Myxovirus Resistance Protein a Is a Useful Additional Histological Marker for Cutaneous Lupus Erythematosus
Wietske M. LambersKarina de LeeuwFemke M HomanBerber Doornbos‐van der MeerHendrika BootsmaJohanna WestraGilles F.H. Diercks
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Cutaneous Lupus Erythematosus
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Blistering in systemic lupus erythematosus has been divided into three groups. A specific subgroup of 'bullous systemic lupus erythematosus' has been defined by Gammon et al. on the basis of a number of criteria. From our experience of seven patients with bullous systemic lupus erythematosus, and after reviewing the literature, we suggest that the current classification is too narrow. Our patients displayed clinical and immunohistological (based on direct and indirect immunofluorescence and Western immunoblotting) heterogeneity. Sera from two patients bound to epidermal epitopes in sodium chloride-split skin, but immunoblotting was negative. In neither of these patients could the target antigen be type VII collagen, the only antigen identified as pathogenic in this disease. Patients with epidermal binding should not be excluded from a diagnosis of bullous systemic lupus erythematosus. SLE is a disease in which there is a genetic predisposition to form antibodies to type VII collagen, along with other autoantibodies, many of which may be implicated in blistering. We suggest that the criteria for the diagnosis of BSLE should be revised. We define this disease as an acquired subepidermal blistering disease in a patient with SLE, in which immune reactants are present at the basement membrane zone on either direct or indirect immunofluorescence.
Immunofluorescence
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The presence of a wide variety of autoantibodies is a characteristic finding in systemic lupus erythematosus. Autoantibodies against nuclear proteins, such as anti-nuclear and anti-double-stranded DNA antibodies, are used as diagnostic markers in systemic lupus erythematosus. Renal involvement is frequently found in systemic lupus erythematosus and is an important risk factor for death. Therefore, markers for the diagnosis and follow-up of nephritis are very important. Anti-C1q autoantibodies are strongly associated with renal involvement in systemic lupus erythematosus. This study will review recent findings on the pathogenic role and clinical importance of anti-C1q antibodies in lupus nephritis.Recent clinical studies have clearly emphasized the diagnostic relevance of anti-C1q autoantibody levels in patients with lupus nephritis. With a possible negative predictive value of 100%, anti-C1q autoantibodies are the only exclusive antibodies associated with the involvement of a single organ in systemic lupus erythematosus. Next to the clinical findings, the pathogenic significance of anti-C1q antibodies has been shown in an animal model. The deposition of autologous C1q in healthy glomeruli of mice after the infusion of anti-C1q antibodies induces moderate tissue damage.The latest insight into the pathogenesis of anti-C1q autoantibodies in the development of lupus nephritis and the recently demonstrated clinical importance of anti-C1q autoantibodies for the diagnosis of lupus nephritis support the value of further investigations. New diagnostic methods for the detection of anti-C1q and an accurate follow-up of antibody levels might be of use in clinical practice.
Clinical Significance
Nephritis
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We studied the autoimmune serologic features and histocompatibility antigen associations of 27 patients who had a widespread, nonscarring and often photosensitive form of histologically specific cutaneous lupus erythematosus. We designated this disorder as subacute cutaneous lupus erythematosus. Skin lesions from this disorder can be distinguished from scarring discoid lupus erythematosus lesions both on a morphologic and histopathologic basis. Antinuclear and anticytoplasmic antibodies (Ro or Ro and La) and circulating immune complexes were frequently present in patients with subacute cutaneous lupus erythematosus, whereas rheumatoid factor and anti-lymphocyte, anti-DNA, anti-nRNP, and anti-Sm antibodies were found less frequently. Patients having annular skin lesions represented a particularly homogeneous subgroup in which there was a striking concordance of anti-Ro antibodies and the HLA-DR3 phenotype. These studies clearly establish that the presence of these lesions can serve as a cutaneous marker for a distinct subset of patients with lupus erythematosus who share similar clinical, serologic, and genetic features.
Discoid lupus erythematosus
Rheumatoid factor
Concordance
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Fifty-five patients with biopsy-proven cutaneous lupus erythematosus (LE) were identified in whom a prospective and retrospective review of the clinical and laboratory data allowed subclassification into systemic (SLE), subacute (SCLE), or discoid (DLE) variants. In addition to conventional direct immunofluorescence, an indirect immunofluorescent technique, using a monoclonal antibody, was employed to assess deposition of the membranolytic attack complex (C5b-9) in skin lesions. Deposition of C5b-9 within the epidermis correlated with a diagnosis of SCLE with or without antibodies to Ro and was seen in SLE patients with antibodies to extractable nuclear antigens Ro, La, Sm, and RNP, and in DLE patients with positive antinuclear antibodies and/or extracutaneous manifestations. In the SLE group, vascular C5b-9 deposition was present in six patients. Of these, four had circulating lupus anticoagulant, one had lymphocytic vasculitis, and two had antibodies to Ro. In two patients with SLE there was keratinocyte decoration for immunoglobulin G but not for C5b-9, in the absence of seropositivity for antibodies to Ro, La, Sm, and ribonucleoprotein (RNP). The immunohistological examination of skin lesions using a monoclonal antibody to C5b-9 is a valuable adjunct in the subclassification of LE. The presence of C5b-9 within skin lesions of patiens with LE implies a pathogenic role for complement-mediated pore formation.
Discoid lupus erythematosus
Immunofluorescence
Skin biopsy
Lupus anticoagulant
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In human patients with systemic lupus erythematosus, cutaneous subepidermal blistering can occur because of the production of antibodies specific for basement membrane antigens. This condition is referred to as bullous systemic lupus erythematosus (BSLE). A dog was diagnosed with BSLE because it because of the production of antibodies specific for basement membrane antigens. This condition is referred to as bullous systemic lupus erythematosus (BSLE). A dog was diagnosed with BSLE because it fulfilled the following criteria: (i) a diagnosis of systemic lupus erythematosus by standard methods; (ii) an acquired, vesicular, erosive and ulcerative eruption; (iii) microscopical subepidermal vesicles with neutrophil‐predominant inflammation at the dermo‐epidermal junction; (iv) deposition of IgG at the epidermal basement membrane zone; and (v) circulating IgG autoantibodies against type VIl collagen. Anti‐collagen Vil type l‐BSLE therefore needs to be considered as a possible differential diagnosis for canine autoimmune subepidermal blistering diseases.
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Serum rheumatologic tests are generally most useful for confirming a clinically suspected diagnosis. Testing for rheumatoid factor is appropriate when rheumatoid arthritis, Sjögren's syndrome or cryoglobulinemia is suspected. Antinuclear antibody testing is highly sensitive for systemic lupus erythematosus and drug-induced lupus. Anti-double-stranded DNA antibodies correlate with lupus nephritis; the titer often corresponds with disease activity in systemic lupus erythematosus. Testing for anti-Ro (anti-SS-A) or anti-La (anti-SS-B) may help confirm the diagnosis of Sjögren's syndrome or systemic lupus erythematosus; these antibodies are associated with the extraglandular manifestations of Sjögren's syndrome. Cytoplasmic antineutrophil cytoplasmic antibody testing is highly sensitive and specific for Wegener's granulomatosis. Human leukocyte antigen-B27 is frequently present in ankylosing spondylitis and Reiter's syndrome, but the background presence of this antibody in white populations limits the value of testing. An elevated erythrocyte sedimentation rate (ESR) is a diagnostic criterion for polymyalgia rheumatica and temporal arteritis; however, specificity is quite low. ESR values tend to correlate with disease activity in rheumatoid arthritis and may be useful for monitoring therapeutic response.
Erythrocyte sedimentation rate
Polymyalgia rheumatica
Rheumatoid factor
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Seventy-two patients were studied whose sera contained antibodies to a cytoplasmic antigen, RO. The majority had clinical features typical of systemic lupus erythematosus and there appeared to be an overlap with Sjögren's syndrome. Of interest is that certain patients with lupus respond predominantly to cytoplasmic antigens and in some cases were ANA negative. Demonstration of this system is important in enlarging the serologic spectrum characteristic of SLE patients, and also may be an important marker for systemic disease in patients with predominantly cutaneous involvement.
Extractable nuclear antigens
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A patient with systemic lupus erythematosus with predominant skin involvement is described. A good correlation between disease activity manifested by skin lesions and between the titer of anti-DNA antibodies and C 3 component of complement was observed. Disease activity was accompanied by a high titer of anti-DNA antibodies and by a low level of the C3 component of complement; these parameters were reversed during remission. We conclude that these parameters are useful in the diagnosis and management of patients with systemic lupus erythematosus limited to the skin.
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