Systemic Lupus Erythematosus Overlap Syndrome Research Articles

Diagnosis of tuberculosis with autoimmune hepatitis–systemic lupus erythematosus overlap syndrome: a case report

BackgroundThere is ample evidence indicating that immunosuppressive therapy or immune dysregulation in systemic lupus erythematosus increases the risk for tuberculosis. Interestingly, a few case reports suggest that tuberculosis could also be a risk factor for systemic lupus erythematosus and other autoimmune diseases.Case presentationWe report the case of a 32-year-old Indian patient who was co-diagnosed with tuberculosis, systemic lupus erythematosus, and autoimmune hepatitis without any history of prior immunosuppression. This stresses the complex relationship between tuberculosis and autoimmune diseases.ConclusionFurther research is warranted in this field to unfold the complex relationship between tuberculosis and systemic lupus erythematosus. It is essential to establish clear guidelines for the management of coexisting tuberculosis and systemic lupus erythematosus to promote individualized treatment.

Overlap syndrome in a 12-year-old girl with systemic lupus erythematosus and anti-oj antibody-positive polymyositis: a case report

BackgroundThe peculiar presentation of overlap syndrome in children makes precise diagnosis difficult. Children with overlap syndrome may or may not have specific antibodies. We present the case of a 12-year-old girl diagnosed with overlap syndrome of systemic lupus erythematosus (SLE) and juvenile polymyositis (JPM) who tested positive for anti-OJ antibodies.Case presentationWe describe the case of a 12-year-old girl diagnosed with SLE at the age of 7 and presented with fever with malar rash, periungual erythema, generalized weakness, and multiple joint pain at admission. The patient had persistent joint pain and weakness after intravenous methylprednisolone administration and complained of an inability to walk with a positive test for Gower's sign one week after admission, accompanied by elevated alanine aminotransferase (ALT) and creatine-phospho-kinase (CPK) levels. The results of nerve conduction velocity test were normal. Electromyography revealed abundant spontaneous activity and myopathic motor unit action potentials in the right deltoid, biceps, and iliopsoas, in addition to fibrillation and mild myopathic motor unit action potentials in the right rectus femoris muscle. Magnetic resonance imaging revealed diffusely increased signal intensities in the myofascial planes of the bilateral iliopsoas, gluteus, obturator, pectineus, and hamstring muscles. Anti-nuclear antibody, anti-RNP, and rheumatoid factor IgG tests were positive, and inflammatory myopathy autoantibodies revealed anti-OJ antibody positivity, which strongly indicated autoimmune myositis. High-resolution computed tomography of the lung revealed mild pericardial effusion without any evidence of interstitial lung disease. We initiated intravenous pulses of methylprednisolone treatment, followed by cyclosporine, mycophenolate mofetil, and oral steroids. Clinical improvement with a delayed, slowly reduced CPK level after the above treatment and she was discharged after the 18th day of hospitalization.ConclusionOverlap syndrome with inflammatory myositis can occur years later in pediatric SLE cases. We should be alert when patients with SLE develop a new presentation characterized by decreased SLE-specific autoantibody titers, positive anti-RNP antibodies, and elevated CPK. Treatment of the overlap syndrome of SLE and JPM is individualized, and the course differs between pediatric and adult patients.

A case of concurrent Kikuchi-Fujimoto Disease and Neuropsychiatric Lupus in a Patient with Rheumatoid Arthritis and Systemic Lupus Erythematosus Overlap Syndrome

Kikuchi-Fujimoto disease is a rare, self-limited, histiocytic necrotizing lymphadenitis characterized by cervical lymphadenopathy and fever. An association has been made between the presence of this disease and systemic lupus erythematosus. We describe a unique case of a 31 year old female with a previous diagnosis of rheumatoid arthritis and lupus overlap syndrome who presented with severe headache and subsequently developed altered level of consciousness and seizures. She underwent an extensive assessment which included investigations for an infectious cause, numerous imaging studies, a lymph node biopsy, a bone marrow biopsy, and a brain biopsy resulting in a final diagnosis of concurrent Kikuchi-Fujimoto disease and lupus-associated encephalitis. In these overlap patients, neurological involvement is usually mild. In contrast, our patient presented with severe neuropsychiatric involvement eventually requiring ICU admission. This case highlights the difficulty of diagnosing and managing neuropsychiatric lupus in these complex overlap patients. Early recognition is important to avoid unnecessary and potentially harmful interventions and treatments.

Jüvenil amiyopatik dermatomiyozit - Sistemik lupus eritematozus örtüşme sendromu

Jüvenil amiyopatik dermatomiyozit - Sistemik lupus eritematozus örtüşme sendromu

The Unusual Coexistence of Achalasia and Scleroderma

Scleroderma is an autoimmune disease characterized by excessive deposition of collagen and other connective tissue macromolecules in skin and multiple internal organs.It is called systemic Sclerosis(SS),when it involves internal organs.SS involves GI tract in 90% of the patients.Esophageal motility is the most frequently affected function in GI tract. It classically manifests as reduced LES pressure and loss of distal esophageal body peristalsis.In comparison Achalasia is a primary esophageal motor disorder characterized by insufficient relaxation of the LES and loss of esophageal peristalsis.While Sclerdoerma leads to low LESP, Achalasia causes insufficient LES relaxation.Coexistence of the two is rare.We report a case of a Scleroderma patient presenting with Achalasia. A 62 years old African American woman with history of Scleroderma and Systemic Lupus Erythematosus overlap syndrome was evaluated by gastroenterology for dysphagia. She had worsening dysphagia to both solids and liquids for three to four years.She underwent EGD which showed dilated, sigmoid shaped esophagus with retained liquid and solid food. LES was found to be hypertensive and resistance was felt while entering stomach.Barium esophagram showed dilated esophagus with narrowing at LES with bird beak appearance.So both studies were suggestive of Achalasia. Patient refused esophageal manometry at multiple occasions.Scleroderma occurs worldwide and affects all races but has higher general and age-specific rates in blacks with more severe disease and poorer outcomes than whites.GI tract involvement is very common and occurs early in the course of the disease. Esophagus is the most frequently involved internal organ causing symptoms of dysphagia, odynophagia,heartburn and regurgitation.Esophageal motility gets affected and is characterized by aperistalsis or low amplitude contractions, decreased or absent resting LES tone and lack of coordination between esophageal contractions and LES relaxations.Esophageal dysmotility in association with delayed gastric emptying results in impaired esophageal clearance of refluxed gastric contents and the development of reflux disease. In comparison Achalasia is characterized by absence of peristalsis in the distal two-thirds and elevated resting pressure in LES, typically above 45 mmhg along with failed LES relaxation with swallowing. LESP stays above 8 mmhg above gastric pressure.When the manometric characteristics of LES are compared, Achalasia is characterized by hypertensive LES and Scleroderma by hypotensive LES.Due to opposite changes in LESP, coexistence of Achalasia and scleroderma is extremely rare and so far only few cases have been reported. This case reports shows the rare coexistence of the two conditions making Achalasia one of differentials of dysphagia in Scleroderma patients.Figure 1Figure 2Figure 3

Late‐onset anaphylactic reactions following i.v. cyclophosphamide pulse in a patient with systemic sclerosis and systemic lupus erythematosus overlap syndrome

Late-onset anaphylactic reaction is a rare adverse effect of i.v. cyclophosphamide pulse (IVCY), which is caused by cyclophosphamide metabolites. We herein report a case of scleroderma and lupus overlap syndrome who developed anaphylactic reactions 3h to 4days after IVCY. Long time lapses between IVCY and appearance of symptoms are compatible with type I hypersensitivity to cyclophosphamide metabolites, appearing as late-onset anaphylactic reaction. Albeit unusual, this is an important anaphylactic reaction to be aware of following IVCY.

Systemic lupus erythematosus and dermatomyositis with symptomatic bilateral sacroiliitis: an unusual and interesting association

Systemic lupus erythematosus (SLE) is a multisystem autoimmune connective-tissue disease with a complex phenotype and varying disease course. Although SLE often causes a polyarticular synovitis, there are only a few case reports of abnormalities of the synovial lined sacroiliac joints. We report a case with an overlap syndrome of SLE and dermatomyositis who subsequently developed bilateral symptomatic sacroiliitis. We conclude that sacroiliitis, although rare, can occur in patients with active SLE as a manifestation of the disease per se.

A case of severe acute pancreatitis, in overlap syndrome of systemic sclerosis and systemic lupus erythematosus, successfully treated with plasmapheresis

Here, we describe a case of severe pancreatitis in overlap syndrome of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc) in an adult female patient. Treatment with plasmapheresis and high-dose prednisone successfully saved her life and led to remission of the pancreatitis. This is the first case report of severe acute pancreatitis in the setting of overlap syndrome of SLE and SSc. The advantages and disadvantages of the use of corticosteroids are discussed.

Prolonged Activated Partial Thromboplastin Time in Systemic Lupus Erythematosus Overlap Syndrome

A prolonged partial thromboplastin time in a patient with systemic lupus erythematosus usually is due to a lupus anticoagulant. Antiphospholipid antibodies may be associated with thrombosis. We describe a patient with an overlap syndrome between systemic lupus erythematosus and mixed connective tissue disease who presented with a prolonged partial thromboplastin time due to a high titer of antibodies to factor VIII (acquired hemophilia). The clinical course resulted in a fatal hemorrhage, illustrating the importance of prompt distinction between lupus anticoagulants and clotting factor inhibitors.

Changes in anti–U1 RNA antibody levels correlate with disease activity in patients with systemic lupus erythematosus overlap syndrome

To evaluate correlations between changes in anti-U1 RNA antibody levels and disease activity in 9 patients with systemic lupus erythematosus (SLE) overlap syndrome who were prospectively followed up for at least 3 years. Anti-U1 RNA antibody levels were measured quantitatively, using a nitrocellulose filter binding assay. Disease activity was measured with a validated SLE activity index. All 9 major disease exacerbations were associated with peaks in anti-U1 RNA antibody level. These results seem to indicate that measuring anti-U1 RNA antibody levels can be useful for monitoring disease activity.