Systemic lupus erythematosus with simultaneous onset of Kikuchi-Fujimoto’s disease complicated with antiphospholipid antibody syndrome: a case report and review of the literature

2005 ◽  
Vol 25 (4) ◽  
pp. 303-306 ◽  
Author(s):  
Hsiang-Cheng Chen ◽  
Jenn-Haung Lai ◽  
Guo-Shu Huang ◽  
Hong-Wei Gao ◽  
Chen-Hung Chen ◽  
...  
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 ◽  
...  

2014 ◽  
Vol 2014 ◽  
pp. 1-9 ◽  
Author(s):  
Mahnoosh Foroughi ◽  
Manouchehr Hekmat ◽  
Mohsen Ghorbani ◽  
Hamid Ghaderi ◽  
Masoud Majidi ◽  
...  

Valvular heart disease is the common cardiac manifestation of systemic lupus erythematosus (SLE) with a tendency for mitral valve regurgitation. In this study we report a case of mitral valve replacement for mitral stenosis caused by Libman-Sacks endocarditis in the setting of SLE. In addition, we provide a systematic review of the literature on mitral valve surgery in the presence of Libman-Sacks endocarditis because its challenge on surgical options continues. Surgical decision depends on structural involvement of mitral valve and presence of active lupus nephritis and antiphospholipid antibody syndrome. Review of the literature has also shown that outcome is good in most SLE patients who have undergone valvular surgery, but association of antiphospholipid antibody syndrome with SLE has negative impact on the outcome.


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.


2016 ◽  
Vol 10 (1) ◽  
pp. 122-128 ◽  
Author(s):  
Emily C. Milam ◽  
Jacobo Futran ◽  
Andrew G. Franks Jr.

Background: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily targets the muscle, skin, and lungs. Many patients have autoantibodies that correspond to distinct clinical phenotypes. Melanoma differentiation-associated gene 5 (anti-MDA5) antibody, a specific antibody that targets the melanoma differentiation-associated gene 5 (MDA5), has been reported in DM cases and is significant for a distinct cutaneous presentation and rapidly progressive interstitial lung disease. Objective: Herein, we describe a patient with DM with a positive anti-MDA5 antibody and characteristic clinical phenotype, who subsequently developed coexisting systemic lupus erythematosus (SLE). A diagnosis of SLE was supported by his clinical phenotype, positive serologies, hypocomplementemia, and progression to glomerulonephritis and lupus cerebritis, features of which fulfilled the American College of Rheumatology criteria for SLE. Conclusion: DM is known to overlap with other autoimmune diseases, including SLE, and coexistence can lead to a wide variety of clinical presentations. SLE overlapping with anti-MDA5 positive DM may present with distinct clinical features.


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