Abstract:To elucidate the clinical characteristics and pathogenesis of scleroderma-rheumatoid arthritis (SSc-RA) overlap syndrome, we analyzed the clinical features of 5 patients with SSc-RA overlap. Their HLA phenotypes and genotypes were also determined. Generalized skin sclerosis, severe seropositive polyarthritis, pulmonary fibrosis, anti-topoisomerase I antibodies, and HLA-DR4,53;DQA1*0301;DQBl*O4 haplotype were observed in all of the patients. Similar clinical features were recognized in most of the 10 cases repo… Show more
“…The simultaneous presence of two systemic autoimmune diseases in the same patient has been reported in several studies [80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, 100, 101]. …”
Background: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. Objective: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. Methods: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. Results: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. Conclusion: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.
“…The simultaneous presence of two systemic autoimmune diseases in the same patient has been reported in several studies [80, 81, 82, 83, 84, 85, 86, 87, 88, 89, 90, 91, 92, 93, 94, 95, 96, 97, 98, 99, 100, 101]. …”
Background: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. Objective: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. Methods: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. Results: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. Conclusion: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.
“…Работы по изучению распределения HLa антиге-нов при перекрестных формах немногочисленны. Иммуногенетические исследования, проведенные японскими учеными, выявили у 4 из 5 обследо-ванных больных ССД-РА наличие гаплотипа HLa DR4, 53; DQ a1*0301; DQ B1*04 [6].…”
Section: резюмеunclassified
“…), когда имеются критерии обоих заболеваний, речь идет о перекрестной форме ССД-РА [5]. Клинико-рен-тгенологическое сходство дополняется нередким обнаружением ревматоидного фактора (РФ), одна-ко чаще выявляются антинуклеарные антитела (АНФ) и антитела к топоизомеразе I (а-Scl-70) [6,7]. Антитела к циклическому цитруллинированному пептиду (АЦЦП) являются высокоспецифичным диагностическим маркером РА, так как более чем в 80% случаев обнаруживаются в сыворотке больных РА.…”
“…This does not preclude occasional diagnostic crrors, nor does it exclude the possibility that any of the CTDs can be a partner in a true overlap disorder. The other partner disease is, most often, RA (28)(29)(30). Another, much rarer, disorder is, for example, an overlap diseasc of SLE with primary Sjogren's syndrome (31,32).…”
Section: Ctds: Entities Syndromes or Spectrums?mentioning
confidence: 99%
“…Another, much rarer, disorder is, for example, an overlap diseasc of SLE with primary Sjogren's syndrome (31,32). Importantly, however, all of these overlap disorders are usually characteriz-ed not only by the concomitant occurrence of the respective clinical features, but also by concurrence of the typical serologic abnormalities of the component diseases (28)(29)(30)(31)(32). (The characteristics of other welldefined primary overlap disorders, such as sclerodermatomyositis with anti-PMiScl autoantibodies, are not discussed in this review.)…”
Section: Ctds: Entities Syndromes or Spectrums?mentioning
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