Undifferentiated connective tissue disease: state of the art on clinical practice guidelines

Antunes, Mónica; Scirè, Carlo Alberto; Talarico, Rosaria; Alexander, Tobias; Avčin, Tadej; Belocchi, Chiara; Doria, Andrea; Franceschini, Franco; Galetti, Ilaria; Govoni, Marcello; Hachulla, É.; Launay, David; Lepri, Gemma; Macieira, Carla; Matucci‐Cerinic, Marco; Montecucco, C.; Moraes-Fontes, Maria Francisca; Mouthon, Luc; Paolino, Sabrina; Ramoni, Vèronique; Tani, Chiara; Tas, Sander W.; Tincani, Anǵela; Vollenhoven, Ronald van; Zen, Margherita; Fonseca, João Eurico; Bombardieri, Stefano; Schneider, Matthias; Smith, Vanessa; Cutolo, Maurizio; Mosca, Marta; Beretta, Lorenzo

doi:10.1136/rmdopen-2018-000786

Cited by 53 publications

(42 citation statements)

References 9 publications

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“…Several conditions in the field of autoimmunity are characterized by non-specific signs and symptoms that cannot be classified into a definite nosological entity according to international criteria. An increasing number of patients have been referred to rheumatology consultation for chronic fatigue, myalgia, muscle weakness, arthralgia/arthritis, and interstitial lung disease [ 91 , 92 ]. The term ‘undifferentiated’ used to describe all these conditions not only reflects an undefined clinical picture but also a poor knowledge of the underlying etiopathogenic mechanisms.…”

Section: Classical Examples Of the Asia Syndromementioning

confidence: 99%

Classical Examples of the Concept of the ASIA Syndrome

et al. 2020

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Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) was first introduced in 2011 by Shoenfeld et al. and encompasses a cluster of related immune mediated diseases, which develop among genetically prone individuals as a result of adjuvant agent exposure. Since the recognition of ASIA syndrome, more than 4400 documented cases have been reported so far, illustrated by heterogeneous clinical manifestations and severity. In this review, five enigmatic conditions, including sarcoidosis, Sjögren’s syndrome, undifferentiated connective tissue disease, silicone implant incompatibility syndrome (SIIS), and immune-related adverse events (irAEs), are defined as classical examples of ASIA. Certainly, these disorders have been described after an adjuvant stimulus (silicone implantation, drugs, infections, metals, vaccines, etc.) among genetically predisposed individuals (mainly the HLA-DRB1 and PTPN22 gene), which induce an hyperstimulation of the immune system resulting in the production of autoantibodies, eventually leading to the development of autoimmune diseases. Circulating autonomic autoantibodies in the sera of patients with silicone breast implants, as well as anatomopathological aspects of small fiber neuropathy in their skin biopsies have been recently described. To our knowledge, these novel insights serve as a common explanation to the non-specific clinical manifestations reported in patients with ASIA, leading to the redefinition of the ASIA syndrome diagnostic criteria.

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Section: Classical Examples Of the Asia Syndromementioning

confidence: 99%

Classical Examples of the Concept of the ASIA Syndrome

et al. 2020

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“…Any known etiology of previous pregnancy loss: 1 Any known severe cardiac, hepatic, renal, hematological, or endocrinal diseases:…”

Section: Exclusion Criteriamentioning

confidence: 99%

“…Undifferentiated connective tissue disease (UCTD) is defined as at least one symptom or sign suggesting connective tissue diseases (CTDs) and with presence of at least one of auto-antibodies, while not fulfilling any classification criteria of a defined CTD [1,2]. UCTD accounts for 20-52% of patients with CTDs and is characterized by varying symptom onset time and antibody profiles.…”

Section: Introductionmentioning

confidence: 99%

A three-arm, multicenter, open-label randomized controlled trial of hydroxychloroquine and low-dose prednisone to treat recurrent pregnancy loss in women with undifferentiated connective tissue diseases: protocol for the Immunosuppressant regimens for LIving FEtuses (ILIFE) trial

Yang

et al. 2020

Trials

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Background Undifferentiated connective tissue disease (UCTD) is known to induce adverse pregnancy outcomes and even recurrent spontaneous abortion (RSA) by placental vascular damage and inflammation activation. Anticoagulation can prevent pregnancy morbidities. However, it is unknown whether the addition of immune suppressants to anticoagulation can prevent spontaneous pregnancy loss in UCTD patients. The purpose of this study is to evaluate the efficacy of hydroxychloroquine (HCQ) and low-dose prednisone on recurrent pregnancy loss for women with UCTD. Methods The Immunosuppressant for Living Fetuses (ILIFE) Trial is a three-arm, multicenter, open-label randomized controlled trial with the primary objective of comparing hydroxychloroquine combined with low-dose prednisone and anticoagulation with anticoagulation alone in treating UCTD women with recurrent spontaneous abortion. The third arm of using hydroxychloroquine combined with anticoagulant for secondary comparison. A total of 426 eligible patients will be randomly assigned to each of the three arms with a 1:1:1 allocation ratio. The primary outcome is the rate of live births. Secondary outcomes include adverse pregnancy outcomes and progression of UCTD. Discussion This is the first multi-center, open-label, randomized controlled trial which evaluates the efficacy of immunosuppressant regimens on pregnancy outcomes and UCTD progression. It will provide evidence on whether the immunosuppressant ameliorates the pregnancy prognosis in UCTD patients with RSA and the progression into defined connective tissue disease. Trial registration ClinicalTrials.gov NCT03671174. Registered on 14 September 2018.

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“…It is difficult to draw clear conclusions from these retrospective studies. Currently there is a lack of prospective data informing on the use of HCQ in undifferentiated mixed connective disease [43].…”

Section: Hcq In Undifferentiated Connective Tissue Diseasementioning

confidence: 99%

Hydroxychloroquine and the Prevention of Pregnancy Losses

Frishman

Radin

Cecchi

et al. 2019

COGO

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Pregnancy loss is a common and devastating pregnancy complication. Recurrent early miscarriage (REM) isdefined as two or more consecutive pregnancy losses during the first trimester of pregnancy. It is a distinct entity and in approximately 50% of these patients, the underlying cause is never established. REM can be idiopathic, i.e. of unknown cause, be related to infections, anatomical or chromosomal abnormalities and can also be related to the presence of autoimmune connective tissue diseases or antiphospholipid antibodies (aPL). Hydroxychloroquine (HCQ) is an antimalarial immunomodulator and is currently being investigated for its role in the prevention of idiopathic REM and REM related to antiphospholipid antibodies (aPL). In this article we review the evidence that exists to date regarding the use of HCQ in the setting of unexplained REM and REM in relation to connective tissue diseases and aPL and antiphospholipid syndrome (APS).

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Undifferentiated connective tissue disease: state of the art on clinical practice guidelines

Cited by 53 publications

References 9 publications

Classical Examples of the Concept of the ASIA Syndrome

Classical Examples of the Concept of the ASIA Syndrome

A three-arm, multicenter, open-label randomized controlled trial of hydroxychloroquine and low-dose prednisone to treat recurrent pregnancy loss in women with undifferentiated connective tissue diseases: protocol for the Immunosuppressant regimens for LIving FEtuses (ILIFE) trial

Hydroxychloroquine and the Prevention of Pregnancy Losses

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