History Of Systemic Lupus Erythematosus Research Articles

Mycobacterium mageritensePulmonary Disease in Patient with Compromised Immune System

<i>Mycobacterium mageritense</i>Pulmonary Disease in Patient with Compromised Immune System

Retiform Purpura and Digital Gangrene Secondary to Antiphospholipid Syndrome Successfully Treated With Sildenafil

A 16-year-old African American female adolescent with a 2-year history of systemic lupus erythematosus (featuring a malar erythema, photosensitivity, arthritis, and anemia in the setting of high-titer antinuclear, anti– double-strand DNA, anti-Smith, and anti-Ro antibodies) and Raynaud phenomenon presented with persistently painful and dusky toes since wearing a new pair of boots 2 weeks previously. She also noted an increased frequency of Raynaud episodes. Her dose of nifedipine was increased (from 30 mg/d to 60 mg/d) because of the latter, and she continued her baseline regimen of prednisone (5 mg/d), mycophenolate mofetil (1500 mg, twice daily), hydroxychloroquine (400 mg/d), and indomethacin (75 mg, twice daily). Despite this, she experienced worsening pain and progressive necrosis of her toes and was hospitalized for evaluation and treatment of impending digital gangrene. On physical examination, retiform purpura was evident on the left first and second toes. There was a hemorrhagic bulla on the distal left first toe, and the left second toenail was absent, replaced by a necrotic ulcer (Figure 1). Milder purpura on a background of dusky reticulated erythema was present on the left medial sole, left third through fourth toes, and right first through fourth toes. Dorsalis pedis pulses were diminished, more so on the left foot than the right foot. Findings from arterial Doppler studies showed a complete absence of blood flow in all the digits of the left foot and in the second through fourth digits of the right foot. A biopsy specimen from the peripheral portion of the purpuric area on the left first toe revealed thrombi in the lumens of small and medium-sized blood vessels throughout the mid and deep reticular dermis (Figure 2). There was no inflammatory infiltrate or fibrin deposition in the vessel walls. Partial necrosis of the eccrine glands and the overlying epidermis was apparent. Laboratory evaluation revealed elevated anticardiolipin IgM antibody levels, which had also been elevated in laboratory testing performed 10 months prior, and presence of the lupus anticoagulant. Anticardiolipin IgG and anti2 glycoprotein I antibodies were not identified. There were no abnormalities found in the remainder of an extensive evaluation for thrombophilia, which included protein C and S levels and activity, antithrombin III activity, homocysteine levels, and testing for factor V Leiden and prothrombin gene mutations. The clinical, histologic, and laboratory findings confirmed a diagnosis of thrombotic vasculopathy second-

Collagen Vascular Diseases and Enhanced Radiotherapy-induced Normal Tissue Effects — a Case Report and a Review of Published Studies

Collagen vascular diseases (CVD) are a group of chronic, autoimmune conditions that can affect multiple organ systems. The mainstay of treatment involves the use of immunosuppressants. CVDs and immunosuppression increase the risk of these patients developing malignancy. The mechanisms through which these patients develop CVDs show similarities to those for radiotherapy late effects, especially fibrosis (via transforming growth factor β). Radiotherapy may in fact cause an active state to develop from a quiescent state of CVD, or exacerbate a pre-existing CVD. CVDs are said to be associated with increased normal tissue toxicity after radiotherapy. Here we present a case report of a patient with a long history of systemic lupus erythematosus and oropharyngeal carcinoma, treated with synchronous chemoradiotherapy. We also review published studies and formulate some guidance on the radiotherapy management of these patients.

Systemic lupus erythematosus in a 3-month old male presenting as thrombocytopenia

We report a case of a 3-month old male infant, born to a mother with a known history of systemic lupus erythematosus (SLE). The infant initially presented with petechiae, anemia, and thrombocytopenia. His evaluation revealed antinuclear antibody (ANA) titer of 1 : 160, negative anti-SS-A/SS-B antibody, positive anti-Smith antibody, elevated anti-dsDNA titer, and a slightly low C4 level. His subsequent development of hematuria with nephrotic grade proteinuria fulfilled criteria for a diagnosis of SLE. His condition improved with corticosteroids, mycophenolate mofetil and low-dose aspirin. At 18 months of age, he is clinically well, off all immunosuppression with normal growth parameters and no detectable autoantibodies.

Successful transplantation of a donor kidney with diffuse proliferative lupus nephritis and crescents--a case report

Pre-existing diffuse proliferative glomerulonephritis (DPGN) in a potential deceased kidney donor has been considered a contraindication for transplantation. We report a case of a patient who underwent a successful deceased donor renal transplantation from a donor with history of systemic lupus erythematosus (SLE) whose baseline biopsy revealed DPGN. Although the histology was relatively benign in the procurement kidney biopsy done by frozen section, the final light microscopy available after transplantation showed diffuse proliferative lupus nephritis, WHO class IV, with 44% crescents. The post-transplant course was complicated by delayed allograft function requiring haemodialysis for the first week. A repeat biopsy performed after 4 months of transplant showed resolution of the proliferative lesions in the glomeruli with disappearance of the crescents. At 5.5 years of follow-up, the patient's creatinine has been stable at 2.0 mg/dL (176.8 μmol/L), but he has persistent proteinuria.

The Natural History of Untreated Asymptomatic Osteonecrosis of the Femoral Head

An asymptomatic hip with osteonecrosis is typically discovered as the contralateral hip of a patient with one symptomatic joint. Treatment of the asymptomatic hip is controversial. While some authors claim a benign natural history, others have reported a rate of femoral head collapse exceeding 50%. The purpose of this report was to systematically review the published literature regarding asymptomatic osteonecrosis of the femoral head to evaluate the overall prevalence of progression to symptomatic disease and/or femoral head collapse as well as to determine whether various radiographic and demographic factors influence progression of the disorder. A comprehensive literature search was performed to identify prognostic studies evaluating asymptomatic hip osteonecrosis. Demographic, radiographic, and outcome data were extracted from all relevant studies. The prevalence of progression to symptomatic disease and/or femoral head collapse was determined. Next, outcomes were stratified by lesion size, lesion location, radiographic stage, associated risk factors and/or disease, and the level of evidence of the study. Sixteen studies that included a total of 664 hips were available for an analysis of outcomes. Overall, 394 hips (59%) had progression to symptoms or collapse. Differences in outcomes based on lesion size, lesion location, and radiographic stage at the time of diagnosis were seen. Small, medially located lesions had the best prognosis, with a prevalence of collapse of <10%. Patients with sickle cell disease had the highest frequency of progression, and those with a history of systemic lupus erythematosus had the most benign course. Data extracted from previously published studies suggest that asymptomatic osteonecrosis has a high prevalence of progression to symptomatic disease and femoral head collapse. While small, medially located lesions have a low rate of progression, the natural history of asymptomatic medium-sized, and especially large, osteonecrotic lesions is progression in a substantial number of patients. For this reason, it may be beneficial to consider joint-preserving surgical treatment in asymptomatic patients with a medium-sized or large, and/or laterally located, lesion.

Atraumatic Avulsion of the Distal Iliopsoas Tendon: An Unusual Cause of Hip Pain

While uncommon, isolated avulsion fractures of the lesser trochanter occur in children and adolescents prior to the fusion of this apophysis as a result of athletic activities. In the elderly, isolated fractures of the lesser trochanter are rare but can occur as a result of trauma. They have been identified in patients with primary or secondary bone malignancies, which were previously considered pathognomonic for metastatic disease. In the absence of trauma, weakening of the bone due to systemic disorders such as osteoporosis or osteomalacia chronica renal failure may also be responsible. Diagnosis may be difficult with physical examination and radiographs alone. This case report details this rare fracture in 2 patients suffering from debilitating chronic disease. Patient 1 was a 30-year-old woman with an 18-year history of type 1 diabetes mellitus, a 6-year history of end-stage renal disease, hypertension, hypothyroidism, peripheral vascular disease, and a 3-year history of systemic lupus erythematosus with antiphospholipid syndrome treated with warfarin. Patient 2 was a 66-year-old woman with a history of type 2 diabetes mellitus, peripheral neuropathy, obesity, chronic obstructive pulmonary disease, gout, hypertension, and chronic neck and low back pain. Both were assessed with magnetic resonance imaging following physical examination, which revealed atraumatic avulsion of the distal iliopsoas tendon from the lesser trochanter. Following retraction of the iliopsoas tendon, the patients were treated with conservative therapy and anti-inflammatory medication. These 2 cases broaden the range of patients for whom spontaneous avulsion of the distal iliopsoas tendon should be considered in the differential diagnosis.

Bilateral Optic Neuritis in Pediatric Systemic Lupus Erythematosus Associated With Antiphospholipid Antibodies and Neuromyelitis Optica Immunoglobulin

The authors report a case of a 16-year-old girl with a history of systemic lupus erythematosus who developed bilateral acute optic neuritis. Systemic lupus erythematosus can present with a vast array of neurological and ophthalmic complications, with optic neuritis being a rare but devastating manifestation and the major cause of blindness in these patients. The patient presented with an acute unilateral visual deficit that progressed to bilateral visual loss with no light perception over the course of days. Treatment included high-dose steroids, cyclophosphamide, intravenous immunoglobulin, and eventually rituximab. Furthermore, the patient was also seropositive for both antiphospholipid and neuromyelitis optica antibodies, which can have implications on prognosis and treatment options.

Antiphospholipid Antibody Syndrome: Coexistence of Left Ventricular Apical Thrombus and Deep Vein Thrombosis Causing Pulmonary Thromboembolism in a Patient with Systemic Lupus Erythematosus

We report a 36-year-old woman with a 1-year history of systemic lupus erythematosus who was admitted with acute onset of dyspnea and chest pain. She presented with a classic medical history of antiphospholipid antibody syndrome, including spontaneous abortion, deep venous thrombosis, and clinical manifestations of lupus activation. The differential diagnosis was made after a detailed history and examinations with transthoracic/transesophageal echocardiography, deep venous ultrasonography, chest computed tomography, and coronary angiography. This case demonstrates a left ventricular apical thrombus in angiographically normal coronary arteries and also deep vein thrombosis causing acute pulmonary thromboembolism. Antiaggregant and anticoagulant therapies were initiated as a result of the presence of a left ventricular apical thrombus and deep venous thrombosis, which is predisposed to recurrent pulmonary or systemic embolization. Control echocardiography demonstrated resolution of apical thrombus and normalized left ventricular systolic function after aspirin, warfarin, and immunosuppressive therapy for 2 months.

Recurrent CMV retinitis in a non-HIV patient with drug-resistant CMV

We report a case of recurrent cytomegalovirus (CMV) retinitis in an HIV-negative patient with CD4+ T lymphocytopenia. Case report. A 41-year-old HIV-negative woman with a history of systemic lupus erythematosus, idiopathic thrombocytopenic purpura requiring splenectomy, and diabetes presented with primary CMV infection, high-grade viremia, CMV pneumonia followed by CMV retinitis (CMVR) and a CD4+ T lymphocyte (CD4) count of 12 cells/mm(3) after therapy with rituximab, prednisone, and methotrexate. Persistent CMV viremia led to genotypic analysis of the circulating virus, which revealed UL97 and UL54 mutations known to be associated with resistance to ganciclovir (GCV) and cidofovir. CMV clearance from the bloodstream followed systemic antiviral therapy and recovery of CD4 cell count. However, CMVR recurred multiple times despite GCV implants, systemic valganciclovir, intravitreal GCV injections, and persistent CD4 counts greater than 100 cells/mm(3). Recurrent episodes of CMVR responded to multiple high dose intravitreal GCV injections (5000-6000 micrograms) and recovery of CD4 cell counts to greater than 200 cells/mm(3). This case demonstrates that recurrent CMVR occurs in HIV-negative patients at CD4 cell counts thought to be protective in HIV patients, and suggests that an ineffective local immune response to retinal infection combined with CMV drug resistance may have been important factors leading to recurrent disease in this patient. Treatment producing high local concentrations of GCV may be effective therapy for CMV retinitis due to GCV-resistant virus.

Herpes vasculitis in systemic lupus erythematosus

A 29-year-old woman with a history of systemic lupus erythematosus (SLE) presented to our medical center with 9 days of worsening left wrist and hand cellulitis and ulceration unresponsive to broad-spectrum antibiotics including cefepime, linezolid, and ciprofloxacin (Panel A). Concurrent immune modulation therapy included pulse dose methylprednisolone. Prior to hospitalization she was on maintenance dose hydroxychloroquine for SLE. In addition to concern of infection, vasculitic and thrombotic etiologies were also entertained. Both arterial and venous imaging studies were unremarkable. Given her unresponsiveness to broad-spectrum antibiotics and immunemodulation therapy, a skin biopsy was obtained. Focal acute fibrinoid vasculitis was present in addition to acantholytic squamous epithelial cells showing viral inclusions consistent with acute herpes vasculitis (Panel B).

Simultaneous herpes simplex virus esophagitis and lupus enteritis in a patient with systemic lupus erythematosus

A 52-year-old woman with a 6-year history of systemic lupus erythematosus (SLE) developed acute abdominal pain, nausea, vomiting, and diarrhea accompanied by hypocomplementemia. Herpes simplex virus (HSV) esophagitis and lupus enteritis were diagnosed on the basis of the results of endoscopic and histological examinations and abdominal computed tomography (CT) findings. Treatment with acyclovir followed by high-dose intravenous steroids improved her symptoms. To our knowledge, this is the first case of simultaneous HSV esophagitis and lupus enteritis.

Hydroxycloroquine-induced Pill Esophagitis

Purpose: Case Report: A 27-year-old female patient with a past medical history of Systemic Lupus Erythematosus (SLE) and hypertension was receiving hemodialysis for end-stage renal disease. The patient was hospitalized for repair of arterio-venous fistula and was recovering from this surgical procedure when she complained of severe odynophagia. The patient was started on Hydroxychloroquine for pain in both hands one week earlier. An upper endoscopy was performed which revealed a mid-esophageal ulcer, otherwise normal esophageal mucosa. The ulcer was horse-shoe shaped, 2-2.5 cm in size and was located at 30cm from incisors. The ulcer was non-bleeding with a clean base. The pathology report showed fragments of granulating tissue consistent with an ulcer bed with severe acute inflammation. No viral inclusions were visualized. PAS stain failed to show fungal hyphae or yeast. The medication was stopped, patient was started on pantoprazole therapy and was instructed on proper oral medication administration. The patient recovered well and was discharged from our care without any complications. In conclusion, this report notes the first case of pill induced esophagitis caused by hydroxychloroquine and it should be added to the list of medication causing pill induced esophagitis. Pill esophagitis is a preventable cause of morbidity. Patients, family members, care takers, nurses and primary care physicians should be aware that the pills are to be taken in the sitting position with at least eight oz. of water. Physicians should be vigilant of this diagnosis and should withdraw or, if possible, avoid use of offending pills, so that further complications i.e. gastrointestinal bleeding can be prevented.

Infestation by Norwegian scabies

A 51-year-old woman with an 8-year history of systemic lupus erythematosus was hospitalized with fever, refractory thrombocytopenia and generalized rash. The patient had previously undergone splenectomy. She had been taking azathioprine and prednisolone daily for more than 1 year. The rash had begun

Mast cell tryptase in a case of anaphylaxis due to repeat antibiotic exposure

Mast cell tryptase can be an indicator of type I hypersensitivity reaction and thus may serve as a surrogate marker of anaphylaxis. A 34-year-old white male patient presented with a history of systemic lupus erythematosus. Shortly after administration of cefazolin for dialysis, he developed pruritis and shortness of breath. He expired an hour later. Autopsy excluded anatomic causes of death. There was an elevated postmortem mast cell tryptase level, 29.2 ng/mL. For mast cell tryptase level to be useful, the patient must survive long enough after exposure to an allergen for mast cells to release this enzyme. A credible allergen must be identified. In this case such, mast cell tryptase could establish anaphylaxis as the cause of death. The case suggests that in a patient with autoimmune disease, it may be prudent to test for immune reaction to a drug before administering it a second time via pinprick or other method.

Methotrexate-induced Hodgkin disease in a patient with systemic lupus erythematosus.

Methotrexate sodium use in the management of various immunologic disorders has increased--as have the number of reported adverse effects associated with this therapy. While methotrexate has helped combat various autoimmune and cancerous disorders, the paradoxical risk of causing an often fatal malignancy may still occur as a result of the drug's effect on suppressing immune function. We present a case of methotrexate-induced Hodgkin disease in a 48-year-old man with a history of systemic lupus erythematosus (SLE). Discontinuation of methotrexate facilitated Hodgkin disease reversal. In addition, we review other lymphoproliferative hematologic malignancies caused by methotrexate.

Cardiovascular magnetic resonance imaging of abortive ventricular septal defect

A 32-year-old woman with a history of systemic lupus erythematosus and previous pericarditis presented with chest pain. A transthoracic echocardiogram demonstrated normal biventricular function. An abortive ventricular septal defect (VSD) without evidence for shunting was found. The pericardium was visualized incompletely, as is typical in echocardiography. A diagnosis of pericarditis could not be established or ruled out. A contrast-enhanced cardiovascular magnetic resonance (CMR) imaging study was performed to assess possible pericarditis and VSD. CMR imaging confirmed normal left ventricular dimensions and function, as well as significant regional thinning in the midseptal segment (Figure 1). An abortive muscular VSD without shunting was visualized. Contrast-enhanced images showed no CMR criteria for acute inflammation (Figure 2). Figure 1 Figure 2 The present case illustrates the the use of CMR imaging to easily perform an integrated cardiac study to assess cardiac function and shunting, and perform tissue characterization to guide future management.

Rapidly fatal HTLV-1-associated T-cell leukemia/lymphoma in a patient with SLE

A 57-year-old Afro-Jamaican woman with an 8-year history of systemic lupus erythematosus, including lupus nephritis, was admitted to hospital with intractable back pain accompanied by fever and severe malaise. At the time of presentation she was receiving immunosuppressive treatment with glucocorticoids and azathioprine. She also had gout, hypertension and type II diabetes. Physical and neurological examination and laboratory analyses, including biochemical, hematological and electrophoresis tests, X-ray of the lumbar spine, pelvis and chest, mammography, MRI of the lumbar spine, thoracic and abdominal CT, and biopsy of a peripheral lymph node and bone marrow with immunohistochemistry and serology for human T-cell lymphotrophic virus (HTLV) 1 and 2. HTLV-1-associated acute adult T-cell leukemia/lymphoma with bone marrow infiltration and hypercalcemia. Reaching the correct diagnosis was difficult and only possible through close collaboration with the pathologist and with consideration of the patient's ethnic and geographical background. Chemotherapy with high-dose prednisone and adjusted doses of cyclophosphamide and doxorubicin. The patient developed tumor lysis syndrome and died 3 weeks after the diagnosis was made.

Disnea en una paciente con lupus eritematoso sistémico

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.

Restrictive Cardiomyopathy Secondary to Hydroxychloroquine Therapy

A 64-year-old woman with a history of systemic lupus erythematosus, treated for more than 10 years with prednisone and hydroxychloroquine, presented with severe progressive dyspnea on exertion. She had no other significant medical history. Examination revealed an S3 gallop. Transthoracic echocardiogram (TTE) demonstrated a 46% ejection fraction (EF) with generalized left ventricular hypokinesis, grade 3/4 …