History Of Systemic Lupus Erythematosus Research Articles

Stevens–Johnson syndrome induced by mizoribine in a patient with systemic lupus erythematosus

A 32-year-old Japanese woman, who had a treatment history of systemic lupus erythematosus (SLE) with lupus nephritis World Health Organization class IV for 11 months, visited our hospital due to fever, facial erythema, and erosion of the oral cavity on November 10, 2003. Her mucosal erosion and facial skin erythema progressed over the following week, and Stevens-Johnson syndrome was diagnosed due to pathological findings of the skin. Among the administrated drugs, only mizoribine, started 6 months earlier, produced a positive reaction in the drug lymphocyte stimulation test. Increased prednisolone and high dose intravenous gamma-globulin were given successfully. Cyclosporine at 50 mg was administered to control the SLE, followed by an increase to 100 mg on January 7, 2004. She suffered from abdominal pain, blindness, and convulsion on January 9. The magnetic resonance image of her brain prompted a diagnosis of reversible posterior leukoencephalopathy syndrome. After withdrawal of cyclosporine and control of hypertension, symptoms disappeared rapidly. Cyclophosphamide pulse therapy was successfully administrated to control lupus nephritis. This is the first report describing the relationship between Stevens-Johnson syndrome and mizoribine. Although the use of mizoribine is thought to be safe, careful observation is necessary.

Acute occlusive large vessel disease leading to fatal stroke in a patient with systemic lupus erythematosus: Arteritis or atherosclerosis?

A woman with a history of systemic lupus erythematosus presented with extensive bilateral strokes due to acute inflammatory, occlusive large vessel disease affecting several aortic branches including the carotid, subclavian, renal, and iliac arteries. We quantitatively characterized the arterial inflammation in this patient and compared it with the inflammatory infiltrates from 22 patients with conventional atherosclerosis. Profound histomorphologic differences from conventional atherosclerosis (predominance of CD8-positive lymphocytes, relative absence of macrophages, no ectopic neovascularization, no signs of plaque hemorrhage, concentric instead of eccentrical stenosis) suggest that this patient's accelerated arteriopathy was precipitated by pathogenic events other than conventional atherosclerosis.

Familial characteristics of autoimmune and hematologic disorders in 8,406 multiple myeloma patients: A population‐based case‐control study

A population-based case-control study was conducted to evaluate risk of developing multiple myeloma (MM) associated with personal history of autoimmune diseases and occurrence of autoimmune and selected hematologic disorders in first-degree relatives. Data were obtained for all (n = 8,406) MM cases diagnosed in Sweden (1958-1998), with linkable relatives, 16,543 matched controls and first-degree relatives of cases (n = 22,490) and controls (n = 44,436). Odds ratios (ORs) were calculated to quantify the risk of MM in relation to personal/family history of 32 autoimmune disorders. Familial aggregation of malignancies was evaluated in a marginal survival model using relatives as the cohort. The risk for MM was significantly elevated among subjects with a personal history of pernicious anemia (OR = 3.27; 2.22-4.83) and individuals with a family history of systemic lupus erythematosus (OR = 2.66; 1.12-6.32). Compared with controls, relative risk (RR) of MM was significantly increased (RR = 1.67; 1.02-2.73) in relatives of cases, particularly relatives of probands aged > or =65 at diagnosis (RR = 2.50; 1.19-5.27). Risks were nearly 4-fold elevated among female relatives (RR = 3.97; 1.54-10.2) and among relatives of female probands (RR = 3.74; 1.58-8.83). MM cases had more cases of monoclonal gammopathy of undetermined significance (MGUS) among their relatives than controls, but the numbers were too small to be conclusive. There was generally no increase in risk of MM in probands whose relatives had hematologic malignancies other than MM. These findings do not support a strong association between personal/familial autoimmune diseases and MM. However, MM itself shows significant familial aggregation, implicating the etiologic importance of this type of hematological neoplasm and perhaps MGUS in germ line genes.

Coronary artery disease in patients with systemic lupus erythematosus

A 24-year-old woman with an 11-year history of systemic lupus erythematosus presented with exacerbation of chronic abdominal pain followed by substernal chest pain. She had a history of pericarditis secondary to systemic lupus erythematosus and of varicella-zoster reactivation secondary to immunosuppression. Long-term medications included prednisolone, hydroxychloroquine, aspirin, and mycophenolate mofetil. Physical examination, mesenteric angiography, CT of the abdomen, esophagogastroduodenoscopy, colonoscopy, pelvic ultrasound, laboratory testing, serologic testing, cardiac echocardiography, electrocardiography and coronary angiography. Acute myocardial infarction secondary to severe multivessel atherosclerotic coronary artery disease. Intra-aortic balloon pump followed by emergent four-vessel coronary artery bypass grafting. Aspirin, hydroxychloroquine, and mycophenolate mofetil were continued and a judicious tapering of prednisolone was initiated.

Hydroxychloroquine-induced Cardiotoxicity in a 39-year-old Woman with Systemic Lupus Erythematosus and Systolic Dysfunction

A 39-year-old woman with a history of systemic lupus erythematosus developed chest pain and conduction abnormalities. An echocardiographic examination revealed systolic dysfunction and ventricular thickening. Because of the unclear nature of her cardiac disease, right ventricular endomyocardial biopsy was performed. Light microscopy showed diffuse myocyte vacuolization without myocarditis, and transmission electron microscopy demonstrated sarcoplasmic myelinoid and curvilinear bodies, diagnostic of hydroxychloroquine toxicity. Among patients with autoimmune connective disorders, cardiac dysfunction may be a result of the disease or occasionally of its treatment. Although the nature of the cardiac disease (myocardial, valvular, or pericardial) can generally be evaluated echocardiographically, endomyocardial biopsy may be indicated to rule out diseases with a specific microscopic appearance, such as myocarditis or hydroxychloroquine cardiotoxicity.

Successful Outcome in Gastrointestinal Mucormycosis

We present a case of a 47 year-old Caucasian female with a history of systemic lupus erythematosus, treated chronically with prednisone, who was admitted to the medical intensive care unit with community acquired pneumonia and sepsis. She subsequently developed hematochezia and required multiple blood transfusions. Colonoscopy revealed active colitis in the ascending colon and biopsies were consistent with invasive Mucormycosis. The patient was tapered off her corticosteroids and treated with amphotericin B for four weeks with marked clinical improvement. There was no detected Mucormycosis on follow up colonoscopy with biopsies. Mucormycosis typically presents as an opportunistic infection in immunocompromised patients. In the majority of cases it follows a fulminate and fatal course. Predisposing factors include solid organ transplants, diabetes mellitus, malnutrition, corticosteroid dependency, hematologic malignancy, iron overload treated with deferoxamine, severe burns, metabolic acidosis, and cancer chemotherapy. Diagnosis is made histologically by demonstrating tissue and vessel invasion by the organism. Survival depends on early diagnosis and intervention. Gastrointestinal involvement is rare. A review of the literature revealed 62 cases of mucormycosis limited to the gastrointestinal tract. These cases were analyzed based on available data regarding age, sex, predisposing factors, clinical presentation, endoscopic appearance, pathology, treatment, and outcome. We found that use of immunosuppressive medications was the most common risk factor associated with the development of gastrointestinal mucormycosis. Those infected typically presented with abdominal pain, fever, and/or gastrointestinal bleeding. The most common site of infection was the stomach, followed by the colon. Over one-third of cases already had multiple sites of gastrointestinal involvement. On endoscopy, ulceration and ischemia were the most common mucosal patterns observed. The outcome was poor as the majority of patients with invasive disease succumbed to the infection. Those with the best chance of survival were treated with a combination of surgical and medical modalities.

Renal artery thrombosis in a pediatric case of systemic lupus erythematosus without antiphospholipid antibodies

A 13-year-old Japanese boy with an 11-month history of systemic lupus erythematosus (SLE) without antiphospholipid antibodies (APAs) suddenly developed severe hypertension, associated with fever and generalized seizures, and mild abdominal pain. Emergency abdominal computed tomography (CT) confirmed left renal artery thrombosis, and a renal scintiscan revealed reduced blood flow to the left kidney. Promptly instituted intravenous anticoagulant therapy was not effective for controlling the infarction-reduced renal arterial blood supply. Moreover, he developed stupor due to central nervous system (CNS) lupus a week after the occurrence of the hypertensive episode. Finally, a percutaneous transluminal angioplasty successfully relieved the occlusion of the left renal artery at its origin. The CNS lupus was also successfully treated with intravenous methylprednisolone pulse therapy combined with intrathecal methotrexate and dexamethasone. Although it is well known that SLE patients with APAs have a high incidence of thrombotic complications, to date, renal artery thrombosis has rarely been reported in young patients. This APA-negative SLE patient unusually manifested renal thrombosis associated with CNS lupus.

Anti-glutamic acid decarboxylase antibodies in a patient with systemic lupus erythematosus and fibromyalgia symptoms

We report the case of a 29-year old female nurse with a five-year history of systemic lupus erythematosus (SLE) involving multiple systems and on chronic prednisone therapy. This patient has a coexisting diagnosis of fibromyalgia fulfilling ACR criteria. A recent deterioration in her level of functioning in addition to a flare of her inflammatory disease led to further evaluation. During the course of investigation an anti-glutamic acid decarboxylase antibody was found to be present and significantly elevated. A therapeutic trial of baclofen did result in improvement of her subjective myalgias. We raise the possibility of an autoimmune contribution to myalgic symptoms in a portion of SLE patients.

Eumycotic mycetoma caused by Cladophialophora bantiana in a patient with systemic lupus erythematosus

We present the case of a 31-year-old patient who developed widespread mycetoma across her back and shoulders caused by Cladophialophora bantiana 16 years after injury to those sites from tornado debris. The patient also had a history of systemic lupus erythematosus treated with systemic steroids. Systemic antifungal treatment with fluconazole and itraconazole proved ineffective, and surgical debridement was required. Nine other culture-confirmed cases of cutaneous infection with this organism have been reported worldwide. This type of infection can occur in immunocompromised or immunocompetent patients, and may respond to systemic antifungals or surgery.

Chloroquine-induced cardiomyopathy-echocardiographic features

A 61-year-old woman with a 30-year history of systemic lupus erythematosus treated with chloroquine sulfate presented with complete heart block, congestive heart failure, and findings of restrictive cardiomyopathy on echocardiogram. Thickening of mitral, aortic, and tricuspid valves along with mild to moderate valve regurgitation was also present. Magnetic resonance imaging showed increased gadolinium uptake in the interventricular septum and the left ventricular lateral wall. Endomyocardial biopsy specimen showed marked myocardial cytoplasmic vacuolation and extensive myelin figures. Seven months after discontinuation of chloroquine, she showed significant clinical improvement and reversal of cardiomyopathy on echocardiography. This is the first case report describing a cardiomyopathy with prolonged use of chloroquine involving the conduction system, cardiac valves, and the myocardium with reversal on discontinuation of the drug.

Lupus Vasculopathy Combined with Renal Infarction: Unusual Manifestation of Lupus Nephritis

A 30-year-old woman with a 10-year history of systemic lupus erythematosus was admitted to our hospital because of the onset of hypertension and renal dysfunction. Renal arteriogram revealed multiple renal infarctions, and cut-off or tapering-stenosis in the interlobular arteries. Renal biopsy showed concentric intimal thickening with narrowed lumen in some arterioles and deposition of IgG/IgM/complement 3 in the wall of arteriole without any active lesions or immune complex deposition in glomeruli. The present case indicates that this type of renal vascular lesion in lupus nephritis, lupus vasculopathy, may cause renal infarction and the loss of renal function without active glomerular lesions.

Successful High Dose Glucocorticoid Treatment for Subacute Neuromyelitis Optica with Systemic Lupus Erythematosus

A 54-year-old Japanese woman with a 6-year history of systemic lupus erythematosus (SLE) was admitted to our hospital suffering from acute blindness in her right eye. Her condition recovered after steroid pulse therapy, however, 18 months later she suffered from nuchal pain for 2 weeks after which right hemiparesis with urinary incontinence developed. A spinal magnetic resonance imaging (MRI) revealed cord swelling from C2 to C7. She was diagnosed with neuromyelitis optica (NMO) and intravenous steroid administrations were immediately commenced. Her condition promptly improved. This case was unique because the steroid treatment was quite effective for this case of myelitis, which had passed the acute phase. We supposed that, because most of the lesion was not necrotic or demyelinated, but rather showed edematous change caused by vasculitis based on autoimmune pathogenesis, the symptoms progressed rather gradually and improved promptly in response to glucocorticoid treatment.

Hypopyon uveitis associated with systemic lupus erythematosus and antiphospholipid antibody syndrome

To report a case of hypopyon uveitis associated with systemic lupus erythematosus and antiphospholipid antibody syndrome. Interventional case report: a 49-year-old woman with a history of systemic lupus erythematosus and antiphospholipid antibody syndrome presented with sudden onset of pain, redness, photophobia, and decreased vision in the left eye. Examination revealed hypopyon uveitis and vaso-occlusive retinopathy. Following treatment with intravenous steroids and cyclophosphamide, the patient's vision improved from CF to 20/80 with no inflammation 6 months following initial presentation. In this case, systemic lupus and antiphospholipid syndrome were associated with hypopyon uveitis. Prompt treatment with systemic immunosuppressive therapy resulted in improvement in the hypopyon uveitis and vaso-occlusive retinopathy.

SPLENIC ABSCESS IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS

A 42 year old female presented to the ER with complaints of body ache, chills, chronic left sided abdominal pain of 4 months duration and 5/10 in intensity, radiating to the left side of chest. The patient had history of Systemic Lupus Erythematosus and was on prednisone since 1985. She also had a history of Asthma and Peptic ulcer disease, not on any medication. Patient had a 40 pack year history of smoking but denied alcohol or drug use. On physical exam there was only mild icterus and tenderness of the left upper quadrant. Temperature was 101.2 F. WBC count was 12,600/mm3 with 75% neutrophils. Hematocrit was 21.3% and MCV was 64.2 f L and Glucose was 130 mg %. Chest × ray and abdominal × ray revealed no acute pathology. Abdominal ultrasound revealed splenomegaly and a thickened gall bladder. CT scan of the abdomen revealed a splenic abscess with air bubbles on the anterior aspect of the spleen, extending into the pancreatic tail. There was also a small right pleural effusion. The patient was started on Ciprofloxacin and Metronidazole parenterally along with prednisone and plaquenil for her SLE. She was given 2 units of packed red cells. Blood cultures subsequently grew E. coli sensitive to all antibiotics. Splenectomy was done and revealed a large infarct with central liquefaction and abscess formation. There was also arterial and arteriolar thickening and concentric fibrosis consistent with SLE but no evidence of active vasculitis. The incidence of splenic abscess ranges from 0.14 to 0.7% in various autopsy series. The association of splenic abscess with SLE is very rare. Early diagnosis is key to successful treatment of splenic abscess and requires a high degree of clinical suspicion. CT scan is the most sensitive diagnostic tool. Ultrasound can also detect splenic abscess but has a lower sensitivity. Splenic abscess is associated with a very high mortality and the treatment of choice is splenectomy with adjunctive antibiotics. Percutaneous drainage may be helpful in some cases.

Acute transverse myelitis in SLE

A 21-year-old woman with a 5-year history of systemic lupus erythematosus (SLE) presented with a 4-hour paraparesis. Examination revealed absent knee and ankle reflexes, extensor plantar responses, and a T4 sensory level. MRI showed …

Intravenous immunoglobulin in treatment of cardiac tamponade in a patient with systemic lupus erythematosus

We describe a 23-year-old female patient with a history of systemic lupus erythematosus and pulmonary hypertension who developed a large pericardial effusion with cardiac tamponade. Invasive interventions such as pericardial window or pericardiectomy were ruled out because of the posterior localization of the effusion and high risk of general anesthesia in a patient with severe pulmonary hypertension. The patient received high-dose steroids intravenously with no response. A 5-day course of intravenous immunoglobulin resulted in gradual decrease of the pericardial effusion and resolution of cardiac tamponade within 2 weeks.

Rupture of the Gallbladder in a Patient with Acquired Factor VIII Inhibitors and Systemic Lupus Erythematosus

A 54-year-old woman with a 21-year history of systemic lupus erythematosus (SLE) was admitted to the Matsuyama Red Cross Hospital due to subcutaneous and gingival hemorrhaging. She was diagnosed with acquired factor VIII inhibitors based on a prolonged activated partial-thromboplastin time (APTT) and factor VIII inhibitors. Steroid pulse and factor VIII plasma concentrate were administered to her, not long after which she was transferred to Ehime University Hospital due to gallbladder hematoma. Although her APTT and factor VIII activity were improved after treatment with human factor VIII, she died of multiple organ failure. The autopsy demonstrated a ruptured gallbladder.

Hemisensory syndrome is associated with a low diagnostic yield and a nearly uniform benign prognosis

Objective: To describe the diagnostic yield and prognosis for patients with hemisensory syndrome. Background: The aetiology, utility of diagnostic procedures, and outcome of hemisensory syndrome in patients with exclusive hemibody...

Demonstration of necrotizing fasciitis on Ga-67 scans in a patient with systemic lupus erythematosus.

A 17-year-old girl with a history of systemic lupus erythematosus with lupus nephritis for 6 years was found to have progressive superficial tenderness over the medial aspect of the right thigh. In addition, darkening of the skin of the right thigh with an ecchymosis-like appearance developed in the following hours. Ga-67 scans performed to evaluate inflammation of the skin revealed increased uptake in the involved areas. Skin biopsy was performed, and the pathologic diagnosis was necrotizing fasciitis.

Central nervous system lupus and pregnancy: 11-year experience at a single center

Objective: To describe the pregnancy outcomes in women with central nervous system (CNS) manifestations of lupus.Methods: Between 1991 and 2002, the outcome of five pregnancies infour patients with CNS lupus were retrospectively reviewed. All patients had an established history of systemic lupus erythematosus (SLE), and either a history of CNS lupus or active CNS lupus. Pregnancyoutcomes assessed included term and preterm birth, intrauterine growth restriction, abnormal antepartum testing, perinatal mortality, pre-eclampsia and other maternal morbidities.Results: Evidenceof active CNS lupus symptoms developed in three of the five pregnancies. Two pregnancies were complicated by early onset pre-eclampsia, abnormal antepartum testing and extreme prematurity, with one subsequentneonatal death. The remaining three pregnancies had good neonatal outcomes, but were complicated by severe maternal post-pregnancy exacerbations, and the eventual death of one patient.Conclusions:CNS lupus in pregnancy represents an especially severe manifestation of SLE, and may involve great maternal and fetal risks.