Biomedical subjects
Publications and source records attributed to E Genth.
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Drug intake had been checked in 58 patients with pseudo-LE-syndrome. In view of the findings of the Zürich group a connection between the intake of Venopyronum¿ coated tablets and a pseudo-LE syndrome was strongly suspected in 45. In a further seven patients who also had taken the drug no definite connection could be shown because there was no temporal relationship between drug and disease. Three patients had taken Venopyronum-triplex capsules. Only three patients categorically denied ever having taken the drug in any form.
Fifteen women with pseudo-LE syndrome were studied. The dissease is characterized by recurrent attacks of fever, myalgia, arthralgia, visceral manifestations (pericarditis, myocarditis, pleurisy, pleural effusion and lung involvement). A prominent feature was an abnormal liver function with elevated GOT and GPT levels in serum, and impaired bromsuophalein elimination. The incidence of varicosities and thrombophlebitis was markedly increased. There was a correlation between complement-fixing antimitochondrial antibody (AMA) titres and the degree of activity of the disease. A reverse correlation was observed between antimitochondrial antibody titres and the absolute lymphocyte count in peripheral blood. In all instances the antimitochondrial antibodies were of polyclonal origin, belonging mainly to immunoglobulin class IgG and, in a few patients, IgM, IgA, or IgD. AMA titres, lymphocyte counts in peripheral blood, sedimentation rate, serum iron level and alpha1- and alpha2-globulins proved to be valuable in following the course of the disease. Other values (C-reactive protein, alpha1-acid glycoprotein, immunoglobulin and beta1c-globulin in serum) were not helpful.
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Diffuse fasciitis (DF) [diffuse fasciitis with eosinophilia-Shulman's syndrome] has occasionally been linked to a precedent infection with Borrelia burgdorferi. Here, we report on another case of DF in a 25 year old male, in whom Borrelia burgdorferi infection as possible inciting agent could be identified based on the patient's history and laboratory data. Efforts to microscopically demonstrate spirochetes or to amplify Borrelia-DNA by nested PCR in lesional tissue failed after antibiotic treatment had already been initiated. Although only a few cases of Borrelia associated diffuse fasciitis have been reported in the literature, the link between typical signs and symptoms as well as laboratory findings of Borrelia infection and the onset of diffuse fasciitis, starting at the primary site of EM, provide indirect evidence for a causative role of Borrelia burgdorferi as a potential infectious agent for DF.
We describe a 53-year-old women with eosinophilia-myalgia syndrome who suddenly developed severe persistent myalgias of her arms, legs, back, and shoulder after a 5-month period of daily L-tryptophan ingestion, associated with fever, progressive stenocardia and left-sided congestive heart failure. Laboratory tests showed a leukocytosis of 11.2/nl with 3.14/nl eosinophils and an elevated erythrocyte sedimentation rate. There was a marked, predominantly proximal sclerosis of her arms, legs and trunk with a brownish discoloration. The skin of her arms and legs appeared dimpled (peau d'orange). Findings of the electrophysiological examinations were consistent with sensory neuropathy and myositis. Remarkable fasciitis and interstitial myositis were present in a biopsy specimen (from skin to muscle) taken from her thigh. However, eosinophilic infiltrates were rare. Angiography revealed an apical obstructive cardiomyopathy. In this paper, we describe the clinical findings, the course over 2 years, as well as the therapeutic management. Furthermore, the most important differential diagnoses are discussed and the literature is reviewed with special attention given to more recent pathogenic insights into this newly recognized multisystem disease.
68 cases with polyarthritis were selected from 406 HLA B 27 positive patients with various rheumatic diseases excluding ankylosing spondylitis (AS) or Reiter's disease. 23 fulfilled at least 5 criteria of the ARA for the diagnosis of rheumatoid arthritis (RA). 5 suffered from polyarthritis and psoriasis. The remaining 40 patients expressed an asymmetric oligarthritis especially of the lower limbs (knee, ankle) affecting predominantly young adult men. Sacroiliitis was observed in 10 cases. Joint erosions, rheumatoid factors and visceral manifestations were uncommon. The arthritic pattern of B 27 positive oligarthritis differed clearly from rheumatoid arthritis (n = 34) and psoriatic arthritis (n = 15), but was similar to peripheral joint involvement in AS (n = 32) except for the higher incidence of coxitis in AS. HLA typing is helpful not only in the early diagnosis of AS but also in the differential diagnosis of unclassifiable polyarthritis.
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Rehabilitation its an important part of the management of patients with chronic rheumatic diseases and it aims to improve or supervene physical or psycho-social disability and handicap. With regard to the fluctuating course of chronic inflammatory rheumatic diseases, a universal concept of rehabilitation always includes the curative aspect of treatment. Based on the legal and organizational premises for rehabilitation, the problems of access, usage, management and of continuous treatment are critically reviewed. The important tasks in rehabilitation of patients with rheumatic diseases by rehabilitation clinics are education and informing of patients about their disease, including the outcome, possible treatments, self management and coping strategies as well as functional therapy of disorders of the locomotor system. In addition, the adaptation of medical and physical treatment, advice and help with regard to work and occupation, and planning of continuing rehabilitative interventions are important activities of rehabilitation clinics. In the future, an improved integration of rehabilitation within concepts of medical and social support, progress in quality of structuring and processing activities in rehabilitation clinics, and the expansion of ambulant rehabilitation to facilitate continuous support has to promoted.
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In more than 95% of patients with systemic sclerosis and in about 60% of patients suffering from idiopathic inflammatory myopathies autoantibodies directed at different nuclear or cytoplasmic antigens can be detected with different methods. Scleroderma-associated autoantibodies can be visualized as antinuclear antibodies (ANA) by immunofluorescence assays using cultured monolayer cells. In case of a negative ANA result the diagnosis of systemic sclerosis is unlikely. In individual patients the different autoantibodies (against DNA topoisomerase I (Scl-70), centromeric antigens, fibrillarin, To (Th), RNA polymerases, NOR-90, U1-nRNP, PM-Scl, Ku) are mutually exclusive. They can be detected early in the course of diseases, most often are persistent, and are closely associated with immunogenetic markers. They are characteristic for distinct subsets of patients homogeneous in clinical manifestations as well as in disease outcome. Myositis-associated autoantibodies are directed to nuclear (about 60% of myositis patients; PM-Scl, Mi-2) or cytoplasmic antigens (about 35-40%; Jo-1 and other aminoacyl-tRNA-synthetases, signal recognition particle (SRP), KJ and others) and likewise are related to distinct clinical, prognostic, and immunogenetic traits leading to the description of characteristic antibody-based syndromes. Based on published results and on our own investigations, the diagnostic potential of scleroderma- and myositis-associated antibodies is evaluated and a new classification of systematic myositic and sclerodermatous disease is proposed.
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