Speckled (particulate) epidermal nuclear IgG deposition in normal skin. Correlation of clinical features and laboratory findings in 46 patients with a subset of connective tissue disease characterized by antibody to extractable nuclear antigen

1978 ◽  
Vol 114 (5) ◽  
pp. 705-710 ◽  
Author(s):  
S. D. Prystowsky
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Normal Skin ◽  
Nuclear Antigen ◽  
Laboratory Findings ◽  
Extractable Nuclear Antigen

Mixed Connective Tissue Disease in Childhood

1986 ◽  
Vol 7 (10) ◽  
pp. 309-314
Author(s):  
Bernhard H. Singsen
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Rheumatic Diseases ◽  
Mixed Connective Tissue Disease ◽  
Overlap Syndrome ◽  
Point Of View ◽  
Nuclear Antigen ◽  
Extractable Nuclear Antigen ◽  
First Child ◽  
One Year

Rheumatic diseases with overlapping clinical features have been known since at least 1900 under such descriptive designations as "sclerodermatomyositis," "lupoderma," "rupus," and others. These hybrid descriptors recognize the occasional mixing of features of the classic rheumatic disorders, rheumatoid arthritis, scleroderma, dermatomyositis, and systemic lupus erythematous (SLE), in the same patient. However, because of the absence of precise pathogenetic and etiologic information, even the traditional rheumatic diseases have been largely classified on the basis of aggregations of clinical, histologic, and immunologic findings. In 1972, Sharp and colleagues first described 26 adults with a new syndrome, which they defined as mixed connective tissue disease (MCTD). A major contribution of Sharp and his colleagues was to name and attempt to classify an overlap syndrome in explicit clinical terms and to associate it with specific autoantibodies directed against "extractable nuclear antigen" (ENA). One year later, the first child with MCTD was reported, and in 1977 a series of 14 children with MCTD was first investigated. Where does mixed connective tissue disease, in both children and adults, stand now after more than a decade of study? Directly, the concept of MCTD has focused our attention upon the description of overlapping features of the rheumatic diseases, and it has forced us to confront whether "lumping" or "splitting" descriptors serves the patient from a therapeutic or prognostic point of view.

scholarly journals Evaluation of an Automated Screening Assay, Compared to Indirect Immunofluorescence, an Extractable Nuclear Antigen Assay, and a Line Immunoassay in a Large Cohort of Asian Patients with Antinuclear Antibody-Associated Rheumatoid Diseases: A Multicenter Retrospective Study

2018 ◽  
Vol 2018 ◽  
pp. 1-12 ◽  
Author(s):  
Seri Jeong ◽  
Hyunyong Hwang ◽  
Juhye Roh ◽  
Jung Eun Shim ◽  
Jinmi Kim ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Indirect Immunofluorescence ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Nuclear Antigen ◽  
University Hospitals ◽  
Screening Assay ◽  
Extractable Nuclear Antigen ◽  
Automated Screening

We assessed the diagnostic utility of the connective tissue disease (CTD) screen as an automated screening test, in comparison with the indirect immunofluorescence (IIF), EliA extractable nuclear antigen (ENA), and line immunoassay (LIA) for patients with antinuclear antibody- (ANA-) associated rheumatoid disease (AARD). A total of 1115 serum samples from two university hospitals were assayed using these four autoantibody-based methods. The AARD group consisted of patients with systemic lupus erythematosus (SLE), systemic sclerosis (SSc), Sjögren’s syndrome (SS), and mixed connective tissue disease (MCTD). The qualitative results of all four autoantibody assays showed a significant association with AARDs, compared to controls (P<0.0001 for all). The areas under the receiver operating characteristic curves (ROC-AUCs) of the CTD screen for differentiating total AARDs, SLE, SSc, SS, and MCTD from controls were 0.89, 0.93, 0.73, 0.93, and 0.95, respectively. The ROC-AUCs of combination testing with LIA were slightly higher in patients with AARDs (0.92) than those of CTD screen alone. Multivariate analysis indicated that all four autoantibody assays could independently predict AARDs. CTD screening alone and in combination with IIF, EliA ENA, and LIA are potentially valuable diagnostic approaches for predicting AARDs. Combining CTD screen with LIA might be effective for AARD patients.

Overlap/undifferentiated syndromes

2013 ◽  
pp. 1069-1078 ◽  
Author(s):  
Ariane Herrick
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Raynaud’S Phenomenon ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Overlap Syndromes ◽  
Clear Cut ◽  
Positive Treatment

Undifferentiated connective tissue disease (UCTD) and overlap syndromes both form part of the broad spectrum of connective tissue disease. They are difficult to define, as the boundaries between them and specific diseases such as systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and myositis are often not clear-cut. This chapter gives a broad overview of diagnosis, clinical features, outcomes, and management. Patients with UCTD have clinical and/or serological features of connective tissue disease but do not fulfil the criteria for any one defined disease. Raynaud’s phenomenon and puffy fingers are often the presenting features but there are many possible others, including arthralgia, sicca symptoms, and breathlessness due to pulmonary fibrosis, usually in the context of a positive anti-nuclear antibody (ANA). A proportion of patients evolve into a defined connective tissue disease: in those who do, this is generally within 5 years of onset. Treatment is dependent upon the clinical features: for examplem vasodilators for Raynaud’s phenomenon, or hydroxychloroquine for arthralgia/arthritis. Patients with overlap syndromes have features of more than one defined connective tissue disease. Overlap syndromes are therefore highly heterogeneous as many combinations of clinical and serological features can occur. Mixed connective tissue disease (MCTD) is the overlap syndrome that has been most described and includes overlapping features of SLE, SSc, and myositis in patients who are anti-U1 ribonucleoprotein (RNP) antibody positive. Treatment is of the specific clinical manifestations. Patients with overlap syndromes should be kept under regular review to allow early identification of internal organ involvement.

scholarly journals Association of Immunofluorescence pattern of Antinuclear Antibody with Specific Autoantibodies in the Bangladeshi Population

2015 ◽  
Vol 40 (2) ◽  
pp. 74-78 ◽  
Author(s):  
S Sharmin ◽  
S Ahmed ◽  
A Abu Saleh ◽  
F Rahman ◽  
MR Choudhury ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Lupus Erythematosus ◽  
Antinuclear Antibody ◽  
Research Work ◽  
Connective Tissue Disorder ◽  
Nuclear Antigen ◽  
Dot Blot ◽  
Serum Samples ◽  
Speckled Pattern ◽  
Extractable Nuclear Antigen

Antinuclear antibody (ANA) is useful in the diagnosis of connective tissue disorder (CTD). Association of specific autoantibodies with the immunofluorescence pattern of ANA in CTD, noted in western literature has been considered as reference in all over the world. However, in Bangladesh no such research work or data correlating the autoantibodies and their ANA patterns is found. Objective of the study was to identify an association between immunofluorescence patterns of antinuclear antibody on HEp-2 cell and more specific antinuclear reactivities (e.g. anti-dsDNA and anti-extractable nuclear antigen) in the serum samples of CTD patients. Serum samples of 152 CTD patients (Systemic lupus erythematosus, Rhumatoid arthritis, Sjogren´s syndrome, Systemic sclerosis, Polymyositis, Mixed connective tissue disease) were diagnosed clinically, attending at Bangabandhu Sheikh Mujib Medical University (BSMMU) during the study period of January, 2010 to December, 2010. Samples were subjected for ANA testing by Indirect Immunofluorescence (IIF) on HEp-2 cell (ALPHADIA) in dilution of 1:40, anti-dsDNA by ELISA and anti- extractable nuclear antigen (anti-ENA) by Dot Immunoblot. Dot blot strips were tested for anti-Sm, anti-RNP, anti-SSA/Ro, anti-SSB/La, anti-Scl-70 and anti-Jo-1. Out of 152 patients 110 (72.3%) cases were ANA positive by IIF on HEp-2 cell. ANA positive sera exhibited four fluorescence patterns such as speckled (50.8%), peripheral (21.6%) ,homogenous (18.1%) and nucleolar pattern (9%). Peripheral pattern and homogenous pattern was predominantly associated with anti-dsDNA (p<0.05). Speckled pattern was significantly associated with anti-ENA (p<0.05).The most commonly identified antinuclear autoreactivity was directed towards anti-RNP (25.7%) then anti-Scl-70 (20%), anti-SSA (14.2%) and anti-SSB (5.7%). Multiple anti-ENA reactivities were identified in 34.28% cases. Peripheral and homogenous pattern is strongly associated with anti-dsDNA and speckled pattern may predict anti-ENA (specially ribonucleoprotiens). As a definite correlation between the ANA patterns and the group of antibodies was detected by dot immunoblot, one could predict presence of certain specific auto antibodies for a particular ANA pattern identified. This may restrict on the cost of laboratory investigations in a developing country like Bangladesh. Thus, ANA-IIF method may reduce the expense of detailed immunological work-up with minimal loss in diagnostic accuracy.Bangladesh Med Res Counc Bull 2014; 40 (2): 74-78

scholarly journals Interleukin 2(IL-2) production in patients with mixed connective tissue disease(MCTD) and its relevance to the diagnosis and clinical features.

1988 ◽  
Vol 77 (5) ◽  
pp. 649-655
Author(s):  
Hisanori UMEHARA ◽  
Shunichi KUMAGAI ◽  
Hiroshi ISHIDA ◽  
Takashi FUJITA ◽  
Hiroo IMURA ◽  
...  
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Mixed Connective Tissue Disease ◽  
Interleukin 2

scholarly journals Association of extended myositis panel results, clinical features, and diagnoses: a single-center retrospective observational study

2021 ◽  
Author(s):  
Shamma Ahmad Al Nokhatha ◽  
Eman Alfares ◽  
Luke Corcoran ◽  
Niall Conlon ◽  
Richard Conway
Keyword(s):  
Connective Tissue ◽  
Connective Tissue Disease ◽  
Clinical Features ◽  
Clinical Utility ◽  
Clinical Symptoms ◽  
Final Diagnosis ◽  
Inflammatory Myopathies ◽  
Inclusion Criteria ◽  
Positive Group ◽  
Positive Results

AbstractMyositis-specific antibodies (MSA) and myositis-associated antibodies (MAA) are a feature of the idiopathic inflammatory myopathies (IIM), but are also seen in other rheumatic diseases, and in individuals with no clinical symptoms. The aim of this study was to assess the clinical utility of MSA and MAA and in particular the clinical relevance of weakly positive results. We included all patients at our institution who had at least one positive result on the Immunoblot EUROLINE myositis panel over a 6-year period (2015–2020). Associations with clinical features and final diagnosis were evaluated. Eighty-seven of 225 (39%) myositis panel tests met the inclusion criteria. There were 52 strong positives and 35 weak positives for one or more MSA/MAAs. Among the strong positive group, 15% (8/52) were diagnosed with IIM, 34.6% (18/52) with interstitial lung disease, 7.7% (4/52) with anti-synthetase syndrome, 25% (13/52) with connective tissue disease, and others accounted for 25% (13/52). In weak-positive cases, only 14% (5/35) had connective tissue disease and none had IIM. 60% (21/35) of weak-positive cases were not associated with a specific rheumatic disease. A significant number of positive myositis panel results, particularly weak positives, are not associated with IIM or CTD.

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