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Mixed Connective Tissue Disease (mixed + connective_tissue_disease)

Distribution by Scientific Domains
Medical Sciences100%

Selected Abstracts

Cutaneous vasculitis: a review

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 3 2003
A. Neil Crowson
As the skin is commonly involved in systemic vasculitic disorders as well as those hypersensitivity states whose expression is largely skin-confined, cutaneous vasculitic lesions offer a window to diagnosis and a ready source of accessible tissue for biopsy. In this review, we discuss the pathologic manifestations of chronic vasculitic syndromes such as granuloma faciale and erythema elevatum diutinum; IgA-associated vasculitis including Henoch-Schonlein purpura; vasculitis seen in the setting of cryoglobulinemia and hypergammaglobulinemia of Waldenstrom, hereditary deficiencies of complement, and IgA deficiency; those leukocytoclastic vasculitides resulting from hypersensitivity reactions to drug, chemical and foodstuff ingestion; and those vasculitides seen in patients with systemic diseases such as polyarteritis nodosa, rheumatoid arthritis, mixed connective tissue disease, systemic lupus erythematosus, Sjogren's syndrome, relapsing polychondritis, Behcet's disease, Wegener's granulomatosis, and allergic granulomatosis of Churg and Strauss. [source]

Desquamative gingivitis: retrospective analysis of disease associations of a large cohort

ORAL DISEASES, Issue 6 2008
JC Leao
Background:, Desquamative gingivitis (DG) is usually a manifestation of immunologically mediated mucocutaneous disorders, although it was previously suggested to be hormonally related. Methods:, One hundred and eighty-seven Caucasian UK residents with clinical features of DG (126 female, median age of 51 years, range 23,93 years) were retrospectively evaluated. Results:, It was established that, in this population, the largest cohort yet reported, oral lichen planus was most common (70.5%) while mucous membrane pemphigoid (14%), pemphigus vulgaris (13%), linear IgA disease (1.6%), dermatomyositis (0.5%) and mixed connective tissue disease (0.5%) were less common. Conclusion:, Oral lichen planus is the main disorder associated with DG. However, DG may be a feature of bullous disease and connective tissue disease. [source]

A murine model of mixed connective tissue disease induced with U1 small nuclear RNP autoantigen

ARTHRITIS & RHEUMATISM, Issue 2 2006
Eric L. Greidinger
Objective To test whether immunizing mice with autoantigens closely linked to mixed connective tissue disease (MCTD) could induce an MCTD-like clinical syndrome distinguishable from systemic lupus erythematosus (SLE). Methods Transgenic and knockout C57BL/6-derived mice were immunized subcutaneously at age 8,12 weeks with U1,70-kd small nuclear RNP (70K) fusion protein along with either Freund's complete adjuvant (CFA) or U1 RNA. After 2 months, mice were killed and analyzed histologically and serologically. Results Immunization of C57BL/6-derived mice transgenic for human HLA,DR4 with 70K and either CFA or U1 RNA led to anti-70K antibodies in 62% of mice (21 of 34), and diversified anti-RNP immune responses. MCTD-like lung disease also developed in 50% of immunized mice (17 of 34), and anti-70K antibodies were strongly correlated with lung disease. CFA and U1 RNA were comparably able to induce this syndrome. Mice deficient in Toll-like receptor 3 (TLR-3) also developed this same syndrome when immunized with 70K and CFA. However, TLR-3,/, mice failed to develop MCTD-like lung disease when treated with 70K and U1 RNA. Rather, TLR-3,/, mice immunized with 70K and U1 RNA developed an autoimmune syndrome characterized by glomerulonephritis typical of SLE. Conclusion Exposure to 70K in an appropriate context is sufficient to induce autoimmunity and target organ injury consistent with MCTD. This system represents a new model of autoimmune interstitial lung disease, and establishes a closer link between anti-70K immunity and MCTD-like lung disease. Of note, changes in innate immune signaling can cause the same trigger to lead to the development of SLE-like nephritis rather than MCTD-like lung disease. [source]