Idiopathic portal hypertension associated with systemic lupus erythematosus

A case of idiopathic portal hypertension (IPH) associated with systemic lupus erythematosus (SLE) is reported in a 38-year-old man who had been diagnosed with SLE and treated for 18 years. Esophageal varices, found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996, he was admitted to Nagoya University Hospital because there was a high risk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytopenia, splenectomy and devascularization of the stomach vessels were performed after endoscopic ligation of the esophageal varices. Histological specimens of wedge biopsied liver showed chronic inactive hepatitis without cirrhosis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunological mechanism in the etiology of SLE and IPH. Received: January 20, 1999 / Accepted: July 23, 1999     

Monoclonal gammopathy in systemic lupus erythematosus

We studied the prevalence, type and associated features of monoclonal gammopathy in patients with systemic lupus erythematosus (SLE). Patients included in the University of Toronto Lupus Database with an abnormal band on serum electropheresis were identified. Monoclonal gammopathy patients were matched with two controls each from the same database by age at SLE diagnosis, sex and disease duration. Of 1083 patients followed at the Lupus Clinic 59 (5.4%) were identified with monoclonal gammopathy. The gammopathies included 32 with IgG, 14 IgM and 12 IgA, one undefined. Nine (15.3%) malignancies were detected in monoclonal gammopathy and 12 (10.1%) in the controls during the entire course of their disease (P = 0.13). None had multiple myeloma. There was no difference between patients with monoclonal gammopathy and their controls with respect to disease activity, damage, or dose of steroids. The mean ESR and gammaglobulin levels in the monoclonal gammopathy patients were higher than the controls at last visit. We conclude that monoclonal gammopathy is more frequent in SLE patients than in the general population and has a benign course in patients with SLE. There were no differences in disease manifestations, treatment approaches, or malignancies between SLE patients with and those without monoclonal gammopathy.     

Monoclonal gammopathy in systemic lupus erythematosus.

In order to ascertain the incidence of monoclonal gammopathy (MG) in systemic lupus erythematosus (SLE), 120 SLE patients were evaluated. Four patients (3.3%), two of whom were under 50 years of age, were found to have a monoclonal immunoglobulin in their serum. Our data agree with the previous literature in demonstrating a higher incidence of MG in SLE patients in comparison with the general population. Neither the clinical importance nor the pathogenetic significance of this association is known.     

Narcolepsy associated with systemic lupus erythematosus

Many neurologic and psychiatric manifestations have been associated with systemic lupus erythematosus. Narcolepsy, currently hypothesized as related to an autoimmune process, has been rarely associated with systemic lupus erythematosus. We report a 36-year-old woman who presented with narcolepsy and who subsequently developed systemic lupus erythematosus. Excessive daytime sleepiness resolved after the administration of four intravenous bolus of cyclophosphamide and methylprednisolone followed by maintenance therapy with hydroxychloroquine, aspirine and prednisone. Narcolepsy should be included in the neuropsychiatric manifestations of systemic lupus erythematosus and it may have a parallel clinical course to the activity of the lupus.     

Idiopathic portal hypertension associated with connective tissue disease similar to systemic lupus erythematosus

A case of idiopathic portal hypertension associated with connective disease resembling systemic lupus erythematosus is described. The patient was a 50-year-old woman with splenomegaly, ascites, esophageal varices, and pancytopenia, but without extrahepatic portal obstruction or cirrhosis of the liver. Electron microscopy of the liver showed perisinusoidal fibrosis. High titers of autoantibodies against proliferating cell nuclear antigen (PCNA) were found in the sera as well as in ascites; anti-DNA antibodies appeared after anti-PCNA antibodies and remained thereafter at a moderate titer. The possibility of an immunological process in the pathogenesis of idiopathic portal hypertension is discussed.     

Acquired ichthyosis associated with systemic lupus erythematosus

Acquired ichthyosis is an uncommon disease, it is characterized by symmetric scaling of the skin, which ranges from minor roughness and dryness to the desquamation of large plaques. It has been associated with various systemic diseases, although malignant conditions, especially Hodgkin's lymphoma are the most commonly cited. Drugs, HIV infection and autoimmune diseases such as dermatomyositis and mixed connective tissue disease have also been associated, and it has only rarely been found among patients with systemic lupus erythematosus (SLE). Herein, we report a patient with active SLE who developed a generalized acquired ichthyosis corroborated with skin biopsy, which completely disappeared after treatment. The association of autoimmune conditions with acquired ichthyosis could indicate that an abnormal host immune response, probably against components of the granular cell layer in particular the keratohyalin granules, may have a role in the pathogenesis. Thus, this finding may be a relevant cutaneous marker for an autoimmune origin.     

Morbidity associated with childhood systemic lupus erythematosus

A complete analysis of the outcome of childhood onset systemic lupus erythematosus (SLE) requires determination of survival statistics and consideration of disease and treatment morbidity. The course of 32 patients with SLE diagnosed at or prior to age 16 and followed from 1979-1988 was analyzed with emphasis on the morbidity of pediatric SLE. Clinical characteristics were similar to those in previous studies. Five year survival, calculated by life-table analysis, was 85.3%, comparable to recent studies. Treatment consisted of corticosteroids (32 patients), antimalarials (14), cyclophosphamide (9) or azathioprine (2). Eighty-four percent of patients experienced a noninfectious drug related complication. A major infection contributed in all 5 patients who died. Higher total corticosteroid dose was associated with more severe infections. Eighty-eight percent of patients were left with chronic organ dysfunction. Survival in childhood onset SLE has improved, but morbidity remains a significant problem from organ dysfunction and complications of therapy. Our study is the first to address the issue of morbidity in childhood SLE.     

AA amyloidosis associated with systemic lupus erythematosus

Summary A 54-year-old female with a 23-year history of systemic lupus erythematosus was admitted because of loss of renal function and nephrotic syndrome. Renal biopsy showed deposition of AA amyloid as demonstrated by Congo red staining and reactivity with protein AA-specific antibodies. Immune deposits were present in the mesangium and the glomerular basement membrane, but histopathological changes consistent with lupus nephritis were not detected. The rare association of systemic lupus erythematosus and amyloidosis is discussed in view of the characteristics of the acute phase reaction in systemic lupus erythematosus.     

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.     

Disseminated zygomycosis associated with systemic lupus erythematosus

We describe a 36-year-old patient who had systemic lupus erythematosus (SLE) and died of disseminated zygomycosis. There was rapid progression of his SLE, leading to suspicion of superimposed infectious disease, but the actual cause of his multiorgan failure was not recognized until after death. The manner in which systemic fungal infection may mimic SLE is discussed.     

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.     

Pulmonary amyloidosis associated with systemic lupus erythematosus

The association between amyloid and systemic lupus erythematosus is rarely reported. Our patient, a 26-year-old woman with systemic lupus erythematosus, developed steroid-resistant exertional dyspnea and pulmonary fibrosis, without proteinuria or the nephrotic syndrome. Our studies showed that she had pulmonary amyloidosis.     

Aortic stenosis associated with systemic lupus erythematosus

Hemodynamically significant valvular lesions have been rarely reported sequelae of Libman-Sacks endocarditis complicating systemic lupus erythematosus (SLE). Furthermore, embolic phenomena associated with these vegetations have not been clearly documented. We present a report of critical aortic stenosis associated with SLE in a patient who had received corticosteroid treatment for several years. An embolus, histologically identical with the aortic valve vegetation, was found in the left anterior descending artery at necropsy. There was no evidence of rheumatic heart disease, bacterial endocarditis or a bicuspid aortic valve. Recent reports suggest an increased incidence of significant valvular dysfunction in patients with SLE who have received long-term corticosteroid treatment.     

Autoimmune cholangiopathy associated with systemic lupus erythematosus

We report a 39-year-old female who presented over 11 years with autoimmune cholangiopathy associated with kaleidoscopic manifestations of systemic lupus erythematosus (SLE), including, arthritis, skin changes, pleuritis, diffuse proliferative glomerulonephritis, lymphadenopathy, splenomegaly, hyperglobulinemia, and major depression. While antimitochondrial antibodies (AMA) were absent, antinuclear (ANA) and anti-DNA antibodies were detected in high titres associated with hypocomplementemia. The patient also had vitamin B12 deficiency and antiphospholipid antibodies. The patient required steroids and repeated courses of cyclophosphamide for the management of lupus nephritis, and ursodeoxycholic acid (ursolite) administration resulted in amelioration of cholestatic laboratory abnormalities. This unusual case report and review of literature illustrate that immune liver disease may be an important clinical manifestation of SLE, especially autoimmune cholangiopathy.     

Eosinophilic gastroenteritis associated with systemic lupus erythematosus

Eosinophilic gastroenteritis is an uncommon disease with an obscure etiology, although associations with allergy, the idiopathic hypereosinophilic syndrome, and connective tissue disease have been reported. We present the case of a 37-year-old woman with a history of idiopathic thrombocytopenic purpura who presented with refractory nausea, vomiting, and abdominal pain. Imaging studies were significant for bowel wall thickening and ascites, while laboratory studies revealed a positive antinuclear antibody (ANA), a positive anti-double stranded (DS) DNA antibody, low complement, and proteinuria. Exploratory laparotomy with gastric and small bowel biopsies established the diagnosis of eosinophilic gastroenteritis. In addition, the patient met clinical criteria for the diagnosis of systemic lupus erythematosus. Previous studies have described eosinophilic gastroenteritis in patients with scleroderma, polymyositis, or dermatomyositis. This is the first report to our knowledge of an individual with eosinophilic gastroenteritis and systemic lupus erythematosus.     

Intravascular lymphoma associated with systemic lupus erythematosus

We report a case of systemic lupus erythematosus (SLE) associated with intravascular (angiotropic) lymphoma. A 27‐year‐old woman had been suffering from uncontrolled severe eruptions for a long time and was admitted because of high fever due to hemophagocytic syndrome (HPS). As her SLE had not been well‐controlled by moderate doses of steroids and azathioprine, autoimmune associated HPS was first considered. She was initially treated with steroid pulses and γ‐globulin for HPS. However, chromosomal analysis of bone marrow cells revealed severe abnormalities. After malignant lymphoma‐associated HPS was diagnosed, chemotherapy was commenced in the intensive care unit with artificial respiration and continuous hemodiafiltration. The patient died of cerebral infarction at day 45. It is suggested that the SLE itself was associated with the development of the intravascular lymphoma rather than due to the azathioprine.