More Evidence of Distinctive Features of Mixed Connective Tissue Disease

Hämeenkorpi, R; Ruuska, P; Forsberg, S; Tiilikainen, R; Mäkitalo, R; Hakala, M

doi:10.3109/03009749309095117

Cited by 33 publications

(8 citation statements)

References 18 publications

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“…All diagnostic criteria for mixed connective tissue disease (MCTD) require a positive ANA (antibodies to extractable nuclear antigens are always associated with a positive ANA) and thus defining sensitivity or specificity is impossible. In patients suspected of having MCTD, a positive ANA should prompt testing for antibodies to RNP (111–114).…”

Section: Indications For the Clinical Use Of The Ana Testmentioning

confidence: 99%

Evidence‐based guidelines for the use of immunologic tests: Antinuclear antibody testing

Solomon

Kavanaugh

Schur

2002

Arthritis & Rheumatism

458

276

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IntroductionThis article is part of a series on immunologic testing guidelines. The series introduction, published in this issue, outlines the full methodology for obtaining data, grading the literature, combining the information from multiple sources, and developing recommendations. Briefly, MEDLINE and Healthstar were searched using a variety of search terms, and all relevant available literature was reviewed. All articles were critically reviewed using published standards for studies of diagnostic tests. Test use was categorized as primarily diagnostic or prognostic (which also included monitoring). Information was extracted from each article to allow for calculation of a weighted average for sensitivity and specificity; likelihood ratios (LRs) were then derived from these values (positive LR ϭ sensitivity / [1-specificity]; negative LR ϭ [1-sensitivity] / specificity). Recommendations for use of tests were based on the LRs, where a test was considered to be "very useful" for a given disease if the weighted average positive LR was Ͼ 5 or negative LR was Ͻ 0.2. A test was considered "useful" if the weighted average positive LR was Ͼ 2 and Յ 5 or negative LR was Ͼ 0.2 and Յ 0.5. A test was considered "not useful" if the positive LR was Յ 2 or the negative LR was Ͼ 0.5. DefinitionAntinuclear antibodies (ANA) directed against a variety of nuclear antigens have been detected in the serum of patients with many rheumatic and nonrheumatic diseases, as well as in patients with no definable clinical syndrome. These antibodies can be detected using a variety of substrates and staining techniques as described below and are directed against many different nuclear antigens.

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Section: Indications For the Clinical Use Of The Ana Testmentioning

confidence: 99%

Evidence‐based guidelines for the use of immunologic tests: Antinuclear antibody testing

Solomon

Kavanaugh

Schur

2002

Arthritis & Rheumatism

458

276

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“…Aus diesen Gründen wurde verschiedentlich vorgeschlagen die Bezeichnung „undifferenzierte” Bindegewebserkrankung (UCTD) oder „Überlappungssyndrome” zu wählen [5, 6, 7, 8]. Andere Autoren wiederum verteidigen die ursprüngliche Bezeichnung als Krankheitsentität [9, 10, 11], wobei insbesondere in Betracht zu ziehen ist, daß auch die sog. klassischen Bindegewebserkrankungen wie SLE, SSc und DM/PM heterogen sind, Untergruppen aufweisen, und des weiteren Überlappungen untereinander nicht selten sind.…”

Section: Einführungunclassified

MCTD – Mixed Connective Tissue Disease

Haustein

2005

J Deutsche Derma Gesell

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Mixed connective tissue disease is a disease entity characterized by overlapping symptoms of lupus erythematosus (LE), systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA). Diagnostic criteria include high titers of antibodies against U1RNP as well as the presence of at least 3 of 5 of the following clinical features: edema of hands, synovitis, myositis, Raynaud phenomenon and acroscierosis. In terms of the pathogenesis, genetic as well as infectious (viral) factors appear to play a role. The acceptance of MCTD as a distinct disease entity is controversial. Terms such as "undifferentiated connective tissue disease" or "overlapping syndromes" are not helpful. One-quarter of MCTD patients transform into LE, while one-third progress to SSc. Therapeutic recommendations are glucocorticoids in combination with immunosuppressive agents and endothelin receptor antagonists. Double blind studies are not available. The prognosis is relatively good. Causes of death include pulmonary hypertension, infections and both pulmonary and cardiac failure.

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“…17 An increased frequency of HLA-DR4 Dw4 subtype has been detected in a subset of patients. 18 The hallmark of this disease is the presence of features of SLE, PSS, and PM, as described in other research. Three quarters of patients have swollen hands, sclerodactyly, dyspnea, Raynaud phenomenon, esophageal dysmotility, myositis, and arthralgias (Table 1), 19,20 although the arthritis is usually polyarticular and nondeforming, an erosive arthritis resembling rheumatoid arthritis has been reported.…”

Section: General Presentation Of Mctdmentioning

confidence: 71%

The Lung in Mixed Connective Tissue Disease

Girod¹,

Schwarz²

1999

Semin Respir Crit Care Med

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Mixed Connective Tissue Disease (MCTD) remains a controversial classification within the field of connective tissue diseases. Since its original description in 1972 by Sharp et al, different alternative descriptions appear in the literature, such as "overlap syndrome" or "undifferentiated connective tissue disease." The diagnosis is based on the presence of overlapping symptoms of systemic lupus erythematosus (SLE), systemic sclerosis (SS), and polymyositis (PM), and the detection of increased titers of anti-RNP (ribonucleoprotein) antibody. Sharp et al thought that these patients had a distinct rheumatological syndrome characterized by the absence of arthritis, renal disease, and pulmonary involvement, as well as a favorable prognosis. Further studies do not support this benign clinical course that spares the lung. Pulmonary abnormalities have been described in 25-85% of MCTD patients. The pulmonary manifestations are similar in presentation and severity as seen in PSS, SLE, and PM.The most common presentations include interstitial lung disease, pleural effusions, and pulmonary arterial hypertension. Less common pulmonary presentations are diffuse alveolar hemorrhage with isolated pulmonary capillaritis, rapidly progressive pulmonary arterial hypertension, small airway disease, pulmonary embolic disease, aspiration syndrome, and neuromuscular respiratory failure. The histologic descriptions of this lung involvement are sparse. Nevertheless, the need for lung biopsy should be determined on an individual basis and reserved for atypical presentations. Corticosteroids may be insufficient for the treatment of MCTD with pulmonary involvement. Early cytotoxic therapy has been advocated for interstitial lung disease and diffuse alveolar hemorrhage. This review will present the diagnosis, clinical features, controversies, pulmonary involvement, therapy, and prognosis of this relatively new disease.

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More Evidence of Distinctive Features of Mixed Connective Tissue Disease

Cited by 33 publications

References 18 publications

Evidence‐based guidelines for the use of immunologic tests: Antinuclear antibody testing

Evidence‐based guidelines for the use of immunologic tests: Antinuclear antibody testing

MCTD – Mixed Connective Tissue Disease

The Lung in Mixed Connective Tissue Disease

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