Localized pericardial inflammation in systemic lupus erythematosus

Regional or localized pericarditis has been infrequently reported. We report a patient with systemic lupus erythematosus (SLE), who presented with retrosternal pleuritic-type chest pain without audible friction rub, electrocardiographic changes or detectable pericardial effusion on echocardiography. Computed tomography, however, revealed a circumscribed area of pericardial inflammation, suggesting a diagnosis of localized lupus-associated pericarditis. This case demonstrates that localized pericarditis may occur in SLE and that chest CT may be required as part of the work-up in the diagnosis of lupus pericarditis.     

Pulmonary hypertension in systemic lupus erythematosus: a report of three cases

Three women with systemic lupus erythematosus (SLE) who developed pulmonary hypertension as a terminal feature of their illness, are reported. One patient with quiescent SLE had marked sicca syndrome when pulmonary hypertension developed. Two of the 3 patients possessed the "lupus anticoagulant" and antibodies to cardiolipin. All 3 patients had positive rheumatoid factor in their sera.     

Pulmonary hypertension in systemic lupus erythematosus: a report of four cases

Overt pulmonary hypertension in systemic lupus erythematosus (SLE) is rarely reported. We have documented 4 such patients, all of whom had severe extrapulmonary manifestations of the disease and also Raynaud's phenomenon. The relationship between Raynaud's phenomenon and pulmonary hypertension in SLE is borne out by a review of the literature. A common vasospastic aetiology is suggested.     

Cardiovascular involvement in systemic lupus erythematosus: report of two cases

Cardiac abnormalities has been receiving increased attention in patients with systemic lupus erythematosus (SLE). Cardiovascular system involvement has been found to have a substantial effect on mortality and morbidity in patients with SLE [1]. Recent diagnostic methods using echocardiography examination have allowed the delineation of cardiac manifestations such as myocarditis and myocardial dysfunction, valvular disease, pericardial disease or pulmonary hypertension. A report of two cases is presented: 23-year-old man with acute myocarditis with left ventricular failure and pulmonary oedema as a initial presentation of active SLE, and 51-year-old woman with SLE, antiphospholipid antibodies, with history of cerebral embolic infarction, TIA and venous thrombosis and with mitral valvular dysfunction in course of nonbacterial thrombotic endocarditis. Pulmonary hypertension has been recognised in both patients probably as a result of vasculaopathy and intimal proliferation, vasculitis, thromboembolic disease or parenchymal lung disease in SLE. Recent advances in diagnosis and treatment have substantially improved the prognosis of patients with systemic lupus erythematosus and cardiovascular system involvement [2].     

Laryngeal involvement in systemic lupus erythematosus: report of two cases.

Systemic lupus erythematosus is a multisystem autoimmune disorder. Joint and skin involvements are the most frequent presenting features. Laryngeal involvement, however, is extremely rare. Symptoms of laryngeal involvement may range from mild hoarseness to life-threatening respiratory distress. In this article, 2 patients with systemic lupus erythematosus and laryngeal involvement are presented. The first patient had hoarseness and noisy respiration, and the second had cough and respiratory distress. Otorhinolaryngological examination revealed laryngeal inflammation in both. Our cases responded well to systemic corticosteroids. Patients with symptoms such as hoarseness, foreign body sensation in throat, and respiratory distress should be evaluated by an otorhinolaryngologist to rule out involvement of the laryngeal structures.     

Childhood systemic lupus erythematosus: a review of 30 cases

We review 30 cases of pediatric systemic lupus erythematosus followed over an 8-year period at our institution. The female to male ratio was 3.3:1; the age at diagnosis ranged between 3.5 and 16 years. On first admission, renal involvement was detected in the majority of the patients, as assessed by laboratory findings and/or clinical manifestations. Other frequently observed symptoms were fever, skin rashes, arthralgias and/or arthritis and serositis. All of the patients were treated with corticosteroids and most of them also received immunosuppressive drugs in order to control disease activity. Two patients were lost to the follow-up, five died and only one of the 23 evaluable patients is off therapy after a median follow-up of 5 years. This study confirms that pediatric systemic lupus erythematosus is a very aggressive disease.     

Protein losing enteropathy due to systemic lupus erythematosus.

We report the case of a 29 year old woman with a protein losing enteropathy caused by systemic lupus erythematosus presenting with periorbital oedema. Only three other cases of protein losing enteropathy due to systemic lupus erythematosus have been described, two of which were thought to be because of a primary enteropathy, although the exact pathogenesis was unknown. We suggest that both the protein losing enteropathy and periorbital oedema in this patient were because of increased capillary permeability to serum albumin, as a result of products of plasma C3 conversion which were present in large amounts. It is also of interest that the antigen/antibody system in this patient was RNP/anti-RNP and that DNA antibodies were not detected. This patient falls into a subset of systemic lupus erythematosus in which anti-DNA antibodies are not present, some of which appear to have a more favourable prognosis.     

Dubin-Johnson syndrome with systemic lupus erythematosus: a case report

Introduction Dubin-Johnson syndrome (DJS) is a rare recessively inherited conjugated hyperbilirubi- nemia caused by deficiency of the canalicular multi-drug resistance/multi-specific organic anionic transporter protein (MDR2/cMOAT). Thus bilirubin is conjugated but inefficiently secreted into bile, which results in accumulation of conjugated and, to some extent, unconjugated bilirubin in blood, leading to hyperbilirubinemia and bilirubinuria. But, the results of liver function tests are no…     

Coeliac disease in systemic lupus erythematosus: a case report

The case of a rare coexistence of systemic lupus erythematosus (SLE) with coeliac disease (CD) is described. Systemic lupus erythematosus was diagnosed prior to CD and initially SLE treatment was administered. After several years, when CD symptoms developed, the diagnosis was corrected and additional treatment with a gluten-free diet was applied with beneficial effects.     

Acute respiratory distress syndrome due to systemic lupus erythematosus with hemophagocytic syndrome: an autopsy report

This report concerns a patient with systemic lupus erythematosus (SLE) who died of acute respiratory distress syndrome (ARDS) 1 day after the onset of pulmonary symptoms. Autopsy demonstrated severe hemophagocytosis in the bone marrow and histopathology indicating a marked increase in vascular permeability in both lungs and kidneys. In this patient, active SLE and associated hemophagocytic syndrome may have induced an increase in the production of inflammatory cytokines, which immediately induced ARDS. Since fatal ARDS can occur as a life-threatening complication of SLE, careful observation is necessary, particularly when there are clinical findings suggestive of associated hemophagocytic syndrome.     

Liver dysfunction due to apoptosis in a patient with systemic lupus erythematosus

We report on a 23-year-old Japanese female with a 13-year history of systemic lupus erythematosus (SLE), and two episodes of deterioration followed by treatment with high dose prednisolone. Although she had been recently treated with prednisolone (12.5 mg daily), her liver function became worse in July 1998. Results of a liver biopsy revealed multi-focal hepatic cell death in a severe fatty liver, without any inflammatory cell invasion. The biopsy also showed a positive TUNEL (Tdt-catalysed DNA nick end labelling) reaction indicating apoptosis. Her liver function recovered rapidly following steroid pulse therapy. Serum soluble Fas ligand (sFasL) was found to be elevated to a concentration of 0.395 ng/ml at the time of liver damage, but was less than 0.03 ng/ml before liver damage and after prednisolone treatment. The liver damage in this case appeared to be involved with apoptosis induced by sFasL. Although hepatitis associated with SLE is rare, apoptosis directly related to elevated sFasL levels might cause this complication.     

Stevens-Johnson syndrome due to prednisone in a patient with systemic lupus erythematosus

Stevens-Johnson syndrome is a systemic, mucocutaneous disease, frequently related to drugs. We present the case of a 35-year old patient with a diagnosis of systemic lupus erythematous treated with prednisone who developed a Stevens-Johnson syndrome and was treated with methylprednisolone.     

Hemochromatosis as a secondary condition to systemic lupus erythematosus: A case report

Systemic lupus erythematosus (SLE) is an autoimmune disorder that affects multiple organs and systems, including joints, the cardiovascular system, lungs, skin, kidneys, the nervous system, and blood. The clinical presentations of SLE are diverse and vary widely. In this report, we present a case of a patient whose SLE was complicated by hemochromatosis to enhance clinicians' comprehension of this infrequent or rare complication of SLE. We aim to provide insights into the diagnosis and treatment processes of this condition.