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Vasculitides
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Ivy M. Obonyo, Virginia A. Jones, Kayla A. Clark, Maria M. Tsoukas
Course: Primarily idiopathic in nature, urticarial vasculitis may also be due to drug reactions, infections, autoimmune disorders, and paraneoplastic syndromes. Patients with hypocomplementemia may have systemic findings and worse prognosis.
Coccidioidomycosis
Published in Rebecca A. Cox, Immunology of the Fungal Diseases, 2020
A decreased serum level of CH50, if not caused by a defect in complement production or catabolism, is indicative of complement consumption.163 Consumption hypocomplementemia can result from the direct activation of the complement pathway by the fungus, as suggested by the studies of McNall et al.136 and Galgiani et al.,137 or by the formation of complementbinding immune complexes. The presence of complement-binding immune complexes in sera of coccidioidomycosis patients was examined by Yoshinoya et al.138 and Cox et al.164 The results established that 42% of patients with active coccidioidomycosis have significant levels of circulating immune complexes. Although the report by Yoshinoya et al.138 did not reveal a correlation between circulating immune complexes and disease severity, analyses of a larger population established that those patients with disseminated disease involving two or more organ systems had the highest frequency of elevated immune complexes (Figure 6).164 Analyses of the composition of immune complexes established the presence of Clq, coccidioidal antigen(s), and anti-Coccidioides IgG.138
The Etiology of the Antiphospholipid Syndrome
Published in Howard J.A. Carp, Recurrent Pregnancy Loss, 2020
Sara De Carolis, Giuseppina Monteleone, Cristina Garufi, Rotem Inbar, Miri Blank, Yehuda Shoenfeld
Hypocomplementemia may be a prognostic factor for poor pregnancy outcome in APS patients, which could be used to identify APS pregnancies at higher risk of obstetric complications [6]. The protective role of heparin in APS in a mouse model has even been related to the anticomplement effect rather than anticoagulant activity [50].
Systemic lupus erythematosus and risk of infection
Published in Expert Review of Clinical Immunology, 2020
Megan R.W. Barber, Ann E. Clarke
Susceptibility to infection is also driven by acquired immune dysregulation, with disease activity identified as an independent predictor of infection in multiple SLE cohorts [20,22,25,42]. Anti-double-stranded DNA antibody positivity, hypocomplementemia, and frequent flares are independent predictors of infection [43]. The pathophysiology is complex, and a wide array of mechanisms are involved. Disease activity can breakdown cutaneous and mucosal barriers allowing microbial entry. Immune complex formation leads to hypocomplementemia which may inhibit the complement cascade. T helper cells have a diminished response to viral antigens, which is worsened by SLE disease activity [44]. Neutropenia and lymphopenia are also associated with disease activity and may increase infection rate [45].
A simultaneous presentation of drug-induced lupus with drug-induced ANCA vasculitis secondary to hydralazine use in a patient with sarcoidosis
Published in Baylor University Medical Center Proceedings, 2019
Maria Catalina Espinosa, Belicia Ding, Kati Choi, Daniel N. Cohen, Marco Marcelli, Onome Whiteru Ifoeze
Given similar features, it has been reported that DIL and ANCA DIV may represent one disease spectrum.2 Our patient demonstrated overlapping features of both drug-induced rheumatologic disorders. ANCA DIV was evidenced by extensive cutaneous leukocytoclastic vasculitis, positive ANCA, and anti-proteinase-3 antibody. DIL was manifested by oral ulcers, positive ANA and anti-histone antibody, thrombocytopenia, and hypocomplementemia. It has been reported that overlapping presentations of DIL with ANCA DIV have been characterized by the presence of high ANCA and ANA titers, positive anti-histone antibody, and positive anti-phospholipid antibodies, as seen in our patient. It has also been reported that such development of multiple antibodies may indicate a drug-induced autoimmune process. Hypocomplementemia has also been described, which was present in our case. Clinically these patients may present with small vessel vasculitis of the skin, as in our patient. Weight loss, cough, lung disease, and kidney disease have also been described.2
Immunophenotype involved in IgG4-related disease
Published in Modern Rheumatology, 2019
Satoshi Kubo, Shingo Nakayamada, Yoshiya Tanaka
In addition to basic research for IgG4-RD, there are hypothesis that cannot be explained theoretically in the clinical setting. In laboratory examinations, elevated serum IgG4, elevated IgE, hypocomplementemia, and low inflammatory response are generally seen in patients with IgG4-RD. However, the pathological significance of IgG4 in patients with IgG4-RD is not known, as mentioned earlier. IgG1 and IgG3 cause inflammation through complement activation and opsonization, while IgG4 lacks these functions. Furthermore, high level of IgG4 is sometimes seen even in patients who are in clinical remission. It is unknown whether IgG4 in IgG4-RD acts as an exacerbating factor. Meanwhile, since hypocomplementemia is seen as a result of the formation of strong immune complex, serious clinical conditions manifests in patients with hypocomplementemia. For example, activity of lupus nephritis is known to be strongly associated with hypocomplementemia. In contrast, there are no evidences of autoantibody production and involvement of immune complex in patients with IgG4-RD, although hypocomplementemia is seen. In order to resolve these clinical questions, there is need for the elucidation of the pathological processes that can offer a theoretical explanation of these phenomenon.