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    Scleroderma overlap syndromes.
    Adv Exp Med Biol 1999 ;455:85-92
    Department of Dermatology, Warsaw School of Medicine, Poland.
    The most common scleroderma overlap syndromes are mixed connective tissue disease (MCTD), scleromyositis and synthetase syndrome. There is controversy concerning MCTD as a separate entity due to heterogeneous clinical manifestations, not infrequent transformation into definite CTD and various classification criteria. Our study of 94 adult patients and 20 children, classified according to the criteria of Alarcon-Segovia, and especially a 5, 9-year follow-up showed transformation into SLE or SSc in over 20% of patients, less frequently than reported by others, whereas over half of the cases remained undifferentiated CTD. In several cases ARA criteria for both SSc and SLE were fulfilled, and there is no consensus whether such cases should be recognized as coexistence of both definite diseases or as MCTD. High titers of U1 RNP antibodies to 70 kD epitope were invariably present, whereas, by transformation into distinctive CTD there appeared, in addition, antibodies characteristic of these CTD. Of 108 cases positive for PM-Scl antibody, 83% were associated with scleromyositis. This scleroderma overlap syndrome differed from MCTD by coexistent features of dermatomyositis (myalgia, myositis, Gottron sign, heliotrope rash, calcinosis) with no component of SLE, characteristic of MCTD. The course was also chronic and rather benign, as in MCTD, and all cases responded to low or moderate doses of corticosteroids. A not infrequent complication was deforming arthritis of the hands. Our immunogenetic study showed an association of cases positive for PM-Scl antibody with HLA-DQA1x0501 alleles in 100% and with HLA-DRB1x0301 in 94% of cases. Synthetase syndrome, associated with anti-histidyl-tRNA synthetase antibodies, studied in 29 patients with myositis and interstitial lung disease (ILD), only in single cases had scleroderma-like features. These cases differed from SSc by acute onset with fever, and by response to moderate doses of corticosteroids. We also studied overlap of localized scleroderma with other CTD: 21 cases of progressive facial hemiatrophy and linear scleroderma, and 55 (39.5%) of atrophoderma Pasini-Pierini (APP) and morphea. As in other autoimmune disorders, two or more connective tissue diseases (CTD) may develop concurrently or sequentially in the same patient. In such overlap syndromes ARA criteria must be fulfilled for each of the disease, and the clinical presentation has features of both. However more frequently overlap syndromes only combine some manifestations of more than one CTD, and present a highly heterogeneous group of disorders with prevailing clinical features of SSc.

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    The HLA profiles of mixed connective tissue disease differ distinctly from the profiles of clinically related connective tissue diseases.
    Rheumatology (Oxford) 2015 Mar 3;54(3):528-35. Epub 2014 Sep 3.
    Department of Medical Genetics, University of Oslo and Oslo University Hospital Ullevål, Rheumatology Unit, Department of Immunology, Oslo University Hospital Rikshospitalet, Oslo and Institute of Clinical Medicine, University of Oslo, Oslo, Norway. Department of Medical Genetics, University of Oslo and Oslo University Hospital Ullevål, Rheumatology Unit, Department of Immunology, Oslo University Hospital Rikshospitalet, Oslo and Institute of Clinical Medicine, University of Oslo, Oslo, Norway.
    Objective: The Norwegian nationwide MCTD cohort was established to obtain unbiased data on key disease issues, and thereby reappraise the concept of MCTD. In the current study, the aims were to obtain detailed HLA profile data on the large Norwegian MCTD cohort and compare these with the HLA profiles of ethnically matched healthy controls and related CTD controls.

    Methods: HLA profiles, determined by sequence-based typing of HLA-B* and DRB1*, were compared between four control groups of Norwegian ancestry, SLE (n = 96), SSc (n = 95), PM/DM (n = 84), healthy individuals (n = 282), the complete MCTD cohort (n = 155) and MCTD subsets defined by key clinical parameters. Read More
    Overlapping syndromes, undifferentiated connective tissue disease, and other fibrosing conditions.
    Curr Opin Rheumatol 1993 Nov;5(6):809-15
    Department of Clinical Immunology, University Hospital Groningen, The Netherlands.
    Connective tissue diseases (CTDs) frequently present as undifferentiated disorders that may or may not develop into one of the well defined CTDs. The natural evolution of the early undifferentiated CTDs is not well known. Some years ago a cooperative study was started on 410 patients with early CTD; within this group, 48 patients had early scleroderma. Read More
    Myositis overlap syndromes.
    Curr Opin Rheumatol 1999 Nov;11(6):468-74
    Centre for Rheumatology, Department of Medicine, University College London, UK.
    Myositis-overlap syndromes are characterized by a heterogeneous group of clinical syndromes of which many are closely linked with specific autoantibodies. Clinical and laboratory-based research into this fascinating group of syndromes have given us clues in elucidating the role of immunogenetic, environmental influences on the etiopathogenesis of autoimmune disease. Patients positive for anti-snU1 RNP antibody often present with a constellation of "autoimmune-related" clinical features, often not fulfilling the diagnostic criteria for a single disease entity. Read More
    Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD).
    Clin Exp Rheumatol 2003 May-Jun;21(3):313-20
    Division of Immunology, 1st Department of Medicine, University of Debrecen, Medical and Health Science Center, Debrecen, Hungary.
    Objective: To determine the clinical symptoms and the panel of autoantibodies of patients with early undifferentiated connective tissue disease (UCTD) followed for at least 1 year.

    Methods: 716 UCTD patients with manifestations suggestive but not diagnostic of specific connective tissue disease (CTD) were recruited and followed up between 1994-1999. The patients with early UCTD were subdivided into those with isolated Raynaud's phenomenon (RP) (50 patients), unexplained polyarthritis (31 patients) and "true" UCTD (665 patients). Read More