Connective tissue diseases systemic sclerosis

Myositis, autoimmune. Rare systemic inflammatory myopathies, including primary polymyositis, primary dermatomyositis, myositis associated with malignancy, childhood dermatomyositis, and myositis with multisystem autoimmune disease (e.g. mixed connective tissue disease, systemic sclerosis). Autoantibodies against aminoacyl-tRNA synthetases (e.g. anti-Jo-1), signal recognition particle (e.g. anti-SRP54), nuclear helicase (anti-Mi-2), tRNA and tRNA-protein complexes (e.g. anti-Mas), and translation factor (anti-KJ) have been described as myositis specific. 

Although there are reports of associations between exposure to organic solvents and various connective tissue diseases, such as systemic sclerosis, scleroderma, undifferentiated connective tissue disease, systemic lupus erythematosus, and rheumatoid arthritis, the evidence of a causal association is weak (44). 

Connective tissue diseases. Systemic autoimmune rheumatic diseases, including systemic lupus erythematosus, Sjogren syndrome, systemic sclerosis (scleroderma), autoimmune myositis (polymyositis, dermatomyositis), mixed connective tissue disease, and other overlapping syndromes. 

It has been shown that lung macrophages from patients with systemic sclerosis (SS) produced the elevated levels of nitric oxide, superoxide, and peroxynitrite and expressed the enhanced level of iNOS [281], NAC administration reduced peroxynitrite production and might be possibly recommended for the treatment SS patients. Solans et al. [282] found the significant enhancement of lipid peroxidation in erythrocytes from SS patients. Cracowski et al. [283] showed that in vivo lipid peroxidation was enhanced in scleroderma spectrum disorders including SS and undifferentiated connective tissue disease. 

Scleroderma is a progressive systemic sclerosis with a multisystem connective tissue disease. A number of industrial chemicals have been implicated as causative factors in human scleroderma. Industrial chemicals (e.g., toluene, benzene, xylene, aromatic mixers or white spirit, vinyl chloride, trichloroethylene, perchloroethylene, naphtha-n-hexane, epoxy resins,... 

In an evaluation of the frequency and clinical characteristics of the underlying connective tissue disorders associated with silicone breast implants, 300 women with silicone breast implants were studied (45). In addition to a history and physical examination, C reactive protein, rheumatoid factor, and autoantibodies were determined. Criteria for fibromyalgia and/or chronic fatigue syndrome were met by 54% connective tissue diseases were detected in 11% and undifferentiated connective tissue disease or human adjuvant disease in 10.6%. A variety of disorders, such as angioedema, frozen shoulder, and a multiple sclerosis-like syndrome, were also found. Several other miscellaneous conditions, including recurrent and unexplained low grade fever, hair loss, skin rash, symptoms of the sicca sjmdrome, Rajmaud s phenomenon, carpal tunnel syndrome, memory loss, headaches, chest pain, and shortness of breath were also seen. Of 93 patients who underwent explantation, 70% reported improvement in their systemic symptoms. 

There have been case-reports of a confirmed diagnosis of systemic sclerosis, rheumatoid arthritis, or systemic lupus erythematosus in patients with silicone breast implants (Silverstein et al., 1992 Sanchez-Guerrero et al., 1994 Cuellar et al., 1995 Field Bridges, 1996). However, no association between silicone breast implants and connective tissue disease has been seen in more than... 

Mixed connective tissue disease (MCTD). Systemic autoimmune disease with features of systemic lupus erythematosus, systemic sclerosis, and dermatomyositis/polymyositis and high-titred autoantibodies against Ul-RNP specific proteins. See also myositis, autoimmune. 

Raynaud phenomenon. Vasospastic condition characterized by acral circulatory disorders affecting the hands and feet. The symptoms can be triggered by cold, dampness, or emotional stress. Characteristic feature of systemic autoimmune diseases. Occurs in all or virtually all patients with systemic sclerosis, mixed connective tissue disease, and polymyositis/scleroderma overlap syndrome. 

Hochberg MC Perlmutter DL (1996) The association of augmentation mammoplasty with connective tissue disease, including systemic sclerosis (scleroderma) a metaanalysis. CurrTop Microbiol Immunol, 210 411-417. 

Anomalies in the acute phase protein response have been reported in various diseases. Studies in systemic lupus erythematosus, dermatomyositis, systemic sclerosis, mixed connective tissue disease, ulcerative colitis, primary biliary cirrhosis, and chronic active hepatitis have shown low acute phase protein responses for the amount of inflammatory activity present (P6). This may be due either to disease-related unresponsiveness or to down-regulation of the acute phase response in chronic inflammation (Ml, W16). Patients with systemic sclerosis do not mount an appreciable acute phase response to therapeutic infusion of PGE, compared with patients with atherosclerosis (W24), although they do to infections (C20). 

The potent vasodilatory effects of PGE, have been used with success in treatment of severe forms of Raynaud s disease as well as vascular insufficiency in systemic sclerosis and other connective tissue diseases [168], PGE, and PGE2 also relax the ductus arteriosus of newborns. Infusions of PGE, (50 ng/kg/min) to newborns with certain cardiac malformations, which make the persistence of the ductus essential for the systemic or pulmonary circulation, improves the clinical condition and allows surgery to be delayed to a more suitable time (see ref. 169 for a review). The tone of the ductus arteriosus seems to be balanced between the constrictor effects of oxygen, possibly also other vasoconstrictor substances, and the dilatory effects of prostaglandins formed intramurally [170], Inhibitors of prostaglandin biosynthesis have been used to induce closure of patent ductus arteriosus of newborns, and in most cases the conventional surgical treatment could be omitted [171], For reviews on the role of prostaglandins in the vascular system see refs. 133, 165, 172 and 173. 

Connective tissue diseases, such as systemic lupus erythematosus, systemic sclerosis, or rheumatoid arthritis, may also be related to mesenteric lymphadenopathy (Calguneri et al. 2003). In these patients, mesenteric lymphadenopathy is more frequently an occasional US finding and seldom the only manifestation of lymph node involvement... 

In June of 1994, a population-based retrospective cohort study was published which examined the risk of a variety of connective tissue diseases and other disorders after breast implantation [65]. In this study, all women in Olmsted County, Minnesota who received a breast implant between 1 January 1964 and 31 December 1991 (the case subjects) were studied. For each case subject, two women of the same age (within three years) from the same population who had not received a breast implant and who underwent a medical evaluation within two years of the date of the implantation in the case subject were selected as control subjects. Each woman s complete inpatient and outpatient medical records were interviewed for the occurrence of various connective tissue diseases (i.e., rheumatoid arthritis, systemic lupus erythematosus, Sjogren s syndrome, dermatomyositis, polymyositis, systemic sclerosis, ankylosing spondylitis. 

In a recent report of an analysis of 28 men suffering from systemic sclerosis collated over a 25-year period, 21 (75%) were suspected to be associated with occupational scleroderma. The exposures were revealed by patients files, reports to health authorities and interviews and/or answers to questionnaires. The most frequent chemical exposure was to organic solvents. This was found in 13 of the patients (46%). A significant difference was found between scleroderma patients and control patients not suffering from connective tissue diseases in relation to total number of subjects exposed to one or several of the agents in question (Zachariae et al. 1997). 

Connective tissue disorders involving occupational factors are, first of all, systemic sclerosis (SSc), probably also lupus erythematosus (LE) and very rarely dermatomyositis, mixed connective tissue disease (MCTD), rheumatoid arthritis (RA) and Sjogren s syndrome (Zschunke et al. 1990 Koeger et al. 1991). 

Owens GR, Medsger TA (1988) Systemic sclerosis secondary to occupational exposure. Am J Med 85 114-116 Panaszek B, Malolepszy J, Wrzyszcz M, Jutel M, Machaj Z (1993) Mixed connective tissue disease in a male patient chronically exposed to toxic chemicals (in Polish). Pol Tyg Lek 48 430-432... 

Also, an increased incidence of cell-mediated and humoral immunity to various collagens has been found in systemic connective tissue disease such as rheumatoid arthritis, juvenile rheumatoid arthritis, and progressive systemic sclerosis (scleroderma). Patients with these diseases may thus have an increased susceptibility to hypersensitivity responses and/or accelerated clearance of their implants w hen injected with bovine dermal collagen preparations. Therefore, caution should be used when treating tliese patients including consideration for multiple skin lesions. 

Connective tissne-associated DPLD Rheumatoid arthritis Systemic sclerosis Systemic lupus erythematosus Polymyositis/dennatomyositis Mised coimective tissue disease Undifferentiated connective tissue disease... 

The connective tissue diseases (CTDs) are a heterogeneous group of immuno-logically mediated inflammatory conditions of unknown etiology, accompanied by diverse autoantibodies and affecting multiple organ systems. In adults, the more frequent CTDs comprise rheumatoid arthritis (RA), systemic sclerosis (SSc), Sjogren s syndrome (SjS), systemic lupus erythematosus (SLE), polymyositis/ dermatomyositis (PM/DM), and mixed CTD (MCTD). 

Abbreviations. RA, rheumatoid arthritis SLE, systemic lupus erythematosus SSc, systemic sclerosis DM/PM, dermatomyositis/polymyositis SjS, Sjogren s syndrome MCTD, mixed connective tissue disease PH, pulmonary hypertension. 

Abbreviations-. ILA, interstitial lung disease RA, rheumatoid arthritis SLE, systemic lupus erythematosus SSc, systemic sclerosis DM, dermatomyositis PM, polymyositis SjS, Sjogren s syndrome MCTD, mixed connective tissue disease NSIP, non-specific interstitial pneumonia UIP, usual interstitial pneumonia OP, organizing pneumonia DAD, diffuse alveolar damage DIP, desquamative interstitial pneumonia LIP, lymphocytic interstitial pneumonia. 

The strength of the association between silicosis and connective tissue disease varies with the type of connective tissue disorder. The risk of developing systemic sclerosis, particularly in workers with high exposure to silica dust, is well established, although such casual associations between silicosis, rheumatoid arthritis and systemic lupus erythematosis are less widely reported (Rosenman 1999 Sluis-Cremer et al. 1985, 1986 Sanchez-Roman et al. 1993)... 

Inpus erythematosus A chronic inflammatory disease of connective tissue, affecting the skin and internal organs, lymphoma A malignant tumor of the lymph nodes, multiple sclerosis A disease of the nervous system, myelodysplasia Abnormal or defective formation of the bone marrow. Mycoplasma Minute primitive bacteria without a rigid cell wall. Mycoplasma pneumoniae causes atypical pneumonia in humans, myeloma cells Malignant tumor cells. 

Xenobiotic exposure can adversely affect bones, joints, connective tissue, and muscles. Rheumatoid arthritis, osteoporosis, osteomalacia, systemic sclerosis, scleroderma, systemic lupus erythematosus, and spina bifida are musculoskeletal diseases that have been associated with toxic chemical exposures. Most of these associations, however, have been made to single chemical exposures and not to mixtures. This chapter cites the evidence on which those associations are based and discusses the available examples of mixtures that have been implicated. 

Systemic sclerosis (SSc), also named scleroderma, is a systemic disease characterised by thickening of the skin due to an accumulation of connective tissue and by the involvement of various organs such as the lungs, gastrointestinal tract, heart and kidneys. The annual incidence has been estimated to be 19 cases per million population, and the reported prevalence of SSc is between 19 and 75 per 100,000 persons. 

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