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      Systemic Lupus Erythematosus with Multiple Autoimmune Disease Presented with Extensive Peripheral Gangrene

      case-report

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          Abstract

          Systemic lupus erythematosus (SLE) is an autoimmune disease and can be associated with other autoimmune diseases. SLE usually presents with skin change and rarely presents with gangrene. SLE gangrene usually involves the digits of upper extremities. We report the first case of SLE associated with an extremely rare constellation of neuromyelitis Optica (NMO) and diabetes mellitus type 1, presented with a rare form of the SLE gangrene which involves bilateral lower extremities up to midlegs, a case that has not yet been reported in the literature. Although SLE gangrene may respond to immunosuppressants, it has a high risk of complications that can end up with amputations.

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          The role of sex hormones in systemic lupus erythematosus.

          R Lahita (1999)
          The disease systemic lupus erythematosus (SLE) is a complex one. One major clinical manifestation that relates to both cause and pathogenesis of all autoimmune diseases but specifically SLE is the sexual predilection for females. A consideration of mechanisms for this manifestation is the subject of this paper. The cytokine network is a major aspect of immune regulation and directly affected by sex steroids. The sexual dimorphism of the immune system relates to both organ-specific and general synthesis of sex steroids that are affected by and in turn affect cytokine profiles of T helper cells. There are also specific responses in cells from diseased patients that support the molecular activities of sex hormones on cells. Among these is the rise of calcineurin mRNA in SLE T cells in response to estrogen. Clinical research provides support for a more estrogenic environment in patients with SLE of both sexes. A preferential hydroxylation of estrone and increased oxidation of testosterone in patients with SLE maximizes the effects of estrogens on T cell functions. The effects of gonadotrophins like prolactin are discussed as stimulants of immune functions when elevated in SLE. Last, while the roles of exogenous estrogens on immune functions are known, the effects of such steroids alone or in combination with progestogens on SLE are not known. Investigation of both hormone replacement therapy and premenopausal hormone use are in progress.
          Article 0 comments Cited 46 times – based on 0 reviews     Review now
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            Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients.

            Norma Nardi, Albert Bové, Miguel Ingelmo … (2006)
            We conducted the current study to determine the prevalence and clinical characteristics of vasculitis in a large series of patients with systemic lupus erythematosus (SLE), focusing on the classification and clinical significance of the different types of vasculitis. We studied 670 consecutive patients who fulfilled 4 or more of the 1997 revised criteria for SLE. Definite vasculitis was diagnosed histologically and/or by arteriography, and probable vasculitis was diagnosed clinically when there were characteristic cutaneous lesions. Vasculitides were categorized according to the definitions adopted by the Chapel Hill Consensus Conference. Seventy-six (11%) patients with SLE had vasculitis (68 female patients and 8 male; mean age, 37.8 yr); only 32 (42%) fulfilled the Chapel Hill definitions. Cutaneous lesions were the main clinical presentation of vasculitis, present in 68 (89%) patients, while the remaining 8 (11%) had isolated visceral vasculitis. Compared with SLE patients without vasculitis, patients with vasculitis had a higher prevalence of livedo reticularis (22% vs. 3%; p = 0.028); a higher mean European Consensus Lupus Activity Measurement (ECLAM) score (5.86 vs. 3.87; p 50 mm/h (60% vs. 15%; p < 0.001), and anti-La/SS-B antibodies (19% vs. 5%; p = 0.014) in the multivariate analysis. With respect to the size of the vessels involved, 65 (86%) patients had small vessel vasculitis (SVV) and 11 (14%) had medium-sized vessel vasculitis (MVV). SLE patients with MVV had a higher prevalence of mononeuritis multiplex (54% vs. 2%; p < 0.001), visceral vasculitis (100% vs. 5%; p < 0.001), and ulcerated/ischemic cutaneous lesions (36% vs. 11%; p = 0.047) and a higher percentage of surgical interventions (45% vs. 0%; p < 0.001) compared with patients with SVV. In conclusion, we observed a heterogeneous presentation of vasculitides arising in the setting of SLE, with nearly 60% of cases not fulfilling the names and definitions adopted by the Chapel Hill Consensus Conference. SVV was the most frequent vasculitis, overwhelmingly cutaneous and clearly differentiated from MVV, which was less frequent but had predominantly visceral involvement (especially of the peripheral nerves). The presence of vasculitis in our patients with SLE was associated with a higher ECLAM score, livedo reticularis, hematologic parameters (anemia, high ESR), and anti-La/SS-B antibodies.
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              Peripheral gangrene in children with atypical hemolytic uremic syndrome.

              A. M. S. Abdul Majid, Arvind Bagga, Eva Simkova … (2012)
              Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy with severe clinical manifestation, frequent recurrence, and poor long-term prognosis. It is usually caused by abnormalities in complement regulation. We report 2 cases of children affected by a catastrophic extrarenal complication. A 4-year-old Indian girl developed gangrene of the finger tips 2 days after initial presentation of aHUS. Factor H autoantibodies were identified. Renal function continued to decline despite daily plasma exchanges, and she was started on peritoneal dialysis 5 days after admission. The distal tips of the left hand remained gangrenous with a line of demarcation. Three weeks later, she did not return for follow-up and died at home because of dialysis-related complications. An Arabic girl developed end-stage renal disease due to aHUS in the fourth month after birth. A de novo activating C3 mutation was found. At age 9 months, she suddenly developed ischemic changes in fingers of both hands and several toes. The lesions progressed, and several finger tips became gangrenous despite intense plasma exchange therapy. The decision was made to administer complement blocking therapy with the C5 antibody eculizumab. All nonnecrotic digits rapidly regained perfusion. The 3 already gangrenous fingers healed with loss of the end phalanges. During maintenance, eculizumab aHUS activity subsided completely and some late recovery of renal function was observed. aHUS may present by thrombotic macroangiopathy of small peripheral arteries. Eculizumab appears effective in preserving tissue viability if administered before gangrene occurs and should be considered as first-line rescue therapy in such cases.
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                Author and article information

                Contributors
                Tareq Z. Alzughayyar:
                ORCID: https://orcid.org/0000-0002-7419-0228
                Journal
                Journal ID (nlm-ta): Case Rep Rheumatol
                Journal ID (iso-abbrev): Case Rep Rheumatol
                Journal ID (publisher-id): CRIRH
                Title: Case Reports in Rheumatology
                Publisher: Hindawi
                ISSN (Print): 2090-6889
                ISSN (Electronic): 2090-6897
                Publication date Collection: 2020
                Publication date (Electronic): 10 March 2020
                Volume: 2020
                Electronic Location Identifier: 8278275
                Affiliations
                1Al-Quds University, Faculty of Medicine, Jerusalem, State of Palestine
                2Pediatric Rheumatologist, Al-Ahli Hospital, Hebron, State of Palestine
                3An-Najah National University, Faculty of Medicine & Health Sciences, Nablus, State of Palestine
                Author notes

                Academic Editor: George S. Habib

                Author information
                https://orcid.org/0000-0002-7419-0228
                https://orcid.org/0000-0002-1309-0880
                https://orcid.org/0000-0003-2055-2681
                https://orcid.org/0000-0002-7433-3698
                https://orcid.org/0000-0002-4042-4743
                https://orcid.org/0000-0002-5461-8346
                https://orcid.org/0000-0002-2356-1375
                https://orcid.org/0000-0002-6376-8805
                Article
                DOI: 10.1155/2020/8278275
                PMC ID: 7085827
                SO-VID: 3487b2d2-8e74-48b3-9750-26cd9ce72ec0
                Copyright © Copyright © 2020 Tareq Z. Alzughayyar et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 8 November 2019
                Date revision received : 15 January 2020
                Date accepted : 17 February 2020
                Categories
                Subject: Case Report

                Data availability:

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