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Systemic lupus erythematosus with multiple organ complications

Systemic lupus erythematosus with multiple organ complications

Systemic lupus erythematosus (SLE) is an autoimmune disease, mostly with a chronic course, that can affect almost all important organs, most commonly skin, joints, heart and vessels, kidneys, central nervous system and lungs. The disease is characterized by B-lymphocyte hyperactivity, which leads to the formation of autoantibodies predominantly directed against non-specific antigens. This case study describes a case of SLE with multiple organ complications in a 29-year-old female patient.

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Anamnesis:

29-year-old patient with SLE in treatment since 2009, with polyarthritis, polyserositis, acral limb circulatory disorder, photosensitivity, limb exanthema, generalized lymphadenopathy, vasculitis, history of glomerulonephritis, secondary arterial hypertension, history of thrombosis of left internal jugulary vein, subclavian vein, axillar vein and brachial vein in 2012 and 2014.

Therapy:

09.01.2017 - hospitalized at department of surgery in Prešov for abdominal pain, performed apendectomy.
15.01.2017 - performed a revision of the abdominal cavity for the development of the paralytic ileus.
22.01.2017- for exacerbation of SLE was subsequently transferred to the department of internal medicine in Prešov, where intensive immunosuppressive treatment was initiated.
07.02.2017 – because the need of plasmapheresis, the patient was transferred to IVth internal clinic. Here, in cooperation with the Logman hemodialysis center, repeated plasma plasmaphereses (10x) were conducted, we continued with immunosuppressive therapy - methylprednisolone (Solumedrol), cyclophosphamide (Endoxan), intravenous immunoglobulin. Despite complex therapy, high levels of autoantibodies (anti-dsDNA, anti-nucleosome antibodies, ANA, anti-Sm, antiU1RNP) persisted. We reported the development of lupus enteritis with numerous diarrhea (on the abdominal ultrasound and CT we detected diffuse evenly groomed gastric wall from the stomach to the rectum based on submucosal oedema - water target sign, small ascites). Severe nephrotic proteinuria (up to 27.96 g / 24 h) on the basis of lupus nephritis continued. Serious hypoproteinemia (total protein 33.0 g / l, albumin 19.0 g / l) was corrected by parenteral infusions of albumin, minerals were supplemented (sodium 130.2 mmol / l, potassium 2.2 mmol / l). In spite of complex intensive treatment, anasarka and hypertension resistant against treatment developed.
02.03.2017 – due to acute respiratory insufficiency we carried out the evacuation puncture of the large pleural effusion, but after the puncture, serious reperfusion pulmonary oedema developed with the necessity of non-invasive ventilation. From the vital indication, we started the extracorporal elimination treatment with SCUF (slow continuous ultrafiltration).
03.03.2017 - after a relative stabilization of the patient's condition, the patient was transferred to I.KAIM, where doctors continued with the extracorporal elimination treatment with CVVHD (continuous venous-venous hemodialysis). Ultrafiltration-induced anuria, weight loss of 15 kg, decline in oedemas, decrease of blood pressure and decline in respiratory insufficiency were recorded.
06.03.2017 – patient transferred back to IVth internal clinic, where due to anuria and progressive increase of renal parameters (urea 25.4 mmol / l, creatinine 387.6 μmol / L) we continued with CVVHD, titrated the dose of oral antihypertensive therapy, continued with immunosuppressive therapy, including intravenous immunoglobulins. We noted a gradual increase in diuresis with a marked reduction of proteinuria (0.63 g / 24 h), but high levels of autoantibodies persisted.
19.03.2017 - development of paralytic ileus, with jejunal paralysis, therefore the patient was transferred to 2nd surgical clinic where the laparotomy was performed with the finding of peritonitis and adhesiolysis and bowel deliberation were conducted.
04.04.2017 – after recovery of the peristalsis patient transferred back to IVth internal clinic intensive care unit, to continue with immunosuppressive therapy. In the lab tests, large proteinuria (13.43 g / 24 h), severe hypoproteinemia (42.1 g / l) and moderate-grade normocytic normochromic anaemia (8.87 g / dl) were present. For the development of anasarka, re-initiated extracorporal elimination treatment.
05.04.2017 – after extraordinary approval, a biological treatment with rituximab (Mabthera) was initiated in the patient, in combination with intravenous immunoglobulins.
09.04.2017 – development of clostridial enterocolitis and sepsis (blood culture positivity - Candida albicans, CRP 236.10 mg / l, PCT 33.87 μg / l), treated with antibiotics, antifungals and intestinal antiinfectives. In spite of the complex therapy, the condition of the patient worsened.
12.04.2017 - sudden loss of consciousness with asystole, apnea, initiated KPCR, which, despite the prolonged course, was unsuccessful.

Authors declare the case report will not be published in any national or international publications.

Classification ICD-10: M32 Systemic lupus erythematosus


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author: Katarína Demková | organization: 4th Department of Internal Medicine | published on: 2.1.2018 | last modified on: 16.4.2018
citation: Demková Katarína: Systemic lupus erythematosus with multiple organ complications. Multimedia support in the education of clinical and health care disciplines :: Portal of Faculty of Medicine [online] 2018-01-02, last modif. 2018-04-16 [cit. 2018-08-17] Available from WWW: <http://portal.lf.upjs.sk/articles.php?aid=352>. ISSN 1337-7000.
 

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