10 results on '"Mixed Connective Tissue Disease pathology"'
Search Results
2. A case of catastrophic antiphospholipid syndrome, which presented an acute interstitial pneumonia-like image on chest CT scan.
- Author
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Kameda T, Dobashi H, Susaki K, Danjo J, Nakashima S, Shimada H, Izumikawa M, Takeuchi Y, Mitsunaka H, Bandoh S, Imataki O, Nose M, and Matsunaga T
- Subjects
- Antiphospholipid Syndrome diagnostic imaging, Antiphospholipid Syndrome pathology, Female, Humans, Lung pathology, Lung Diseases, Interstitial diagnostic imaging, Lung Diseases, Interstitial pathology, Middle Aged, Mixed Connective Tissue Disease diagnostic imaging, Mixed Connective Tissue Disease pathology, Radiography, Antiphospholipid Syndrome complications, Lung diagnostic imaging, Lung Diseases, Interstitial etiology, Mixed Connective Tissue Disease complications
- Abstract
We report the case of catastrophic antiphospholipid syndrome (CAPS) complicated with mixed connective tissue disease (MCTD). A female patient was diagnosed with acute interstitial pneumonia (AIP) with MCTD by chest CT scan. Corticosteroid therapy was refractory for lung involvement, and she died due to acute respiratory failure. The autopsy revealed that AIP was compatible with lung involvement of CAPS. We therefore suggest that chest CT might reveal AIP-like findings in CAPS patients whose condition is complicated with pulmonary manifestations.
- Published
- 2015
- Full Text
- View/download PDF
3. ANCA-associated vasculitis with dual ANCA positivity in coexistence with mixed connective tissue disease.
- Author
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Murakami M, Shimane K, Takahashi H, Tomiyama J, and Nagashima M
- Subjects
- Adult, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis blood, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis genetics, Antibodies, Antineutrophil Cytoplasmic blood, Antibodies, Antineutrophil Cytoplasmic immunology, Azathioprine therapeutic use, Drug Therapy, Combination, Female, Genetic Predisposition to Disease, Glucocorticoids therapeutic use, HLA-DR Serological Subtypes genetics, Humans, Immunosuppressive Agents therapeutic use, Methylprednisolone therapeutic use, Mixed Connective Tissue Disease blood, Mixed Connective Tissue Disease genetics, Myeloblastin blood, Myeloblastin immunology, Peroxidase blood, Peroxidase immunology, Prednisolone therapeutic use, Pulse Therapy, Drug, Risk Factors, Treatment Outcome, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis complications, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis pathology, Mixed Connective Tissue Disease complications, Mixed Connective Tissue Disease pathology
- Abstract
We here report a rare case of dual antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a 38-year-old Japanese woman previously diagnosed with mixed connective tissue disease. The patient was found to be positive for myeloperoxidase- and proteinase 3-ANCA, and was diagnosed with AAV following admission to hospital with fervescence, polyarthralgia, purpura, and asymmetric numbness of the extremities. Examination of her genetic background revealed that she carried HLA-DR9, which confers risk of both diseases in Japanese populations.
- Published
- 2013
- Full Text
- View/download PDF
4. Subcutaneous calcification presenting in a patient with mixed connective tissue disease and cutaneous polyarteritis nodosa.
- Author
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Goolamali SI, Gordon P, Salisbury J, and Creamer D
- Subjects
- Adult, Calcinosis pathology, Female, Humans, Leg Dermatoses pathology, Mixed Connective Tissue Disease pathology, Polyarteritis Nodosa pathology, Calcinosis etiology, Mixed Connective Tissue Disease complications, Polyarteritis Nodosa complications
- Abstract
Subcutaneous calcification often occurs in connective tissue diseases, most commonly scleroderma and dermatomyositis, but is rarely found in mixed connective tissue disease (MCTD). Cutaneous polyarteritis nodosa (PAN) is usually a primary skin disorder and although associated with connective tissue disease, has not been reported previously in MCTD. Calcinosis in cutaneous PAN is not a recognized feature. We describe the case of a 37-year-old woman who presented with tender ulcerated subcutaneous nodules on the lower legs consistent with cutaneous PAN, and she also showed features of MCTD with extensive secondary subcutaneous calcification. The use of systemic immunosuppressive treatment has improved the clinical features of PAN and MCTD but treatment of the calcification has proved challenging. No single medical or surgical treatment has been shown to be consistently effective in subcutaneous calcification, but the introduction of diltiazem in our patient has resulted in some improvement.
- Published
- 2009
- Full Text
- View/download PDF
5. Human voltage-dependent anion selective channel 1 is a target antigen for antiglomerular endothelial cell antibody in mixed connective tissue disease.
- Author
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Kikuchi T, Yoshida Y, Morioka T, Gejyo F, and Oite T
- Subjects
- Autoantibodies analysis, Autoantibodies blood, Cells, Cultured, Electrophoresis, Gel, Two-Dimensional, Endothelium, Vascular chemistry, Endothelium, Vascular cytology, Glomerular Mesangium cytology, Glomerular Mesangium immunology, Humans, Kidney Glomerulus blood supply, Mixed Connective Tissue Disease blood, Mixed Connective Tissue Disease pathology, Proteomics, Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization methods, Autoantibodies immunology, Autoantigens immunology, Endothelium, Vascular immunology, Mixed Connective Tissue Disease immunology, Voltage-Dependent Anion Channel 1 immunology
- Abstract
The purpose of this study was to identify the endothelial cell antigens that react with circulating antiendothelial antibody (AECA) in mixed connective tissue disease (MCTD). We screened serum AECA reactivity in 23 patients with MCTD using a human glomerular endothelial cell (HGEC) cellular ELISA. Proteomics, two-dimensional gel electrophoresis and matrix-assisted laser desorption ionization time-of-flight (MALDI-TOF) mass spectrometry were used to identify the endothelial cell antigens of HGECs that reacted with serum antibodies from MCTD patients. Sera from 12 patients (52.0%) were positive for anti-HGEC antibody based on cellular ELISA. MALDI-TOF mass spectrometry used in combination with immunoblotting using serum antibody revealed one protein spot that represented a 36-kDa cell component of HGECs, with an isoelectric point (IP) of about 9, which had a high homology with the voltage-dependent anion-selective channel 1 (VDAC-1). This protein spot was confirmed to react with the antibody specific to VDAC-1. This is the first report of the presence of antibody to VDAC-1 from HGECs in the sera from MCTD patients. Although future studies will be needed to clarify the disease specificity of the a-VDAC-1 antibody in MCTD, the results show that modern proteomics technology is useful for identifying antigens that react with AECA in autoimmune diseases such as MCTD.
- Published
- 2008
- Full Text
- View/download PDF
6. Electron microscopy and capillaroscopically guided nailfold biopsy in connective tissue diseases: detection of ultrastructural changes of the microcirculatory vessels.
- Author
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von Bierbrauer A, Barth P, Willert J, Baerwald C, Mennel HD, and Schmidt JA
- Subjects
- Adult, Aged, Capillaries ultrastructure, Dermatomyositis diagnosis, Dermatomyositis pathology, Diagnosis, Differential, Female, Humans, Lupus Erythematosus, Systemic diagnosis, Lupus Erythematosus, Systemic pathology, Male, Middle Aged, Mixed Connective Tissue Disease diagnosis, Mixed Connective Tissue Disease pathology, Scleroderma, Systemic diagnosis, Scleroderma, Systemic pathology, Biopsy methods, Connective Tissue Diseases diagnosis, Connective Tissue Diseases pathology, Microscopy, Electron methods
- Abstract
The aims of the study were to describe and compare the frequency and nature of histologically detectable microvascular lesions in patients with various connective tissue diseases (CTD). An electron microscopic examination of specimens obtained by the technique of capillaroscopically guided nailfold biopsy was performed in 52 patients with CTD [nine systemic lupus erythematosus (SLE), eight mixed CTD, 18 scleroderma, 17 undifferentiated CTD] and 27 controls. The microvascular changes most frequently observed by electron microscopy were multilayering of the basal lamina (approximately 70% of the CTD patients), an increased amount of perivascular connective tissue, perivascular oedema formation, and an increased number of perivascular fibroblasts and mast cells (each in 30-37% of the CTD patients). In contrast, no particular histopathological feature was found in > 25% of the controls, multilayering (22.6%) being the most frequently observed. Comparing the different conditions studied, there were distinct differences in the frequency and nature of the histologically observed microvascular changes. In particular, SLE seems to be based on a separable type of vasculopathy consisting of significantly less frequent microvascular abnormalities. In conclusion, ultrastructural abnormalities of the microvascular system are a frequent finding in CTD. Electron microscopic examination of specimens obtained by capillaroscopically guided nailfold biopsy is able to disclose histopathological differences between defined entities. Therefore, this approach may be a useful tool to gain further insights into potentially separable aetiopathological mechanisms of the various types of CTD.
- Published
- 1998
- Full Text
- View/download PDF
7. Mixed connective tissue disease.
- Author
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Davis P and Adebajo AO
- Subjects
- Humans, Mixed Connective Tissue Disease pathology, Mixed Connective Tissue Disease physiopathology, Terminology as Topic, Mixed Connective Tissue Disease diagnosis
- Published
- 1993
- Full Text
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8. Mixed connective tissue disease--goodbye to all that.
- Author
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Black C and Isenberg DA
- Subjects
- Autoantibodies metabolism, Autoantigens immunology, Autoimmune Diseases epidemiology, Autoimmune Diseases immunology, Autoimmune Diseases pathology, Humans, Lupus Erythematosus, Systemic pathology, Prevalence, Prognosis, Ribonucleoprotein, U1 Small Nuclear immunology, Scleroderma, Systemic pathology, Mixed Connective Tissue Disease epidemiology, Mixed Connective Tissue Disease immunology, Mixed Connective Tissue Disease pathology
- Abstract
Since it was first described mixed connective tissue disease (MCTD) has been the subject of much debate. In particular the question of whether it is a truly distinctive disease entity has been challenged. It seems clear that the original description of MCTD as a mild disorder, rarely affecting the lungs or kidneys and requiring small doses of corticosteroids only, is no longer tenable. In this review a historical analysis of the clinical and serological features is presented. It is suggested that the concept of MCTD as a distinct disease entity is better replaced by the term 'undifferentiated autoimmune rheumatic/connective tissue disorder'. Many of these patients will later 'convert' into scleroderma or lupus; some will remain undifferentiated.
- Published
- 1992
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9. Defects of the retinal pigment epithelium in scleroderma.
- Author
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Kraus A, Guerra-Bautista G, Espinoza G, Barojas E, Quiroz-Mercado H, Sanchez-Echeverri G, and Alarcon-Segovia D
- Subjects
- Adult, Aged, Fluorescein Angiography, Fundus Oculi, Humans, Lupus Erythematosus, Systemic pathology, Male, Middle Aged, Mixed Connective Tissue Disease pathology, Retina pathology, Sjogren's Syndrome pathology, Pigment Epithelium of Eye pathology, Scleroderma, Systemic pathology
- Abstract
We completed ocular examination, including retinal fluoroangiography, in 19 unselected patients with diffuse systemic sclerosis (scleroderma) and compared the findings with those made in 50 consecutive patients with systemic lupus erythematosus, 18 with primary Sjögren's syndrome, 20 with mixed connective tissue disease, and 20 healthy women. Five of 19 scleroderma patients had atrophy of the retinal pigment epithelium (26.3%) while none of the controls and only four of the 88 (4.5%) patients with other connective tissue diseases (P less than 0.01) had this anomaly. Atrophy of the pigmented epithelium of the retina may occur in scleroderma as a result of damage of the choroidal plexus.
- Published
- 1991
- Full Text
- View/download PDF
10. Mixed connective tissue disease presenting as trigeminal neuropathy.
- Author
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Edmondstone WM, Shepherd TH, Price DK, and Gooddy WW
- Subjects
- Adult, Cranial Nerve Diseases etiology, Humans, Male, Mixed Connective Tissue Disease complications, Mixed Connective Tissue Disease pathology, Mixed Connective Tissue Disease diagnosis, Trigeminal Nerve pathology
- Published
- 1982
- Full Text
- View/download PDF
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