Primary pulmonary hypertension associated with systemic lupus erythematosus

Summary A 24-year-old West Indian woman with a four-year history of systemic lupus erythematosus presented with progressive dypsnoea due to primary pulmonary hypertension. Despite showing a response to vasodilators, these failed to control the condition. Her pulmonary hypertension increased in severity, eventually resulting in her death. We believe primary pulmonary hypertension to be an unusual complication of systemic lupus erythematosus. We suggest that this diagnosis should be considered in all patients with systemic lupus erythematosus and progressive dypsnoea, as optimum benefit can only be obtained by early institution of vasodilator therapy.     

C4 deficiency state in antiphospholipid antibody-related recurrent preeclampsia evolving into systemic lupus erythematosus

The case of an apparent healthy woman who developed recurrent preeclampsia with antiphospholipid antibodies and evolved towards systemic lupus erythematosus during her last pregnancy is presented. The diagnostic dilemma between lupus renal flare and toxemia is discussed. The potential role of immunological alterations, such as complement genetic deficiencies, in women with primary antiphospholipid syndrome who develop systemic lupus erythematosus is also discussed.     

Acute pancreatitis: an initial presentation of systemic lupus erythematosus

Acute pancreatitis is a rare, but fatal, manifestation of systemic lupus erythematosus. Only 10 systemic lupus erythematosus-associated pancreatitis cases were found in a search of published articles. We report a 24-year-old woman without significant medical history, who was admitted with abdominal pain, nausea and vomiting, which was diagnosed as pancreatitis. It was discovered to be the initial presentation of systemic lupus erythematosus. The first time she was admitted, she recovered with conservative management and steroid therapy. Two months later, she was readmitted to our hospital with symptoms and signs of acute abdomen, which was attributed to her discontinuation of the therapeutic regimen with corticosteroids just after her previous discharge. She underwent laparotomy twice for signs of peritonitis. Despite administration of a monoclonal antibody, rituximab, she died due to the progression of systemic lupus erythematosus activity.     

OESTROGEN-INDUCED SYSTEMIC LUPUS ERYTHEMATOSUS

The subject of this case report, a 64-year-old white female,was treated for osteoporosis with oestrogens. She developedunequivocal systemic lupus erythematosus, from which she hadsuffered as a young woman and which had remitted with her menopauseat 38. The close relationship of the relapses and remissions of herdisease with her hormonal status underlines the importance ofendocrine factors in the clinical expression of systemic lupuserythematosus. KEY WORDS: Oestrogens, Systemic lupus erythematosus, Osteoporosis     

Chronic predominant interstitial nephritis in a patient with systemic lupus erythematosus: a follow up of three years and review of the literature.

Predominant interstitial nephritis is a rare manifestation of systemic lupus erythematosus. Only seven cases have been reported in the literature. Owing to the rarity of this entity, the natural history of predominant interstitial nephritis in lupus has not been adequately recorded and an appropriate therapeutic approach has yet to be defined. In this report we present the case of a 25 year old woman with active systemic lupus erythematosus complicated by kidney failure and renal tubular acidosis due to predominant interstitial nephritis. We describe the course of her disease over a three year period. Seven additional patients with systemic lupus erythematosus and predominant interstitial nephritis are reviewed.     

Encefalitis con estatus convulsivo localizado en una paciente con lupus eritematoso sistémico

We describe a 42-year old woman with inactive systemic lupus erythematosus (SLE) at the time of her visit. She presented with an atypical movement disorder and partial continuous epilepsy. A brain biopsy excluded cerebral vasculitis and tumoral processes. We discuss the differential diagnosis of status epilepticus in a patient with systemic lupus erythematosus.     

Primary malignant lymphoma of the spleen in systemic lupus erythematosus

Malignant lymphoma has been infrequently reported in the course of systemic lupus erythematosus (SLE). We report a case of primary malignant lymphoma of the spleen, an unusual site of primary lymphoma, complicating the course of systemic lupus erythematosus (SLE). Our patient had been treated with low dose prednisone and hydroxychloroquine only, and her lupus was quiescent at the time of presentation with the lymphoma. Thus primary malignant lymphoma should be considered in the differential diagnosis of splenomegaly in SLE.     

Primary Sclerosing Cholangitis Occurring in a Patient with Systemic Lupus Erythematosus and Diabetes Mellitus

A 42-year-old woman with a longstanding history of systemic lupus erythematosus and insulin-dependent diabetes mellitus was admitted to the hospital because of icterus, anorexia, pruritus, and weight loss of 3 months duration. During her evaluation the diagnosis of primary sclerosing cholangitis was established. The following case documents the unusual association of systemic lupus erythematosus and insulin-dependent mellitus with primary sclerosing cholangitis.     

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.     

Bilateral nephrectomy for the treatment of refractory lupus nephritis with features overlapping with thrombotic microangiopathy resembling thrombotic thrombocytopenia purpura

We report a patient with a diagnosis of systemic lupus erythematosus who concurrently developed a syndrome of thrombotic microangiopathy that resembled thrombotic thrombocytopenic purpura. The patient underwent plasma exchange and immunosuppressive therapy for months before clinical improvement was finally achieved through bilateral nephrectomy. Ultimately, our patient died of disseminated aspergillosis from prolonged immunosuppression. We believe that recognition of bilateral nephrectomy as a potential treatment earlier in her course would have spared her this unfortunate demise. We hope that this review of current literature will help the reader to consider bilateral nephrectomy in patients with refractory systemic lupus erythematosus with clinical overlap of thrombotic microangiopathy resembling thrombotic thrombocytopenic purpura.     

RAPID DEVELOPMENT OF CEREBRAL ATROPHY IN SYSTEMIC LUPUS ERYTHEMATOSUS

A 21-year-old female is described who developed severe cerebrallupus 9 months after the onset of her disease. A dramatic changein her CT brain scan from normal to gross cerebral atrophy wasobserved in the month following onset of her cerebral disease. This patient developed hepatitis during the course of her hospitalization.This is an unusual manifestation of systemic lupus. The patient'sserum also contained anti-Sm antibodies. This is a relativelyinfrequent finding in the serum of lupus patients outside theUnited States. KEY WORDS: Systemic lupus erythematosus, Cerebral disease, Sm antibodies     

Immunological studies of the placenta in systemic lupus erythematosus.

An immunological study was made of the placentae from 5 mothers with lupus erythematosus. 3 of the 5 mothers had anti-DNA antibodies in their sera at the time of delivery and in one of these anti-DNA antibodies were detected in the cord blood. This patient had active renal disease and serological evidence suggestive of circulating immune complexes in her blood at the time of delivery. Immunofluorescence studies showed granular deposition of immunoglobulin and C3 on the trophoblast basement membrane similar to that previously described on the glomerular basement membrane in systemic lupus erythematosus. Anti-DNA antibodies were eluted from the placenta in this case. We suggest that immune complex deposition on the trophoblast basement membrane in patients with active systemic lupus erythematosus may play a part in the increased fetal mortality in this disease.     

Portal and pulmonary hypertension in a case of systematic lupus erythematosus: possible relationship with a clotting abnormality

A young woman with systemic lupus erythematosus developed portal hypertension and pulmonary hypertension. This is the first report of such a case. We suggest that the presence of the lupus anticoagulant may have been related to the development of these two unusual features of her illness.     

A human T-cell lymphotropic virus type-1 (HTLV-1) carrier complicated with various autoimmune diseases including primary biliary cirrhosis.

A 47-year-old woman with primary biliary cirrhosis and scleroderma was examined at our hospital for a 1-week history of non-resolving fever, arthralgia, myalgia, muscle weakness and fatigue. A diagnosis of systemic lupus erythematosus was made based on arthralgia, low leukocyte count, low lymphocyte count, low serum concentration of complements, positive anti-nuclear antibody and positive anti-double-strand-DNA antibody. She was negative for anti-U1RNP antibody, but positive for anti-Jo1 antibody, and her initial serum concentration of creatine phosphokinase was elevated. We diagnosed her as having overlap syndrome with scleroderma, systemic lupus erythematosus and possible polymyositis associated with primary biliary cirrhosis. Prednisolone rapidly improved her symptoms. Lobulated leukocytes were observed in her peripheral blood specimen. She was positive for anti-HTLV-1 antibody, but Southern blot hybridization did not confirm monoclonal integration of HTLV-I proviral DNA in her peripheral blood. This suggests the possibility of a relationship between HTLV-1 infection and various autoimmune disorders including primary biliary cirrhosis.     

Pulmonary haemorrhage, pulmonary infarction, and the lupus anticoagulant.

A patient with systemic lupus erythematosus developed pulmonary haemorrhage and pulmonary infarction as rare initial manifestations of her disease. The latter was associated with the presence of the circulating lupus anticoagulant. She recovered with pulse doses of methylprednisolone and plasmapheresis. Anticoagulants were not administered.     

Autoimmune hyperlipidemia in a child with autoimmune hepatitis

The first reported case of a girl with a combination of autoimmune hyperlipidemia and autoimmune hepatitis is described. She presented at the age of 9 years with fever, headaches, and abnormal lipid profile. Months later, she had clinical manifestations, biochemical findings, and the histologic picture of autoimmune hepatitis. Subsequently, she also showed signs and symptoms of systemic lupus erythematosus. All of her clinical manifestations and biochemical abnormalities dramatically improved with immunosuppression. The overlapping syndrome of systemic lupus erythematosus, autoimmune hepatitis, and autoimmune hyperlipidemia is discussed.     

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.     

Budd-Chiari syndrome as the major thrombotic complication of systemic lupus erythematosus with the lupus anticoagulant.

A patient with systemic lupus erythematosus was first diagnosed after she had developed the Budd-Chiari syndrome. We believe that the Budd-Chiari syndrome was part of her tendency for thrombosis, induced by the presence of a lupus anticoagulant. The association between enhanced thrombosis and the lupus anticoagulant is discussed, and the need for prompt recognition and administration of anticoagulant therapy is emphasised.     

Idiopathic anasarca associated with oligoclonal gammopathy in systemic lupus erythematosus

We describe a young woman with systemic lupus erythematosus with acute onset of severe idiopathic anasarca and the appearance of serum IgG kappa oligoclonal bands. We hypothesize that her anasarca was due to a defect in capillary permeability, perhaps related to the clonally restricted immunoglobulins detected in her serum.     

Clinical and laboratory characteristics of Finnish lupus erythematosus patients with cutaneous manifestations

Our objective was to characterize clinical features, laboratory findings, concomitant autoimmune diseases, and smoking habits of lupus erythematosus subgroups in genetically homogeneous patients from two Dermatology Departments of Finnish University hospitals. One hundred and seventy eight discoid lupus erythematosus, 55 subacute cutaneous lupus erythematosus, and 77 systemic lupus erythematosus patients were enrolled using patients' charts from institutional database (1995-2006) and during routine control visits. Clustering analysis was performed to reveal natural groupings. Smoking at the onset of disease was significantly more common in all subgroups (57% for discoid lupus erythematosus, 35% for subacute cutaneous lupus erythematosus, and 34% for systemic lupus erythematosus) compared with the age/gender-matched prevalence in the Finnish population, suggesting smoking to be a trigger factor for cutaneous lupus. Leukopenia (38%) and lymphopenia (52%) were observed more often in patients with systemic lupus erythematosus than reported previously. Photosensitivity characterized all groups, especially patients with subacute cutaneous lupus erythematosus (87%). Of the autoimmune diseases, Sj?gren's syndrome was the most common (22% of patients with systemic lupus erythematosus), followed by autoimmune thyroid disease (13% of patients with subacute cutaneous lupus erythematosus). The clustering analysis showed environmental factors (smoking) to be more involved in disease development in discoid lupus erythematosus, whereas immunological factors were more significant in initiating systemic lupus erythematosus. The high prevalence of autoimmune thyroid disease, together with photosensitivity, and the clustering profiles suggest that lupus erythematosus subtypes, especially discoid lupus erythematosus, are heterogeneic in their pathomechanisms.