Cerebral large vessel vasculitis in systemic lupus erythematosus

Lupus ◽  
2014 ◽  
Vol 23 (13) ◽  
pp. 1417-1421 ◽  
Author(s):  
B C Böckle ◽  
D Jara ◽  
K Aichhorn ◽  
D Junker ◽  
T Berger ◽  
...  

Neuropsychiatric systemic lupus erythematosus (NPSLE) is defined by involvement of the central nervous system in systemic lupus erythematosus (SLE), with a wide range of both neurological and psychiatric manifestations. Although its aetiopathogenesis is not fully elucidated, NPSLE seems to be a consequence of cerebral vascular pathology including thromboembolism, small-vessel vasculopathy and, in rare cases, true vasculitis. Cerebral vasculitis is rare, and cerebral large-vessel vasculitis in SLE is even more unusual. We report the case of a female patient with the diagnosis of SLE. She presented with stroke-like symptoms, headache and vertigo, and palpable purpura on her legs. Further investigations revealed that she suffered from both vasculitis of the cerebral large vessels and coexisting cutaneous small-vessel vasculitis.

Lupus ◽  
2019 ◽  
Vol 28 (5) ◽  
pp. 685-694
Author(s):  
N Mvambo ◽  
A I Bhigjee ◽  
G M Mody

Neuropsychiatric systemic lupus erythematosus (NPSLE) is an important cause of morbidity and mortality. We undertook this observational retrospective study of patients with NPSLE who had brain magnetic resonance imaging (MRI) to determine the indications for MRI and the correlation of clinical and laboratory findings with MRI. We identified 83 NPSLE patients (84.3% women) seen at Inkosi Albert Luthuli Central Hospital in Durban, South Africa, between June 2003 and May 2017. The mean age at SLE diagnosis was 26.24 ± 12.81 years and the median interval to NPSLE was 11.0 (interquartile range, 4.0–39.0) months. The most common indications for MRI were seizures (45.8%), psychosis (18.1%) and cerebrovascular disease (18.1%). The MRI was abnormal in 68 (81.9%) with small-vessel disease in 65 (78.3%) and large-vessel disease in eight (9.6%). The small-vessel abnormalities were white-matter hyperintensities (WMH) (59.0%), atrophy (55.4%) and lacunae (4.6%). Our patients had high disease activity at NPSLE. Cerebrovascular disease was associated with an abnormal MRI ( p = 0.018) and large-vessel disease ( p = 0.014) on MRI. Our NPSLE patients were younger and had high disease activity, and seizures were more common compared with other studies. The most common MRI abnormalities were WMH and cortical atrophy, in agreement with other studies.


2019 ◽  
Vol 8 (2) ◽  
pp. 101
Author(s):  
SubbalaxmiV S. Malladi ◽  
Sumanth Vittamraj ◽  
Anu Kapoor ◽  
Naval Chandra ◽  
Liza Rajasekhar

Author(s):  
Elias Manca

AbstractSystemic lupus erythematosus is a complex immunological disease where both environmental factors and genetic predisposition lead to the dysregulation of important immune mechanisms. Eventually, the combination of these factors leads to the production of self-reactive antibodies that can target any organ or tissue of the human body. Autoantibodies can form immune complexes responsible for both the organ damage and the most severe complications. Involvement of the central nervous system defines a subcategory of the disease, generally known with the denomination of neuropsychiatric systemic lupus erythematosus. Neuropsychiatric symptoms can range from relatively mild manifestations, such as headache, to more severe complications, such as psychosis. The evaluation of the presence of the autoantibodies in the serum of these patients is the most helpful diagnostic tool for the assessment of the disease. The scientific progresses achieved in the last decades helped researchers and physicians to discover some of autoepitopes targeted by the autoantibodies, although the majority of them have not been identified yet. Additionally, the central nervous system is full of epitopes that cannot be found elsewhere in the human body, for this reason, autoantibodies that selectively target these epitopes might be used for the differential diagnosis between patients with and without the neuropsychiatric symptoms. In this review, the most relevant data is reported with regard to mechanisms implicated in the production of autoantibodies and the most important autoantibodies found among patients with systemic lupus erythematosus with and without the neuropsychiatric manifestations.


2019 ◽  
Vol 72 (7) ◽  
pp. 1359-1363
Author(s):  
Marcin Zarzycki ◽  
Magdalena Flaga-Łuczkiewicz ◽  
Joanna Czuwara ◽  
Lidia Rudnicka

Systemic lupus erythematosus (SLE) is a chronic multiorgan autoimmune disease belonging to spectrum of interest of many medical specialties. Wide range of patients 14−75% with SLE suffers from neuropsychiatric disorders. The problematic diagnosis of neuropsychiatric SLE has generated many studies focusing on etiology of the disease with the presence of specific autoantibodies, abnormalities which can be detected by imaging examinations or correlation with catecholamine levels. The aim of this review paper is to discuss the frequency of neuropsychiatric disturbances in patients with SLE and their potential association with immunological abnormalities and specific disease markers. So far published literature regarding this topic indicates the usefulness of autoantibodies specificity. The use of the specific antibodies may be helpful in targeting diagnostics towards psychiatric disorders, especially depressive ones. Imaging scanning techniques such as computed tomography (CT) have limited value in psychiatric disorders diagnosis but can be useful in neurological symptoms and complains. Therapeutic use of systemic glucocorticosteroids due to anti-inflammatory properties with multidirectional action, may also significantly influence the course of neuropsychiatric diseases, especially in patients with SLE. Awareness of the morbidity of neuropsychiatric disorders and the possibilities of their diagnosis are important in the management of patients with systemic lupus erythematosus, which significantly affects the quality of life of patients, treatment efficacy and psyche.


Lupus ◽  
2018 ◽  
Vol 27 (7) ◽  
pp. 1198-1201
Author(s):  
H Elsayed Mansour ◽  
S Gamal Arafa ◽  
W Abdelfatah Shehata

A 30-year-old female presented to the rheumatology outpatient clinic of the Internal Medicine Department, Ain Shams University Hospital, Cairo, Egypt, complaining of a large right leg ulcer consistent with pyoderma gangrenosum. There was history of recurrent attacks of bleeding per rectum of one-year duration. During hospitalization she noticed blurring of vision in the left eye with diffuse blackish discoloration of the feet and toes, consistent with small-vessel vasculitis. Colonoscopy with biopsy and histopathology confirmed the diagnosis of inflammatory bowel disease-ulcerative colitis (IBD-UC). Meanwhile, the patient fulfilled the SLICC classification criteria for systemic lupus erythematosus (SLE): recurrent oral ulcers, positive antinuclear antibody testing, proteinuria >0.5 gm/24-hour urine, positive test for lupus anticoagulant and consumed C3 complement component. Herein we report a rare case of coexistence of SLE and IBD-UC.


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