Connective tissue diseases mixed

The definition of an overlap syndrome dictates that the criteria for diagnosis of both disorders (in the present context, of PM/DM and of some other connective tissue disorder), are fulfilled. It is not unexpected that those syndromes which overlap with PM/DM are also either known autoimmune conditions or ones in which an autoimmune basis is strongly suspected. The association of these disorders with PM/DM syndromes may not materially alter the basic histopathological featmes expected in PM/DM but some differences may be identifiable. The disorders most frequently associated with an overlap syndrome are rheumatoid arthritis, systemic lupus erythematosis, scleroderma, and mixed connective tissue disease. 

The autoimmune rheumatic diseases consists of Rheumatoid Arthritis (RA), Spondylarthritis (SpA), Systemic Lupus Erythematosus (SLE), Polymyositis, Dermatomyositis, Polymyalgia Rheumatica, Acute Temporal Arteritis, Giant Cell Arteritis, Behcet s Disease, Sjorgren s Syndrome, Felty s Syndrome and Mixed Connective Tissue Disease (MCTD). Spondylarthritis (SpA) can be subdivided in Reactive Arthritis (ReA), Ankylosing Spondylitis (AS), Psoriatic Arthritis (PsA), Arthritis associated with the inflammatory bowel diseases are Crohn s disease and Ulcerative Colitis (IBD), Undifferentiated SpA (UspA) and Sacro-ilitis, Juvenile SpA and Acute Anterior Uveitis (AAU). 

B12. Bennett, R. M., Cornell, K. A., Merritt, M. J., Bakke, A. C., Hsu, P. H., and Hefeneider, S. H., Autoimmunity to a 28—30 kD cell membrane DNA binding protein Occurrence in selected sera from patients with SLE and mixed connective tissue disease (MCTD). Clin. Exp. Immunol. 86, 374-379 (1991). 

H4. Halla, J. T., Volanakis, J. E., and Schrohenloher, R. E., Circulating immune complexes in mixed connective tissue disease. Arthritis Rheum. 22, 484-489 (1979). 

SweUing and wrinkling of the pahns soon after exposure to water occurred in an 18-year-old-woman taking rofecoxib for mixed connective tissue disease (120). The swelling followed exposure for 1 to a few minutes to water at any temperature. The swelhng developed rapidly and was accompanied by symptoms of mild pain and discomfort. Over the following 2 hours, the palms would return to normal. Because of the temporal correlation with the use of rofecoxib, it was withdrawn, and 3 weeks later her symptoms had almost completely resolved. 

Two studies have provided insights into the incidence and risk factors of the immune-mediated comphcations of interferon alfa in patients with chronic myeloid leukemia. In the first study, 13 of 46 patients had autoimmune manifestations consisting of a combination of autoimmune thyroiditis in four, a direct antiglobulin test without hemolysis in eight, cryoagglutinins in one, Raynaud s phenomenon in two, and chronic autoimmune hepatitis in one (343). Overall, six patients had chnically symptomatic manifestations after a median of 15 months of treatment. In the second study, there were autoimmune diseases in seven of 76 patients after a median of 19 months of treatment, including hypothyroidism in one, immune-mediated hemolysis in two, systemic lupus erythematosus in two, Raynaud s phenomenon in one, and mixed connective tissue disease in one (344). In... 

Wallace JR, Luchi M. Fatal cytomegalovirus pneumonia in a patient receiving corticosteroids and methotrexate for mixed connective tissue disease. South Med J 1996 89(7) 726-8. 

Penas PF, Buezo GF, Carvajal I, Dauden E, Lopez A, Diaz LA. D-penicillamine-induced pemphigus foliaceus with autoantibodies to desmoglein-1 in a patient with mixed connective tissue disease. J Am Acad Dermatol 1997 37(l) 121-3. 

A 37-year-old woman with mixed connective tissue disease took pentoxifylline 400 mg/day for Raynaud s... 

The interstitium of the kidney is also susceptible to injury from a variety of causes. Although acute interstitial nephritis is most commonly caused by medications (see Chap. 46), infections (e.g., streptococcal, leptospirosis, hantavirus, and human immimodeflciency virus), selected autoimmune disorders (systemic lupus erythematosus or mixed connective tissue disease) also may produce a similar syndrome. The presence of white blood cells (WBCs), WBC casts, and coarse granular casts in the urine aU suggest interstitial inflammation. The presence of eosinophUia and eosinophiluria also strongly suggest the presence of an interstitial nephritis. Occasionally low to moderate proteinuria can be seen on urinalysis. 

Extractable nuclear antigen Scleroderma Mixed connective tissue disease... 

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. 

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. 

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. 

Photosensitivity. Skin reddening due to an abnormal reaction to sunlight. A characteristic symptom of systemic autoimmune diseases (e.g. systemic lupus erythematosus, mixed connective tissue disease), cutaneous and subacute cutaneous lupus erythematosus. 

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. 

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). 

Nested case control 121 700 Connective tissue disease Five cases with BI were identified among 300 patients with RA 0 cases with BI among 123 with SLE, 20 patients with SS, 3 with Sjogren s syndrome, 13 with dermato/polymyositis, and 2 with mixed connective tissue disease. No association was found between BI and CTD. 

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). 

Silica-induced LE is a chronic multiorgan system autoimmune disease with frequent exacerbations and remissions similar to the natural course of mixed connective tissue disease. The pathomechanism of LE is not yet completely understood. Based on a genetic background, a major role seems to be played by disturbances of immune regulation, such as T-cell abnormalities, including T-cell cytokine network. 

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... 

UVR can also worsen autoimmune diseases such as chronic discoid lupus erythematosus and subacute and systemic lupus erythematosus, as well as mixed connective tissue diseases and dermatomyositis or other dermatoses, such as rosacea, etc. 

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. 

VIII. Mixed Connective Tissue Disease and Overlap Syndromes... 

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