01 00:00:00
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journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1985-09-01 00:00:00
abstract：:The supernatant of peripheral blood monocytes, cultured with autologous sera from patients with rheumatoid arthritis, stimulates healthy human granulocytes to significantly elevated chemiluminescence (p = 0.05) compared with supernatants of monocytes from osteoarthritic patients or healthy volunteers. Cultures without...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1988-05-01 00:00:00
abstract：:The case described concerns a patient initially presenting the clinical symptoms of rheumatoid arthritis, including the presence of nodules, and slowly developing into systemic lupus erythematosus (SLE). Besides the signs of chronic inflammation in the synovial tissue, many granulocytes and PAS-positive macrophages we...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1979-07-01 00:00:00
abstract：:The Lequesne algofunctional indices for the knee and the hip require a physician interview. We adapted the Lequesne indices for the German language and tested the metric properties, test-retest reliability and validity of a self-report questionnaire-format in 51 patients with hip or knee OA. The symptom and function c...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1996-01-01 00:00:00
abstract：:Antibodies to double-stranded DNA (anti-dsDNA, dsDNA-Ab) are frequently found in systemic lupus erythematosus (SLE), especially during active disease and differ with respect to immunoglobulin class and avidity. The detection of anti-dsDNA is one of the diagnostic criteria for SLE according to the American College of R...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s003930050020
更新日期：1997-03-01 00:00:00
abstract：:The influence of the oxicams, a special group of non-steroidal anti-inflammatory drugs, to the sister chromatid exchange (SCE) was determined on human lymphocytes in vitro and in vivo. The analysis of SCE is a sensitive parameter indicating chromosomal damage. The cytogenetic examinations of Lornoxicam, Tenoxicam, and...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：
更新日期：1990-03-01 00:00:00
abstract：AIM:To explore the circadian rhythm of serum interleukin (IL)-6 in collagen-induced arthritis (CIA) rats and compare the safety and effectiveness of methotrexate (MTX) administered traditionally and via chronotherapy. METHODS:CIA rat models were immunized with bovine type II collagen. Serum IL-6 levels in normal and C...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章
doi：10.1007/s00393-016-0236-6
更新日期：2018-04-01 00:00:00
abstract：:Interstitial nephritis is responsible for about 12 % of end-stage renal disease in Germany. It comprises an etiologically heterogenous group of inflammatory renal disorders which primarily affect the renal interstitium and tubuli. Drugs, predominantly antibiotics, nonsteroidal anti-inflammatory drugs and proton pump i...
journal_title：Zeitschrift fur Rheumatologie
pub_type： 杂志文章,评审
doi：10.1007/s00393-014-1482-0
更新日期：2015-05-01 00:00:00
