China
Africa
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Pregnancy in SLE
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
There are several forms of SLE-like illness.2,9,15Subclinical or atypical SLE is diagnosed when symptoms compatible with SLE, not fulfilling ARA criteria and unaccompanied by diagnostic serological tests, are present. Undifferentiated connective tissue disease is diagnosed when symptoms are present but the serological criteria for SLE (strongly positive anti-nuclear antibody, and anti-DNA or anti-Sm antibody) are absent. The diagnosis of mixed connective tissue disease is used when overlapping symptoms of SLE and scleroderma or dermatomyositis are present together with high titer antibody to U1RNP and absence of anti-DNA or anti-Sm. While these diagnoses imply different long-range prognoses for the patient, in pregnancy they are equivalent to SLE.
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Autoimmune or autoinflammatory diseases: JIA-particularly the systemic form.JDM.SLE.undifferentiated connective tissue disease.sarcoidosis.Behçet disease.
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
On the other hand, it is no longer required to have at least four items. For example, SLE joint involvement (weight 6) plus antibodies to dsDNA in a specific test (weight 6) will be sufficient for SLE classification (≥10). If it is a rather specific form of organ involvements, such as lupus nephritis, joint involvement, serositis, seizures, SLE-specific skin manifestations (acute or subacute or discoid lupus erythematosus), thrombocytopenia, or autoimmune hemolytic anemia, the combination with an SLE-specific antibody (to Sm or dsDNA) or with low C3 and low C4 will always be sufficient for classification in an ANA-positive patient. Indeed, in two cohorts of patients previously classified as undifferentiated connective tissue disease (UCTD) patients, more SLE patients were identified by the EULAR/ACR criteria [25,51].
Relapsing polychondritis: state-of-the-art review with three case presentations
Published in Postgraduate Medicine, 2021
Bogna Grygiel-Górniak, Hamza Tariq, Jacob Mitchell, Azad Mohammed, Włodzimierz Samborski
Case 1: A 46-year-old woman was suspected of undifferentiated connective tissue disease and the ears’ cartilage inflammation. Her first symptoms were in 2017 of both ears’ cartilage inflammation, which recurred in 2018 (Figure 1). Finally, in February 2019, she was admitted to the Rheumatology Department and diagnosed with RPC. In October 2019, she was diagnosed with undifferentiated connective tissue disease (UCTD) and treated with hydroxychloroquine. Additionally, Raynaud’s phenomenon was diagnosed and episodes of joint pain in both hands. Physical examination showed deformation of the auricles and arthritis of small joints of hands with thickening of the skin on the hands and slow wound healing after minor injuries. Serological tests showed elevated autoantibodies: PM 100, Th/To, and the slightly increased RNP/Sm, Sm, PCNA. An MRI of the cervical spine revealed degenerative disc disease. Capillaroscopy showed reduced capillary density and an increased number of twisting capillaries. Finally, the recurrent polychondritis with UCTD was confirmed. Since the clinical state was stable and no RPC progression was present, hydroxychloroquine (200 mg QD) treatment with occasionally taken diclofenac (50 mg QD) was maintained.
Podocyte infolding glomerulopathy with undifferentiated connective tissue disease: a case report
Published in Ultrastructural Pathology, 2020
Jiaoyu Shi, Rong Zheng, Hongyang Gao, Zhonghua Zhao, Huijuan Wu, Zhigang Zhang
Podocyte infolding glomerulopathy (PIG) is a rare type of glomerular disease characterized by the formation of microspheres and microtubules in thickened glomerular basement membrane (GBM). The term was first proposed by Joh et al in 2008, which was suggested as a new type of glomerular disease.1,2 In recent years, there have been more than 30 cases of PIG reported worldwide, all of them being from Asia.3–18 These reports suggested that PIG tended to be associated with autoimmune abnormalities, such as systemic lupus erythematous(SLE). Some scholars thought that PIG might be a transient morphological change in patients with other glomeruli diseases such as SLE and membranous nephropathy (MN). However, the mechanisms of PIG remain unclear. Therefore, more cases are needed for investigation. This study reported a PIG patient with undifferentiated connective tissue disease (UCTD), from China. The patient complained about repeated foam urine for about three months and was suspected as having UCTD. Electron microscopy (EM) examination revealed microsphere formation in an irregularly thickened GBM, which was diagnosed as PIG.