Multiple brain calcifications in a patient with systemic lupus erythematosus

We describe a rare case of severe neuropsychiatric systemic lupus erythematosus with cerebral atrophy and multiple paraventricular, basal ganglia, cortex, cerebral white matter, and cerebellum calcifications detected on cerebral CT. These calcifications are probably secondary to necrosis focus of repeated episodes of vessels inflammation.     

Multiple brain calcifications in a patient with systemic lupus erythematosus

We describe a rare case of severe neuropsychiatric systemic lupus erythematosus with cerebral atrophy and multiple paraventricular, basal ganglia, cortex, cerebral white matter, and cerebellum calcifications detected on cerebral CT. These calcifications are probably secondary to necrosis focus of repeated episodes of vessels inflammation.     

Rosai-Dorfman disease in a patient with systemic lupus erythematosus

Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a clinically benign, frequently chronic, painless lymphadenopathy. It can also involve extranodal sites. We describe a 37-year-old man with a recent diagnosis of systemic lupus erythematosus and antiphospholipid antibody syndrome who had lacrimal gland and orbital involvement and nodal and extranodal sites with RDD.     

Legionnaires' disease in a patient with systemic lupus erythematosus

A young woman with systemic lupus erythematosus developed a rapidly fatal pneumonia from which no visible or culturable organisms were found. Subsequent stains disclosed typical findings of Legionnaires' disease. A cutaneous portal of entry was suspected and a fulminant lung abscess developed, neither of which has been previously reported in Legionnaires' disease.     

Multiple renal infarction associated with lupus anticoagulant in a patient with systemic lupus erythematosus]

We report a rare case with multiple renal infarction associated with lupus anticoagulant and SLE. A 20-year old woman presented with remitent fever, butterfly rash and, abdominal pain. Laboratory findings showed leukopenia, positive antinuclear and anti-DNA antibodies, and biological false positive for syphilis. Despite a therapy with prednisolone 25 mg/day, the patient showed hypocomplementemia, high titer of anti-DNA antibody and a development of proteinuria and an elevation of serum creatinine. Renal biopsy revealed no abnormalities. She presented abdominal pain with an elevation of serum LDH. Abdominal dynamic computed tomography demonstrated multiple perfusion defects in both kidneys indicating multiple renal infarction. Brain MRI showed multiple micro infarction in the anterior lobes. She was treated with 80 mg of aspirin and have been in remission for two years. Although there have been reported 18 cases with renal infarction associated with antiphospholipid syndrome, this is the first report in Japan. Renal infarction should be differentiated from renal involvement in patients with SLE who have antiphospholipid antibodies.     

Multiple avascular necrosis in a patient with systemic lupus erythematosus/systemic sclerosis overlap syndrome

A white female patient developed overlapping features of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) with severe pulmonary compromise. She was treated with steroids and azathioprine, which improved her clinical condition and spirometric status. In May 2002 she presented with continuous pain in her left ankle that continued even during rest and under treatment with nonsteroidal anti-inflammatory drugs (NSAIDs). Magnetic resonance imaging (MRI) showed multiple avascular necrosis (AVN). Rest and kinesitherapy were indicated for 1 year, and gradually an orthosis was introduced allowing the patient to walk normally.     

Dyspnea in a patient with systemic lupus erythematosus

A 33-year-old woman with a previous history of systemic lupus erythematosus complained of exerptional dyspnea and pleuritic chest pain accompanied by polyarthritis. Chest-X-rays revealed an elevation of the right hemidiaphragm. We discuss the diagnostic and therapeutic approach.     

Multiple microcrystal deposition within a family.

A family is described in which four members in three generations showed evidence of crystal deposition disease: two developed calcium pyrophosphate dihydrate (CPPD) crystal deposition, one calcific periarthritis, and one mixed crystal deposition disease (gout + chondrocalcinosis). This previously undescribed observation supports a possible role for nonspecific heritable connective tissue factors in predisposing to crystal deposition.     

Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus.

We describe a patient with longstanding systemic lupus erythematosus (SLE) in remission who presented with recent onset neurological symptoms. Magnetic resonance imaging, followed by a brain biopsy and in situ hybridization, confirmed the diagnosis of progressive multifocal leukoencephalopathy (PML). The clinical findings in this patient emphasize the importance of considering PML in an individual with SLE and neurological abnormalities.     

Primary hyperparathyroidism in a patient with systemic lupus erythematosus

Primary hyperparathyroidism (PHP) is a metabolic illness that results from autonomous secretion of parathyroid hormone and is one of the most common causes of hypercalcemia. We present the case of a 47-year-old female with a previous diagnosis of systemic lupus erythematosus (SLE) in whom clinical (diffuse bone pain, emotional lability, jaw tumor) and laboratory features (calcium= 13.5 mg/dL, phosphate= 1.8 mg/dL, alkaline phosphatase= 3028 U/L, PTH intact= 1472 pg/dL) prompted the diagnosis of PHP secondary to parathyroid adenoma as demonstrated by the anatomopathology. After treatment with calcitonin spray 400 UI per day, IV pamidronate 90 mg/week, and subtotal parathyroidectomy, the patient status improved with normal laboratory tests. This is the second report to describe the coexistence of these two disorders in a single patient. Although the pathophysiology of the association of PHP and SLE is not known, the recognition of this association has a practical implication since the therapeutical strategy is completely different.     

Lymphocytic hypophysitis in a patient with systemic lupus erythematosus

A case of lymphocytic hypophysitis in a patient with systemic lupus erythematosus is described. A 20-year-old woman was admitted to our hospital with generalized myalgia and facial rash in May 1998. The patient had a medical history, physical examination, and laboratory findings compatible with systemic lupus erythematosus (SLE). Headache and nausea had developed 3 months previously and worsened over the following months. Hormonal investigation showed hypopituitarism except for prolactin. A magnetic resonance image of the brain showed a mass lesion in the pituitary fossa. A trans-sphenoidal surgical procedure was performed which revealed a dark-yellowish hematoma. Microscopic examination showed diffuse infiltration of lymphocytes and plasma cells with fibrosis in the anterior pituitary. Post-operatively the patient's headaches and nausea resolved. This indicates that lymphocytic hypophysitis may be associated with SLE.     

Hydroxychloroquine-induced seizure in a patient with systemic lupus erythematosus

We report on a case of a 17-year-old female with systemic lupus erythematosus (SLE), with a clinical history of complex partial seizure, who developed a tonicoclonic crisis after receiving hydroxychloroquine for 2 weeks at a dosage of 200 mg/day (5 mg/kg). The absence of previous similar episodes and of recurrences after withdrawal of the drug in subsequent months, the short latency after administration and the favourable short-term evolution raised suspicions for a potential role of the drug in the development of the isolated convulsive crisis. It is possible for hydroxychloroquine to be responsible for tonicoclonic seizures in predisposed subjects. Received: 19 March 2000 / Accepted: 16 June 2000     

Acquired Brown''s syndrome in a patient with systemic lupus erythematosus.

A 27 year old woman with systemic lupus erythematosus (SLE) developed vertical diplopia with an apparent bilateral inferior oblique muscle palsy, resulting in a limitation of elevation of the globe in adduction. It resolved with systemic steroid treatment. A transient tenosynovitis affecting the superior oblique tendons was the probable underlying pathological mechanism. This is the first described case of Brown's syndrome associated with SLE.     

Acquired hemophilia A in a patient with systemic lupus erythematosus.

A patient with systemic lupus erythematosus (SLE) developed acquired hemophilia A. The patient, a 24-year-old Japanese woman, was referred to our hospital because of uncontrollable bleeding following a tooth extraction. Laboratory examination revealed prolonged APTT (116 seconds), reduced factor VIII activity (2.8 %) and the presence of factor VIII inhibitor at a titer of 46.5 Bethesda units/ml. Transfusion of prothrombin complex concentrate and activated prothrombin complex concentrate followed by administration of prednisolone and cyclophosphamide successfully arrested bleeding and reduced the factor VIII inhibitor level. Acquired hemophilia A is a rare but lethal condition. Rapid diagnosis and introduction of adequate therapies are critical.