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Spinal Cord Disease
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
Inflammatory disorders: SLE.Sjögren's syndrome.Behçet's disease.Mixed connective tissue disease (MCTD).Scleroderma.Neurosarcoidosis.Idiopathic transverse myelitis.
Noninfectious Pulmonary Manifestations of Renal Disease In Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Stephen T. Lawless, H. Jorge Baluarte
Mixed connective tissue disease is characterized by an admixture of features resembling SLE, polymyositis, and scleroderma. Raynaud’s phenomenon, arthritis, sausage-shaped fingers, and various cutaneous manifestations are present. Laboratory findings of significance are antibody against ribonucleoprotein (RNP) and speckled antinuclear antibody. Renal manifestations (10 to 40% patients) include interstitial nephritis, membranous glomerulo-nephropathy (nephrosis) and proliferative glomerulonephritis (nephritis). Pulmonary manifestations are one of the worst and most common late complications in this disease but happen less often than they do in SLE. Response to corticosteroids is generally favorable, although severe renal involvement occasionally requires more aggressive therapy.
Sjögren syndrome and mixed connective tissue disease
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Mixed connective tissue disease (MCTD) was first described by Sharp et al. as a clinically and serologically distinct entity in 1972 [29]. Patients exhibit clinical features overlapping with those of systemic lupus erythematous, systemic sclerosis, and polymyositis, and have high titers of anti-U1RNP (ribonucleoprotein) antibody. However, there is controversy whether MCTD exists as a distinct clinical entity or is merely an overlap of clinical features of different connective tissue diseases. As a subset of patients with MCTD develops features consistent with classic systemic lupus or scleroderma over time, some researchers believe that it represents an early nonspecific phase of an evolving connective tissue disease (often referred to as undifferentiated connective tissue disease) [30–32]. MCTD is the least common of all the connective tissue disorders. A population-based study in Norway estimated its point-prevalence as 3.8 per 100,000 adults and the annual incidence rate as 2.1 per million. Females are affected more frequently than males (female-to-male ratios range from 3.3:1 to 16:1) [33–35]. Most patients are affected in their second or third decades of life. MCTD can have a juvenile onset in 7%–23% patients [36].
Assessing the EULAR/ACR classification criteria for patients with systemic lupus erythematosus
Published in Expert Review of Clinical Immunology, 2022
Martin Aringer, Karen Costenbader, Sindhu R Johnson
Mixed connective tissue disease (MCTD) has been a hotly disputed concept [58–63]. While we believe that MCTD is a meaningful disease entity of its own [64,65], the disease concept strongly relies on serology, namely the presence of higher titer antibodies to the 70 kDa U1RNP antigen [66]. Based on clinical and animal data, it is apparent that antibodies to U1RNP can also occur in SLE, but are then accompanied by specific SLE antibodies to Sm and/or dsDNA [67,68]. In contrast to this concept of MCTD and SLE, the pediatric lupus study by Dr. Smith and colleagues, for one example, considers a patient with positive anti-dsDNA antibodies and serositis to be misclassified as SLE and actually have MCTD [27]. We would rather argue that the EULAR/ACR criteria correctly classified SLE in this patient, based on both autoantibody and clinical features.
Two patients with mixed connective tissue disease complicated by pulmonary arterial hypertension showing contrasting responses to pulmonary vasodilators
Published in Modern Rheumatology Case Reports, 2020
Katsuhide Kusaka, Kazuhisa Nakano, Shigeru Iwata, Satoshi Kubo, Tomoya Nishida, Yoshiya Tanaka
Mixed connective tissue disease (MCTD) was proposed by Sharp et al. [1] in 1972. It clinically involves at least 2 of the following 3 manifestations: systemic lupus erythematosus (SLE)-like manifestation, systemic sclerosis (SSc)-like manifestation, and polymyositis/dermatomyositis (PM/DM)-like manifestation. Serologically, it is characterised by positivity for only high-titer anti-U1-ribonucleoprotein (U1-RNP) antibody. This disease is associated with a wide range of organ dysfunctions. Raynaud’s phenomenon and finger swelling are physical findings that aid in its diagnosis. In this disease, pulmonary arterial hypertension (PAH) is the most important organ dysfunction that defines prognosis. PAH is a prognostic factor for not only MCTD but also SLE and SSc. On comparing PAH with idiopathic pulmonary arterial hypertension (IPAH) without any underlying diseases, such as connective tissue diseases (CTDs), PAH might be more responsive to immunosuppressive therapy as well as pulmonary vasodilators. PAH is one of the disease types of broadly defined pulmonary hypertension (PH), and its recommended therapies differ from those for other types of PH. CTDs, such as MCTD, are complicated by multiple organ dysfunctions because of their various clinical pathological conditions. Thus, it is necessary to appropriately diagnose the disease type of PH, classify the severity, and select treatment accordingly. Here, we present 2 cases of MCTD complicated by PH that had contrasting clinical courses.
2019 Diagnostic criteria for mixed connective tissue disease (MCTD): From the Japan research committee of the ministry of health, labor, and welfare for systemic autoimmune diseases
Published in Modern Rheumatology, 2021
Yoshiya Tanaka, Masataka Kuwana, Takao Fujii, Hideto Kameda, Yoshinao Muro, Keishi Fujio, Yasuhiko Itoh, Hidekata Yasuoka, Shusaku Fukaya, Konomi Ashihara, Daisuke Hirano, Koichiro Ohmura, Yuya Tabuchi, Hisanori Hasegawa, Ryo Matsumiya, Yuichiro Shirai, Takehisa Ogura, Yumi Tsuchida, Mariko Ogawa-Momohara, Hidehiko Narazaki, Yoshino Inoue, Ippei Miyagawa, Kazuhisa Nakano, Shintaro Hirata, Masaaki Mori
In 1972, Sharp et al. proposed mixed connective tissue disease (MCTD) as a disease entity characterized by overlapping clinical features of systemic lupus erythematosus (SLE), systemic sclerosis, and polymyositis, as well as high titers of serum anti-U1 ribonucleoprotein (U1-RNP) antibody [1]. Although MCTD was previously considered a subtype of systemic sclerosis, it has recently been recognized as an independent disease entity in terms of organ involvement because of its characteristic association with conditions such as pulmonary arterial hypertension, aseptic meningitis, and trigeminal neuropathy [2–9].