Anetoderma associated with antiphospholipid syndrome and systemic lupus erythematosus

Anetoderma is an uncommon disorder characterized by the loss of elastic fibres in the dermis histologically and herniation of subcutaneous tissue clinically. Recent studies indicate that immunologic mechanisms may play a role in this process. Here we report a 33-year-old woman with numerous well-circumscribed, asymptomatic skin lesions in whom clinical and histopathologic features were consistent with anetoderma. Additionally, history and investigations revealed antiphospholipid syndrome and systemic lupus erythematosus. It has been speculated that immune deposits in the dermis or within the capillary walls may lead to ischaemia and subsequent degeneration of the elastic fibres.     

Pregnancy in systemic lupus erythematosus and antiphospholipid syndrome

Systemic lupus erythematosus (SLE) is the autoimmune disease that most commonly compromises pregnancy. Moreover, the relationship between SLE and pregnancy is in both directions. However, the current experience indicates that pregnancy in patients with SLE should not be regarded as an unacceptable high risk condition for the mother or her baby provided that careful planning of conception and multidisciplinary monitoring and treatment are carried out.     

Devic's syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome

Neuropsychiatric lupus is common and results in significantmorbidity [1, 2]. Antiphospholipid antibodies (aPL) may playa major role and are associated with transverse myelitis [2–4],often with a significant response to anticoagulation [4, 5].Devic's syndrome is described in multiple sclerosis (MS) [6]and has rarely been associated with systemic lupus erythematosus(SLE) [5, 7–9]. We present a 44-yr-old woman with SLEwho developed neuromyelitis optica and probable antiphospholipidsyndrome (APS). She was healthy until 1990 when schizophrenia was     

Canada-Cronkhite syndrome associated with systemic lupus erythematosus

A 43-year-old woman with a history of systemic lupus erythematosus manifested severe diarrhea, generalized gastrointestinal polyposis, pigmentation, and onychodystrophy, which were typical of Canada-Cronkhite syndrome. The exacerbation of systemic lupus erythematosus, presenting with hemolytic anemia and subsequently with profound proteinuria, was seen during the course of this syndrome. The patient achieved complete symptomatic remissions of both entities for more than four years following prednisolone therapy; the resolution of the gastrointestinal polyps was confirmed histologically. This case showed that this syndrome could be completely reversible in its course and that it might have an exogenous cause, as previously suggested.     

Moyamoya syndrome associated with systemic lupus erythematosus

Moyamoya disease is a rare, progressive cerebrovascular disorder that is characterized by a stenosis or occlusion of the bilateral internal carotid arteries and the development of collateral vessels. Transient ischemic attacks or seizures are the usual presentation of moyamoya disease in children, whereas cerebral hemorrhage is the most common symptom in adults. We report an 18-year-old female patient with active lupus nephritis who presented with the sudden onset of left hemiparesis. Brain magnetic resonance imaging showed acute infarctions in the right basal ganglia and subcortical white matter of the right frontal lobe. Cerebral angiography showed the stenosis of the bilateral internal carotid arteries with rich basal collateral vessels (moyamoya vessels). There was no evidence of atherosclerosis or antiphospholipid syndrome. Glucocorticoid therapy was used to control the systemic lupus erythematosus. Prophylactic bypass surgery was performed to prevent recurrent ischemic attacks. This case report shows that an underlying cerebrovascular lesion of moyamoya vessels in a patient with systemic lupus erythematosus is susceptible to cerebrovascular accidents.     

Thymoma associated with systemic lupus erythematosus and immunologic abnormalities

The association between Systemic Lupus Erythematosus (SLE) and thymoma occurs with a greater frequency than dictated by coincidence alone. The immunologic effects of thymectomy on the appearance and/or the course of SLE are still to be elucidated. We report one case of SLE diagnosed at the same time as thymoma, and two cases of thymoma associated with immunologic disorders in the absence of clinical signs and symptoms diagnostic of SLE.     

Lupus mastitis associated with severe systemic erythematosus lupus

We report a 46-year-old woman with a systemic lupus erythematosus complicated with a lupus panniculitis. The patient developed initial deep breast nodules secondarily completed by superficial erythematosus and keratotic cutaneous lesions. Neoplasia was ruled out by X-ray and echographic investigations. Both subcutaneous and skin biopsies were consistent with the diagnosis of lupus mastitis. Lupus mastitis is a form of lupus panniculitis seldom associated with systemic lupus. A breast neoplasia should be ruled out by appropriate investigations and deep biopsies. The first-line treatment is based on antimalarial drugs.     

Stroke in patients with systemic lupus erythematosus and antiphospholipid antibody syndrome

Opinion statement Primary prevention of strokes in patients with antiphospholipid antibodies (APLs) with or without systemic lupus erythematosus (SLE) is not well known. The same applies to patients with SLE and valvular heart disease. The decision should be made on an individual basis until further studies become available. Special consideration for preventive antithrombotic treatment should be given to patients with persistent high titers of immunoglobulin G APLs, which require protein cofactor for detection, presence of lupus anticoagulant, or left-sided cardiac valve lesions. High-level oral anticoagulation with warfarin is still the preferred treatment for secondary prevention of strokes in patients with antiphospholipid antibody syndrome (APS) with or without SLE or with cardiac valvular lesions. Immunosuppression should only be used in patients with active SLE disease. There is no evidence so far to support its use in patients with primary APS. Advances in identifying unique APL features that are associated with increased risk for thrombosis will hopefully allow a more rational treatment for primary and secondary prevention of strokes in these patients in the near future.     

Stroke in patients with systemic lupus erythematosus and antiphospholipid antibody syndrome

Opinion statement  Primary prevention of strokes in patients with antiphospholipid antibodies (APLs) with or without systemic lupus erythematosus (SLE) is not well known. The same applies to patients with SLE and valvular heart disease. The decision should be made on an individual basis until further studies become available. Special consideration for preventive antithrombotic treatment should be given to patients with persistent high titers of immunoglobulin G APLs, which require protein cofactor for detection, presence of lupus anticoagulant, or left-sided cardiac valve lesions. High-level oral anticoagulation with warfarin is still the preferred treatment for secondary prevention of strokes in patients with antiphospholipid antibody syndrome (APS) with or without SLE or with cardiac valvular lesions. Immunosuppression should only be used in patients with active SLE disease. There is no evidence so far to support its use in patients with primary APS. Advances in identifying unique APL features that are associated with increased risk for thrombosis will hopefully allow a more rational treatment for primary and secondary prevention of strokes in these patients in the near future.     

Aortic occlusion in systemic lupus erythematosus associated with antiphospholipid antibodies.

The patient, a Caucasian woman of 22 years, developed malignant hypertension at the age of 16 years. An abdominal bruit was found on routine examination. She had two spontaneous abortions and systemic lupus erythematosus was diagnosed at age 21 years. She was found to have a persistently low positive VDRL and antibodies to mitochondria were also present on repeated examinations, to a titre of 1/160. Because of angiographic findings, demonstrating an occlusion of the aorta, an endarterectomy of the descending thoracic and upper abdominal aorta was performed. This showed mainly intimal thickening and the presence of thrombus. She then had four further spontaneous abortions (with good blood pressure control). The lupus anticoagulant and antibodies to cardiolipin were found to be positive at this time.     

Association between antiphospholipid antibodies and cardiac abnormalities in patients with systemic lupus erythematosus

PURPOSE: Although the antiphospholipid antibodies are well recognized to be associated with thrombosis, recurrent abortion, and thrombocytopenia in patients with systemic lupus erythematosus (SLE), their relationship with cardiac disease is less clear. The purpose of this study was to evaluate the association between anti-phospholipid antibodies and cardiac abnormalities in patients with SLE. PATIENTS AND METHODS: A total of 75 consecutive SLE patients and 60 healthy sex- and age-matched control subjects were evaluated in a case-control study. All participants underwent M-mode, two-dimensional, and Doppler echocardiography. Antiphospholipid antibodies levels were assayed in each patient. The prevalence of antiphospholipid antibodies in patients with and without echocardiographic abnormalities was compared. RESULTS: Compared with the control group, SLE patients had significantly more pericardial abnormalities, left ventricular hypertrophy, left atrial enlargement, left ventricular dysfunction and verrucous valvular thickening, global valvular thickening with dysfunction, and mitral and aortic regurgitation. Among these abnormalities, antiphospholipid antibodies were significantly associated with isolated left ventricular (global or segmental) dysfunction (four of five positive; p less than 0.05), verrucous valvular (mitral or aortic) thickening (seven of nine positive; p less than 0.005), global valvular (mitral or aortic) thickening and dysfunction (five of six positive; p less than 0.02), as well as mitral regurgitation (16 of 19 positive; p less than 0.001) and aortic regurgitation (five of six positive; p less than 0.02). CONCLUSION: Valvular lesions and myocardial dysfunction are associated with elevated antiphospholipid antibodies. This study has important implications for the pathogenic role of anti-phospholipid antibodies in relation to these cardiac abnormalities.     

Cardiac dysfunction in patients with systemic lupus erythematosus and antiphospholipid syndrome

OBJECTIVE: To comparatively assess the parameters of systolic and diastolic cardiac function in patients with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS). METHODS: Consecutive patients (n=74) who were free of cardiovascular symptoms were divided into four groups: (1) SLE (n=23); (2) SLE with antiphospholipid antibodies (aPL; n=18); (3) SLE with APS (n=20); and (4) primary antiphospholipid syndrome (PAPS; n=13). Pulsed, continuous, colour Doppler echocardiography, and M-mode and B-mode studies were performed. RESULTS: Left ventricular end diastolic and end systolic dimensions were higher in SLE as compared with patients with PAPS (p=0.022 and 0.022, respectively), with a trend towards a lower fractional shortening in SLE (p=0.07), suggesting systolic dysfunction. Parameters of diastolic function were more impaired in patients with APS, reflected by lower left ventricular and right ventricular E wave to A wave (E:A) ratios in patients with APS (groups 3, 4) compared with those without APS (groups 1, 2; 1.15 (0.40) v 1.49 (0.43), p=0.001 and 1.19 (0.31) v 1.49 (0.41), p=0.001, respectively) and a more prolonged left ventricular isovolumic relaxation time (IVRT; 94.2 (24.6) v 84.4 (17) ms, respectively, p=0.055). Patients with APS were older than those without APS (47.12 (14.86) v 34.29 (12.6), p=0.0001). Patients with SLE were younger than those with PAPS (38.19 (14.68) v 48.53 (13.97), p=0.023). CONCLUSION: Abnormal echocardiographic findings were detected frequently in asymptomatic patients with SLE or PAPS. Although patients with SLE were younger, left ventricular systolic function was more impaired in patients with SLE compared with those with PAPS, whereas left ventricular and right ventricular diastolic function, as reflected by IVRT and E:A ratios, were significantly more impaired in patients with APS.     

Communicating hydrocephalus in systemic lupus erythematosus with antiphospholipid antibody syndrome.

We report a case of communicating hydrocephalus in a 24-year-old woman with previously undiagnosed systemic lupus erythematosus (SLE) presenting with malignant hypertension, nephritis, serositis, and a seizure disorder of 16 months' duration. The patient demonstrated features of the antiphospholipid antibody syndrome (APS). In proposing cerebral venous thrombosis as a possible, yet unproven, pathophysiologic mechanism for the hydrocephalus in this case we have reviewed and summarized literature relating to SLE, APS, hydrocephalus and pseudotumor cerebri. In cases of unexplained pseudotumor cerebri or hydrocephalus, a search for SLE and APS should be considered.