Mixed connective tissue disease: To be or not to be?

Smolen, Josef S.; Steiner, Günter

doi:10.1002/1529-0131(199805)41:5<768::aid-art3>3.0.co;2-z

Cited by 146 publications

(39 citation statements)

References 77 publications

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“…First, we included both patients with MCTD and those with SSc. As mentioned in previous studies, most patients with MCTD-ILD have characteristics of SSc and ultimately develop the disease (4,15,32,33). Moreover, the progression of ILD in patients with SSc and MCTD exhibits similar disease behavior, which often stabilizes after a couple of years (9,31).…”

Section: Variables (N=22)mentioning

confidence: 99%

“…Moreover, patients with mixed connective tissue disease (MCTD) will likely suffer from ILD at some time (3), and these patients often have original features of SSc or develop SSc (4). Typically, the onset of SSc-ILD is insidious, with subtle clinical symptoms, which may explain why SSc-ILD is often diagnosed at an advanced stage, after extensive lung fibrosis is already present (5).…”

Section: Introductionmentioning

confidence: 99%

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Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease

Yamakawa¹,

Hagiwara²,

Kitamura³

et al. 2017

J. Thorac. Dis.

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Section: Variables (N=22)mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease

Yamakawa¹,

Hagiwara²,

Kitamura³

et al. 2017

J. Thorac. Dis.

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“…Antibodies to the ribosomal P0, P1 and P2 antigens or to the proliferating cell nuclear antigen are also highly specific for SLE. High levels of antibodies to U1 ribonuclearo proteins (RNP) without Sm, are found in MCTD [17]. Anti-Sm antibodies are directed predominantly against the SmB/B' and SmD proteins whereas anti-U1 RNP antibodies react with at least one of the three U1 snRNP-specific proteins, namely U1±70K, U1A and U1C [18].…”

Section: Linked Sets Of Autoantibodies In Nonorgan (Systemic) Autoimmmentioning

confidence: 99%

Key Sequences Involved in the Spreading of the Systemic Autoimmune Response to Spliceosomal Proteins

Monneaux

Muller

2001

Scand J Immunol

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Immune spreading to multiple intracellular antigens is likely to be of primary importance in organ-specific and systemic autoimmune diseases. A number of mechanisms by which immune spreading may occur from only a single autoreactive epitope have been proposed. Search for an initiator or early epitope thus represents an important area of investigation. For example, many studies have focused on the identification of epitopes recognized by the antibodies from both patients with systemic lupus erythematosus (SLE) and lupus-prone mice. Recently, an autoepitope present in the 70K U1 ribonucleo protein (RNP) and recognized by CD4 1 T cells from lupus mice has also been identified. Here, we analyze the results of B-and T-cell-epitope mapping studies of several RNPs present in the spliceosome and propose a model of epitope spreading. In this model, a consensus sequence (the RNP motif) conserved in many nuclear, nucleolar and cytoplasmic antigens, might play a role as`driver' epitope. This hypothesis is based on the observation that this sequence is recognized by CD4 1 T cells from lupus mice and is often targeted by autoantibodies, very early during the course of the disease. Targeting this region that is repeated in different self-antigens, might represent an interesting strategy to interfere with the continuous T-cell stimulation and exposure to specific antigens.

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“…Later, a high prevalence of arthritis resembling rheumatoid arthritis was observed in patients with MCTD. Pulmonary involvement is commonly observed in patients with MCTD (2)(3)(4)(5)(6). The prevalence of interstitial lung disease has been suggested to be as high as 67%, based on findings of high-resolution computed tomography (HRCT) and gadolinium diethylenetriaminepentaacetic acid scans.…”

mentioning

confidence: 99%

Treatment of pulmonary fibrosis for twenty weeks with imatinib mesylate in a patient with mixed connective tissue disease

Distler¹,

Manger²,

Spriewald³

et al. 2008

Arthritis & Rheumatism

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Interstitial lung disease, which is common in patients with mixed connective tissue disease (MCTD), can progress to severe pulmonary fibrosis. The tyrosine kinase inhibitor imatinib mesylate has recently been shown to prevent experimental pulmonary, dermal, and renal fibrosis. Our patient, a 64‐year‐old woman with MCTD and rapidly progressive pulmonary fibrosis, presented with rapid deterioration despite treatment with immunosuppressants. During 20 weeks of treatment with imatinib mesylate at 400 mg/day, our patient improved significantly according to several outcome measures, including New York Heart Association classification, diffusing capacity for carbon monoxide, 6‐minute walking distance, arterial oxygen pressure, and reduction of ground‐glass opacities seen on high‐resolution computed tomography. No adverse effects of imatinib mesylate were observed. These findings suggest that imatinib mesylate might be effective in patients with fibrotic diseases and warrant the initiation of larger clinical studies on the safety and efficacy of imatinib mesylate in connective tissue diseases.

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Mixed connective tissue disease: To be or not to be?

Cited by 146 publications

References 77 publications

Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease

Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease

Key Sequences Involved in the Spreading of the Systemic Autoimmune Response to Spliceosomal Proteins

Treatment of pulmonary fibrosis for twenty weeks with imatinib mesylate in a patient with mixed connective tissue disease

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