“Mixed connective tissue disease”: a condition in search of an identity

Alves, Marta Rebocho; Isenberg, David

doi:10.1007/s10238-020-00606-7

Cited by 34 publications

(30 citation statements)

References 36 publications

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“…Though anti-U1RNP was present, she had no qualifying features for a mixed connective tissue disease, such as Raynaud’s phenomenon, puffy fingers, arthritis, myositis, pulmonary hypertension, or interstitial lung disease. Some authors firmly believe that mixed connective tissue disease is not different from UCTD, and it is a precursor of systemic sclerosis [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Diagnosis of Undifferentiated Connective Tissue Disease in a Patient With Digital Gangrene and Positive Antinuclear Antibodies

Ravi¹,

Thabah²,

Verghese³

et al. 2021

Cureus

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The occurrence of ischemia of the digits or digital gangrene is a well-known complication of systemic autoimmune diseases, such as systemic sclerosis, systemic lupus erythematosus, and anti-phospholipid syndrome, among others. The pathophysiological mechanisms are small vessel vasculitis, vasospasm of Raynaud's phenomenon, microthrombi due to antiphospholipid syndrome, and/or accompanying accelerated atherosclerosis. Digital ischemia can also occur in the context of disseminated bacterial infections and sepsis. We present here the case of a patient who had digital ischemia and positive antinuclear antibodies but without well-defined clinical features of a connective tissue disease. A diagnosis of undifferentiated connective tissue disease was made.

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Section: Discussionmentioning

confidence: 99%

Diagnosis of Undifferentiated Connective Tissue Disease in a Patient With Digital Gangrene and Positive Antinuclear Antibodies

Ravi¹,

Thabah²,

Verghese³

et al. 2021

Cureus

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“…Such discrepant findings leave a debate on the natural course of MCTD-ILD, and further cohort studies at a larger scale are needed. Together with ILD, PAH is a major prognostic factor of MCTD [ 125 , 126 ]. The Norwegian population-based prevalence of pulmonary hypertension was reported to be less than 5% over 5.6 years [ 125 ], but PAH has been recognized as the leading cause of death in patients with MCTD, explaining 41% of all deaths [ 126 ].…”

Section: Pulmonary Manifestations Of Other Rheumatic Diseasesmentioning

confidence: 99%

Pharmacological Interventions for Pulmonary Involvement in Rheumatic Diseases

Kang

Song

2021

Pharmaceuticals

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Among the diverse forms of lung involvement, interstitial lung disease (ILD) and pulmonary arterial hypertension (PAH) are two important conditions in patients with rheumatic diseases that are associated with significant morbidity and mortality. The management of ILD and PAH is challenging because the current treatment often provides only limited patient survival benefits. Such challenges derive from their common pathogenic mechanisms, where not only the inflammatory processes of immune cells but also the fibrotic and proliferative processes of nonimmune cells play critical roles in disease progression, making immunosuppressive therapy less effective. Recently, updated treatment strategies adopting targeted agents have been introduced with promising results in clinical trials for ILD ad PAH. This review discusses the epidemiologic features of ILD and PAH among patients with rheumatic diseases (rheumatoid arthritis, myositis, and systemic sclerosis) and the state-of-the-art treatment options, focusing on targeted agents including biologics, antifibrotic agents, and vasodilatory drugs.

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“…There is no consensus on the treatment of mixed connective tissue disease (MCTD) or undifferentiated connective tissue disease (UCTD), which is related to the lack of commonly approved classification criteria for these diseases and, as a result, the lack of RCT studies. Most authors, however, recommend the use of non-steroidal anti-inflammatory drugs, HCQ and low doses of systemic glucocorticosteroids in both diseases [52][53][54][55]. The effectiveness of HCQ in slowing the progression of UCTD to SLE was suggested based on a retrospective analysis [55].…”

Section: Mechanizm I Efekty Działaniamentioning

confidence: 99%

“…Niestety nie ma konsensusu dotyczącego leczenia mieszanej choroby tkanki łącznej (mixed connective tissue diseases -MCTD) i niezróżnicowanej choroby tkanki łącznej (undifferentiated connective tissue diseases -UCTD), co powiązane jest z brakiem powszechnie uznanych kryteriów klasyfikacyjnych tych schorzeń i w rezultacie brakiem badań RCT. Większość autorów zaleca jednak stosowanie niesteroidowych leków przeciwzapalnych, HCQ i niskich dawek glikokortykosteroidów ogólnie w obu jednostkach chorobowych [52][53][54][55]. Skuteczność HCQ w spowolnieniu progresji UCTD do SLE sugerowano na podstawie analizy retrospektywnej [55].…”

Section: Mechanizm I Efekty Działaniaunclassified

The place of hydroxychloroquine in modern dermatology

Dańczak‐Pazdrowska¹,

Polańska²

2021

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Hydroxychloroquine has been known since the 1940s. It was initially used to treat malaria. Currently, it has a well-established position in the treatment of lupus erythematosus. The knowledge about its benefits is increasing over time. The mechanism of action of hydroxychloroquine reaches far beyond the immunomodulatory effect, and includes a beneficial effect on glycemic control in patients with diabetes and it is believed to have an effect on normalization of lipid levels, including total cholesterol, LDL cholesterol and triglycerides. In treatment of lupus erythematosus hydroxychloroquine allows decreasing the dose of immunosuppressive drugs, thus not only increasing the treatment efficacy, but also reducing the risk of adverse effects. Possible indications for hydroxychloroquine include also granulomatous skin diseases, such as disseminated annular granuloma, cutaneous sarcoidosis, or reactive granulomatous dermatitis. This article discusses the current indications for hydroxychloroquine with focus on recommended therapeutic regimens and monitoring treatment. streszczenie Hydroksychlorochina jest lekiem znanym od lat 40. ubiegłego wieku. Początkowo stosowano ją w leczeniu malarii, a później tocznia rumieniowatego. Obecnie ma ugruntowaną pozycję w terapii tego ostatniego schorzenia. Jednocześnie znanych jest coraz więcej korzyści płynących ze stosowania leku. Wykraczają one daleko poza działanie immunomodulujące, obejmując liczne aspekty zespołu metabolicznego, a wdrożenie hydroksychlorochiny do leczenia umożliwia ograniczenie dawek leków immunosupresyjnych, nie tylko zwiększając skuteczność, lecz także zmniejszając ryzyko wystąpienia działań niepożądanych. We współczesnej dermatologii, poza schorzeniami z kręgu autoimmunizacyjnych chorób tkanki łącznej, coraz częściej rekomendowana jest w innych chorobach, np. ziarniniakowych. W pracy omówiono aktualne wskazania do leczenia hydroksychlorochiną, zwracając szczególną uwagę na zalecane schematy terapeutyczne i postępowanie monitorujące leczenie, mające poprawiać bezpieczeństwo stosowania leku.

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“Mixed connective tissue disease”: a condition in search of an identity

Cited by 34 publications

References 36 publications

Diagnosis of Undifferentiated Connective Tissue Disease in a Patient With Digital Gangrene and Positive Antinuclear Antibodies

Diagnosis of Undifferentiated Connective Tissue Disease in a Patient With Digital Gangrene and Positive Antinuclear Antibodies

Pharmacological Interventions for Pulmonary Involvement in Rheumatic Diseases

The place of hydroxychloroquine in modern dermatology

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