Recurrent acute proptosis in atypical systemic lupus erythematosus

Summary A patient is described who developed acute proptosis, a rare complication of systemic lupus erythematosus, 16 years after the onset of the disease. Treatment with intravenous methylprednisolone produced rapid improvement, but swelling recurred a few days later. Intravenous corticosteroid was again effective and was continued orally without further episodes of proptosis. This patient is unusual in the long period of mild disease before the development of systemic complications, and in having antibodies to nuclear ribonucleoprotein but persistently normal titres of DNA-binding antibodies in her serum.     

Cerebral infarction in systemic lupus: association with anticardiolipin antibodies

We report fifteen patients, thirteen with systemic lupus and two patients with a "lupus-like" illness who developed cerebral infarction. All fifteen patients were shown to have elevated anticardiolipin antibody levels using a newly devised solid phase radioimmunoassay. The lupus anticoagulant was detected in all eleven patients tested. It is proposed that anticardiolipin antibodies and the lupus anticoagulant make up a population of antiphospholipid antibodies capable of causing cerebral vascular injury and thrombosis resulting in cerebral infarction. These antibodies may also play a pathogenic role in autoimmune disorders other than lupus where cerebral thrombotic disease is a prominent feature.     

New onset systemic lupus erythematosus with pheochromocytoma

We describe a patient with positive antinuclear and anti-Smith antibodies, proteinuria, and thrombocytopenia suggesting systemic lupus erythematosus (SLE). During hospitalization, the patient developed labile hypertension, tachycardia, and intermittent fever. A computer tomography scan of the abdomen showed an extraadrenal mass, which was confirmed as a pheochromocytoma. After removal of the pheochromocytoma, the patient's symptoms resolved and her serology normalized. Previous case reports describe SLE patients with adrenal pheochromocytomas that presented many years after the diagnosis of lupus. This is a novel case of pheochromocytoma discovered at the onset of SLE, with resolution of SLE manifestations shortly after its removal.     

Recurrent cerebral ischemia and mitral valve vegetation in a patient with antiphospholipid antibodies

A 37-year-old woman presented with a history of acute loss of vision in her left eye and a history of recurrent transient ischemic attacks. Subsequent investigations revealed a prolonged PTT. The lupus anticoagulant and anticardiolipin antibodies (aCL) were identified in her serum. A cardiac murmur was heard and echocardiography demonstrated a mass on the mitral valve. Extensive studies for infection were negative. Cardioembolic phenomena were considered a possible cause of her cerebral ischemic events. The occurrence of nonbacterial endocardial verrucae is well described in systemic lupus erythematosus. The pathogenesis of this lesion remains speculative, however, its occurrence in our patient, with a lupus anticoagulant and aCL suggests a possible association. The clinical manifestations of thrombosis in patients with antiphospholipid antibodies are varied, and may include the development of thrombotic endocardial lesions.     

Antiphosphatidylethanolamine antibody as the sole antiphospholipid antibody in systemic lupus erythematosus with thrombosis.

A previously healthy 34-year-old woman, was diagnosed as having systemic lupus erythematosus (SLE), with membranous glomerulopathy which improved rapidly. Neither lupus anticoagulant nor anticardiolipin antibodies were detected in her plasma. After three months of total remission, she developed a severe pulmonary thromboembolism for which no specific biological cause was found. Her plasma was analysed for different antiphospholipid antibodies: lupus anticoagulant and anticardiolipin antibodies were again negative. Using an ELISA prepared with either five different anionic phospholipids or zwitterionic phosphatidylethanolamine, solely an anti-phosphatidylethanolamine IgG was discovered in her plasma. In lupus patients, the presence of antiphospholipid antibodies is now well recognized as a high risk factor for repeated thrombosis and/or recurrent abortions. This case suggests that the presence of antiphosphatidylethanolamine antibody should be investigated in cases of unexplained thrombosis in SLE, where the usual clinical and biological investigations have failed to shed light.     

An unusual transitory increase of lupus anticoagulant in dengue virus infection complicated with cerebral ischaemia

Dengue is a common mosquito-transmitted viral disease prevalent at many undeveloped and developing countries. Although, neurological complication and hemostatic disturbance are common in dengue virus infection, cerebral ischaemia is barely mentioned. A 61-year-old woman experienced an acute onset of right hemiparesis in her early course of dengue virus type II infection. Extensive laboratory investigation revealed an increase of lupus anticoagulant activity and a prolonged activated partial thromboplastin time that were normalized in the convalescent stage. Her human leucocyte antigens were A2, A11, B8, Cw7, DR4 and DR9. This patient highlights the co-operation between transient activation of humoral immunity and preferential immunogenetic for coagulopathic thrombosis in specific viral disease.     

A case of systemic lupus erythematosus with various central and peripheral neurological disorders developed with motor paralytic bladder as a major manifestation

A 29-year-old female with systemic lupus erythematosus who developed accerelated hypertension, motor paralytic bladder and various other neurological abnormalities is described. Cystometry demonstrated flaccid atonic neuropathic bladder. Elevated protein level and albuminocytologic dissociation were recognized in her cerebral spinal fluid. Magnetic resonance imaging study detected high signal intensities in themedulla oblongata andcauda equina. Her clinical symptoms and laboratory abnormalities were resolved after two courses of methyl-prednisolone pulse therapy. Possible roles of antiphospholipid antibodies were considered in the pathogenesis of her neurologic abnormalities.     

Erratum to: PADI4 polymorphisms and susceptibility to rheumatoid arthritis: a meta-analysis

Abstract

A 29-year-old female with systemic lupus erythematosus who developed accerelated hypertension, motor paralytic bladder and various other neurological abnormalities is described. Cystometry demonstrated flaccid atonic neuropathic bladder. Elevated protein level and albuminocytologic dissociation were recognized in her cerebral spinal fluid. Magnetic resonance imaging study detected high signal intensities in the medulla oblongata and cauda equina. Her clinical symptoms and laboratory abnormalities were resolved after two courses of methyl-prednisolone pulse therapy. Possible roles of antiphospholipid antibodies were considered in the pathogenesis of her neurologic abnormalities.     

Atrioventricular Heart Block and Syncope Coincident With Diagnosis of Systemic Lupus Erythematosus

We describe a 59-year-old woman with cardiac conduction abnormalities caused by lupus-induced myocardial damage. She had a history of arthralgias and antinuclear antibodies but no clinical history of systemic lupus erythematosus. She presented with syncope and Mobitz type II second-degree atrioventricular block. Anti-double-stranded DNA antibodies developed coincident with the identification of heart block. Cardiac magnetic resonance imaging showed late enhancing foci of gadolinium uptake that anatomically correlated with her conduction abnormalities. We conclude that her conduction disease represents an early and structural cardiac manifestation of systemic lupus erythematosus that is unusual in its presentation at the time of initial diagnosis.     

CEREBRAL VENOUS THROMBOSIS AND ACQUIRED PROTEIN S DEFICIENCY: AN UNCOMMON CAUSE OF HEADACHE IN SYSTEMIC LUPUS ERYTHEMATOSUS

A 42-yr-old woman with hypertension and renal involvement dueto systemic lupus erythematosus (SLE) developed unilateral headachefollowed by the sudden onset of confusion and a grand mal convulsion.Cerebral computed tomography was normal. A magnetic resonanceimaging angiogram revealed cerebral venous thrombosis and avenous infarct. Nephrotic syndrome had resulted in an acquiredprotein S deficiency. A review of previous cases suggests thateither renal disease with proteinuria or features of the antiphospholipidsyndrome are prerequisites for the development of cerebral venousthrombosis in SLE. Low free-protein S levels may be an additionalrisk factor. Furthermore it is likely that this condition isunderdiagnosed. KEY WORDS: Cerebral venous thrombosis, Systemic lupus erythematosus, Protein S, Magnetic resonance imaging, Nephrotic syndrome     

Guillain-Barré syndrome accompanied by central nervous system lupus in a patient with juvenile rheumatoid arthritis

We describe a 17-year-old female with juvenile rheumatoid arthritis accompanied by Guillain-Barré syndrome (GBS) during the course of central nervous system (CNS) lupus. She initially developed CNS lupus, including headache and convulsion. A high-signal area in a magnetic resonance scan of her brain with T2-weighted images was noted, and her cerebrospinal fluid exhibited increased levels of IgG and interleukin-6. Eighteen days after the onset of CNS lupus, polyneuropathy in the lower extremities developed, and a diagnosis of GBS was made. No obvious preceding infections in the upper respiratory or gastrointestinal systems were noted before the onset of GBS, indicating that GBS might be part of the symptoms of CNS lupus. Received: June 28, 2000 / Accepted: January 15, 2001     

Complete congenital heart block followed by anti-Ro/SSA in adult life. Studies of an informative family

A family is described in which an asymptomatic woman gave birth to a male child with complete congenital heart block. Precipitating antibodies to the Ro/SSA antigen have developed in this child, now aged 33, who is clinically well. In the mother, features of both systemic lupus erythematosus and Sj?gren's syndrome developed 26 years after the birth of her son. Her serum currently has precipitating antibodies to both the Ro/SSA and La/SSB antigens. The presence of anti-Ro/SSA in the child with complete congenital heart block and the extremely long delay of 26 years between the delivery of the child and the development of clinical disease in the mother are discussed in light of investigators' developing knowledge of both neonatal and adult systemic lupus erythematosus.     

Guillain-Barré syndrome accompanied by central nervous system lupus in a patient with juvenile rheumatoid arthritis

Abstract

We describe a 17-year-old female with juvenile rheumatoid arthritis accompanied by Guillain-Barré syndrome (GBS) during the course of central nervous system (CNS) lupus. She initially developed CNS lupus, including headache and convulsion. A high-signal area in a magnetic resonance scan of her brain with T2-weighted images was noted, and her cerebrospinal fluid exhibited increased levels of IgG and interleukin-6. Eighteen days after the onset of CNS lupus, polyneuropathy in the lower extremities developed, and a diagnosis of GBS was made. No obvious preceding infections in the upper respiratory or gastrointestinal systems were noted before the onset of GBS, indicating that GBS might be part of the symptoms of CNS lupus.     

Dual anca positivity in a child with moyamoya-like cerebral vascular changes: an unusual presentation with sudden homonymous hemianopsia

A 12-year-old girl presented with a sudden decrease in her right visual acuity and homonymous hemianopsia. An angiography of the retinal arteries demonstrated recanalized occlusion of the right retinal artery. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation. Laboratory evaluations revealed dual antineutrophil cytoplasmic antibodies (ANCA) positivity [anti-proteinase (anti-PR3) ANCA and anti-myeloperoxidase (anti-MPO) ANCA], anticardiolipin (aCL) antibodies, and low titers of antinuclear antibodies (ANA). There was no evidence of active systemic lupus erythematosus (SLE), ANCA-related vasculitis, or other risk factors for cerebral occlusion, such as antiphospholipid syndrome (APS). Dual positivity for both cytoplasmic (c-ANCA) and perinuclear (p-ANCA) antineutrophil antibodies has been found previously in a small number of reports, but to our knowledge, this case represents the first case of moyamoya disease associated with dual ANCA positivity.     

Lupus enteritis during pregnancy: A case-based review

Although the symptoms of systemic lupus erythematosus (SLE) worsen during pregnancy, few previous studies have reported lupus enteritis in pregnant women with SLE. A 29-year-old pregnant Japanese woman presented with acute abdomen. Six years before pain onset, she developed pure red cell aplasia and tested positive for anti-Ro (SS-A) and anti-La (SS-B) antibodies. Anti-DNA antibodies were detected two and a half years later. The patient remained asymptomatic until she developed acute abdomen. A mild increase in anti-DNA antibody levels and a mild decrease in complement levels were observed, and abdominal ultrasound and magnetic resonance imaging revealed the presence of large-volume ascites and edematous thickening of the small intestinal wall. These findings established the diagnosis of lupus enteritis. Her condition improved after treatment with prednisolone 50 mg/day, and she delivered a female infant weighing approximately 1810 g at 37 weeks of gestation. Our study suggests that lupus enteritis should be suspected in female patients with autoimmune disease who develop acute abdomen during pregnancy, and that magnetic resonance imaging is useful in its diagnosis.     

Systemic lupus erythematosus and pheochromocytoma.

A 40-year-old Chinese woman with systemic lupus erythematosus (SLE) developed labile blood pressure 3 years after her onset of high blood pressure. Pheochromocytoma was subsequently found and removed. Postoperatively her hypertension improved without change in her SLE activity. Our case demonstrates pheochromocytoma can be a cause of hypertension in SLE.     

Tetraplegia as a presenting feature of systemic lupus erythematosus complicated by pulmonary hypertension.

A 28-year-old woman presenting with subacute onset of a tetraplegia is described who was shown to have active systemic lupus erythematosus in association with high circulating anticardiolipin binding and lupus anticoagulant activity. The patient later developed severe symptomatic systemic and pulmonary hypertension and required emergency resuscitation. This case provides further support for an association between antiphospholipid antibodies and the clinical features of central nervous system (CNS) involvement and pulmonary hypertension in SLE.     

Sarcoidosis presenting as antinuclear antibody positive glomerulonephritis.

A 43 year old woman who initially presented with the nephrotic syndrome, glomerulonephritis, and antinuclear antibodies (ANAs) was given the diagnosis of systemic lupus erythematosus (SLE). One year later the patient developed progressive subcutaneous nodules on her forearms, with histopathology of non-caseating granulomas. Further evaluation of the patient showed mediastinal lymphadenopathy and interstitial lung disease with numerous granulomas, establishing the diagnosis of sarcoidosis. The presence of autoimmune antibodies and glomerulonephritis has been reported in sarcoidosis, but this case is believed to be the first in which both glomerulonephritis and ANAs are present in a sarcoid patient.     

Long-term membranous glomerulonephritis as the presenting manifestation of systemic lupus erythematosus in a patient with human immunodeficiency virus infection

The coexistence of human immunodeficiency virus (HIV) infection and systemic lupus erythematosus (SLE) is unusual, but the occurrence of SLE after HIV infection is even less common. Both conditions share similar clinical features including constitutional symptoms, facial rash, oral ulcers, alopecia, arthralgias, arthritis, seizures, cytopenias, glomerulonephritis, and antinuclear and antiphospholipid antibodies. This clinical overlap makes the diagnosis of SLE in a patient with pre-existing HIV infection difficult. Furthermore, immune complex glomerulonephritis with features resembling lupus nephritis has been described in HIV-positive patients. We present the case of a 45-year-old Hispanic woman with long-standing HIV infection who developed membranous glomerulonephritis with histological features of lupus nephritis. Five years after onset of renal disease she developed clinically evident SLE.     

Reactivation of systemic lupus erythematosus in a dialysis patient after tuberculous peritonitis

The disease activity of patients suffering from lupus nephritis usually becomes quiescent after the onset of end stage renal failure. Reactivation of lupus activity, especially after a long period of dialysis, is uncommon. Factors that might trigger off lupus reactivation after dialysis have not been well defined. We report a case of a 43-year-old Chinese woman on long-term peritoneal dialysis, who developed lupus reactivation with cerebral involvement 2 weeks after she was diagnosed to have tuberculous peritonitis. The close temporal relationship between the tuberculous peritonitis and the lupus reactivation raise the possibility that the tuberculous infection might have triggered off the lupus reactivation.