Gastrointestinal vasculitis due to systemic lupus erythematosus treated with rituximab: a case report

Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 640-643
Author(s):  
Gökçe Kenar ◽  
Kadri Atay ◽  
Gül Emek Yüksek ◽  
Burak Öz ◽  
Süleyman Serdar Koca

Background Patients with systemic lupus erythematosus may present with variable gastrointestinal manifestations including peritonitis, pancreatitis, enteritis, and vasculitis. Gastrointestinal vasculitis is one of the most devastating complications of systemic lupus erythematosus, with a mortality rate of 50% when it progresses to bowel ischemia and is complicated by hemorrhage or perforation. Case report A young female patient known to have systemic lupus erythematosus, lupus nephritis, and antiphospholipid antibody syndrome, on immunosuppressive treatment presented to the emergency department with acute abdominal pain and nausea. The clinical findings were first associated with an acute flare of lupus nephritis according to the assessments with active laboratory parameters. However, over a short time the abdominal pain worsened and was accompanied by peritonitis and gastrointestinal hemorrhage. The computed tomography scans demonstrated a dilated bowel and thickening of the bowel compatible with gastrointestinal vasculitis. The upper and lower gastrointestinal endoscopy supported the diagnosis of vasculitis by showing multiple ulcerative lesions along the gastrointestinal tract. The patient was successfully treated with pulse corticosteroids urgently, with a fast response to subsequent rituximab therapy without any relapses. Treatment with cyclophosphamide was not preferred because the patient had a high cumulative dose. Conclusion Gastrointestinal vasculitis should be a primary differential diagnosis in patients with systemic lupus erythematosus presenting with abdominal pain because of its rapid progression and high mortality. The treatment choice has been suggested as cyclophosphamide for severe cases in the literature. In this case report, a patient successfully treated with rituximab without any relapses was documented.

Lupus panniculitis occurs in 1–3% of the patients diagnosed with systemic lupus erythematosus (SLE) and 10% of the patients diagnosed with discoid lupus erythematosus (DLE). It is a disorder of autoimmune origin, manifesting as deep erythematous plaques and nodules involving the trunk, breasts, buttocks, face, and proximal extremities. It does not commonly ulcerate. This report highlights the case of a 22-year-old Asian female with a history of coeliac disease and significant family history of antiphospholipid antibody syndrome (APS) who presented with fever, malaise, weight loss, and subcutaneous non-tender nodules over the forearm, back, bilateral thighs, and feet. Laboratory investigations revealed positive antinuclear antibodies, anti-Ro/SSA antibody, and lupus anticoagulant, resulting in a diagnosis of APS. Biopsies of lesions were consistent with findings of lupus panniculitis. Every case of SLE and DLE with discrete skin lesions should be reviewed for any distinct entity such as lupus panniculitis, as it may be associated with greater risk of flares and systemic involvement. The purpose of this case report is to emphasise that early diagnosis and prompt treatment is crucial to improving the prognosis of such patients.


2000 ◽  
Vol 39 (10) ◽  
pp. 856-859 ◽  
Author(s):  
Reiko MIZUNO ◽  
Shinichi FUJIMOTO ◽  
Takashi FUJIMOTO ◽  
Toshihiko NISHINO ◽  
Hideo SHIIKI ◽  
...  

2021 ◽  
Vol 14 (1) ◽  
pp. e236592
Author(s):  
Ying Ling ◽  
Mary Jane Bell ◽  
Lisa Chodirker ◽  
Shirley Lake

A high functioning 74-year-old man with systemic lupus erythematosus presented to the emergency department with acute anxiety. He was found to have elevated cardiac enzymes and admitted to the cardiology service for investigation. In hospital, he developed an erythematous papular rash, and deteriorated to being somnolent and bedridden. He was found to have new multiterritory ischaemic strokes. It was eventually noted that he had persistent eosinophilia, present even on admission, which had been overlooked as the total leucocyte count was normal. Serology for antiphospholipid antibody syndrome (APS) was positive. He was diagnosed with hypereosinophilic syndrome (HES) secondary to new APS, and responded to high-dose steroids. This case highlights the importance of fully evaluating a leucocyte differential to make a diagnosis of HES. We discuss the definition, clinical manifestations, diagnostic approach and management of this important condition.


2020 ◽  
Author(s):  
metin kaya gürgöze ◽  
Aslıhan Kara ◽  
Mehmet yusuf sarı ◽  
İlknur Çalık ◽  
Saadet Akarsu

Abstract Background: Although pyoderma gangrenosum (PG) -like lesions have been rarely described in adults with the antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE), the occurrence of PG as a preceding manifestation of APS in children with SLE has not been reported until. We present a young girl with SLE and APS who developed progressive extstensive ulcerations that were consistent with PG.Case presentation: A 14-year-old girl with a 2-year history of SLE was admitted to our department, complaining painful crusted ulcerations on her legs. Skin biopsy was reported as PG. However, she did not respond to immunosuppressive therapy administered. When her skin biopsy findings is reassessed in keeping with the positive anticardiolipin antibody results, superficial small vessel microthrombosis was observed. Diagnosis of APS and PG developing secondary to SLE were made. It was resulted in marked clinical improvement with anticoagulation therapy in addition to immunosuppressives as is recommended in APS. Conclusions: Based in clinical, pathological and response to proposed treatment, we can state that PG -like lesions in children with SLE could be considered as a secondary form of APS.


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