Initial Manifestation Of Systemic Lupus Erythematosus Research Articles

A Case Report of Systemic Lupus Erythematosus Presenting as Isolated Mononeuropathy Multiplex.

Mononeuropathy multiplex is a pattern of progressive sensory and motor deficits in the distribution of two or more peripheral nerves. The differential for mononeuropathy multiplex includes vasculitis, autoimmune disorders, infectious diseases, sarcoidosis, amyloidosis, cryoglobulinemia, and paraneoplastic disease. We present a case of a 42-year-old woman with hypothyroidism who presented with 1week of ascending numbness and weakness, persistent fevers, and 3months of constant burning pain in both feet. Her neurologic exam was notable for asymmetric weakness, loss of sensation, and diminished reflexes of her lower extremities. Other than mild fever and tachycardia, there were no systemic exam findings. Her laboratory workup was significant for elevated inflammatory markers, positive ANA, anti-ribonucleoprotein, smooth muscle antibody, and lupus anticoagulant, high anti-dsDNA titers, and low complement levels. An MRI of her lumbar spine was negative for lumbosacral nerve root pathology. Cerebrospinal fluid studies demonstrated normal cell counts, glucose, and protein and no evidence of infection. An electromyography (EMG) and nerve conduction studies (NCS) demonstrated asymmetric involvement of multiple individual peripheral nerves. Given the patient's symptoms, asymmetric exam findings, laboratory results, and EMG/NCS findings, she was diagnosed with mononeuropathy multiplex secondary to systemic lupus erythematosus (SLE) and started on steroid and immunosuppressant therapy with symptomatic improvement. Overall, this case illustrates the unique presentation of mononeuropathy multiplex as the initial manifestation of SLE. The identification of this specific type of neuropathy may play a crucial role in the prompt diagnosis of SLE and timely initiation of treatment, preventing further complications of the disease.

Eighty-six cases of clinical characteristics and outcomes of systemic lupus erythematosus-associated macrophage activation syndrome: A meta-analysis study.

To improve our understanding of systemic lupus erythematosus (SLE)-macrophage activation syndrome (MAS). A systematic review was performed, to retrieve all those papers on patients with SLE-MAS, in individual or aggregated form. The data in each of these medical records were extracted and analyzed to identify the characteristics of SLE-MAS. A total of 86 SLE-MAS patients were included (25 males and 61 females. The mean (±standard error of the mean) age was 31.21 ± 1.694 years. MAS occurred as the initial presentation of SLE in 47 people (54.65%) and during the course of SLE in 39 (45.35%). A coinfection was reported in 23 (26.74%) patients. The mean Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) score was 16.54 ± 0.9462. Overall, 10 patients (11.63%) died. The SLEDAI-2K score was higher in patients with MAS as an initial manifestation of SLE than in those where MAS occurred during the course of SLE. The proportion of patients receiving steroid pulse therapy was lower in patients with coinfections. The deceased group demonstrated lower platelet and ferritin levels. Multiple regression analysis revealed that age and thrombocytopenia were independent factors associated with poor prognosis. In receiver operating characteristic analysis, a platelet count cutoff value of ≤47 × 109/L was a predictor of poor outcome. SLE-MAS patients demonstrated high lupus activity, and lupus activity was especially higher in patients with MAS as an initial manifestation. Lupus activity was the predominant trigger of lupus MAS. Thrombocytopenia was an independent factor for poor prognosis.

#2139 Non-lupus full house nephropathy: a debatable entity

Abstract Background and Aims The presence of a full house (FH) pattern at immunofluorescence on kidney biopsy in a patient without clinical and laboratory features of systemic lupus erythematosus (SLE) has led to the descriptive term non-lupus full house nephropathy (NLFHN). This systematic review and meta-analysis focus on NLFHN nomenclature, clinical findings and outcomes. Method In a reiterative process, all identified terms for NLFHN and other MeSH terms were searched in PubMed. Out of 344 results, 57 records published between 1982 and 2022 were included in the analysis. Clinical data of single patients from different reports were collected and analysed. Results Out of the 57 records, 61% were case reports. NLFHN was addressed with 17 different names; the same name used by different authors could refer to different entities. We identified 148 patients: 75 (51%) were males; median age 35 (23-58) years. Serum creatinine and proteinuria at onset were 1.4 (0.8-2.5)mg/dL and 5.7 (2.7-8.8)g/day. Overall, about half of patients achieved complete response. A causative agent was identified in 51 (44%) patients, predominantly infections(41%). Secondary NLFHN was mostly non-relapsing with worse kidney function at onset compared to idiopathic NLFHN (P = 0.001). Among the 57 (50%) patients with idiopathic NLFHN, complete response was comparable between patients treated with immunosuppression and supportive therapy; however, proteinuria and creatinine at onset were higher in patients treated with immunosuppression (P = 0.089 and P = 0.066). The remaining 7 (6%) patients developed SLE after a median follow-up of 5.0 (1.9-9.0) years. Conclusion The place of NLFHN among glomerular diseases is still uncertain, due to the lack of high-quality evidence and consensus on nomenclature. NLFHN can be evaluated as initial manifestation of SLE, isolated manifestation of SLE or a new disease unrelated to SLE. Data support the classification of SLE and NLFHN as distinct clinical entities, with comparable outcome. However, RCTs are needed to drive strong conclusions on treatments and outcomes.

Abstract 14188: Myopericarditis as the Initial Presentation of Systemic Lupus Erythematosus

Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disorder that can affect any organ. However, myocardial involvement is uncommon and rarely presents as the initial manifestation of SLE. Case: We present a case involving a 31-year-old male with no past medical history who presented to the emergency department with chest pain, fatigue, and intermittent swelling in his hands and feet. Initial workup was remarkable for high sensitivity troponin of 11, 821 and respiratory viral panel positive for parainfluenza. Results: Evaluation with left heart catheterization showed no obstructive coronary artery disease thus a cardiac MRI was obtained for further investigation. It showed late gadolinium enhancement involving the lateral apical wall extending to the apex as well as the lateral wall at the base (figure 1). The initial diagnosis was suspected to be viral-induced myopericarditis. However, he started developing accelerated acute kidney injury out of proportion to the current clinical presentation leading us to pursue rheumatological workup as it could explain the cardiac and renal pathologies. He was found to have ANA >= 1:2560, positive dsDNA, and positive anti-smith antibody. Kidney biopsy revealed diffuse spike formation on the glomerular basement membrane and focal endocapillary proliferation and segmental sclerosis consistent with lupus nephritis. He was started on prednisone and mycophenolate mofetil resulting in symptom and disease control. Conclusion: Myocardial involvement of SLE is uncommon and can be a life-threatening condition in the acute phase; however, the disease has an overall good prognosis with early treatment.

Systemic lupus erythematosus presenting as lupus erythematosus tumidus and lupus nephritis: a case report

BackgroundLupus nephritis and lupus erythematosus tumidus (LET) are uncommon manifestations of systemic lupus erythematosus (SLE), and their coexistence as the initial presentation of SLE is exceedingly rare. Here, we report such a case, emphasizing the diagnostic challenges and therapeutic implications of this unusual association.Case reportA 38-year-old North African woman presented in Nephrology department with a history of lower extremity edema, fatigue, and weight loss of 3 kg in 4 weeks. Physical examination revealed LET lesions on the chest and the Neck. Laboratory investigations showed lymphopenia, low C3 and C4 complement levels, positive antinuclear antibodies, anti-dsDNA antibodies, and anti-SSA/Ro antibodies. Renal function tests showed normal serum creatinine and nephrotic proteinuria. Renal biopsy revealed Class V lupus nephritis. Skin biopsy confirmed the diagnosis of LET, with the presence of lymphohistiocytic infiltrates and dermal mucin. The patient was diagnosed with SLE based on the 2019 EULAR/ACR criteria and treated with prednisone (1 mg/kg/day) and hydroxychloroquine. She showed significant improvement in her cutaneous and renal symptoms at 6 and 12 months follow-up.ConclusionThe rarity of the coexistence of LET and lupus nephritis as the initial manifestation of SLE, especially in the North African population, underscores the need for further research to elucidate the immunopathogenic mechanisms and prognostic factors associated with this association.

Lupus nephritis: from diagnosis to treatment

Renal involvement in systemic lupus erythematosus (SLE), so-called lupus nephritis (LN), is one of the most frequent organ manifestations with an incidence of approximately 40-60%. It is not uncommon for renal involvement to be the initial manifestation of SLE or to occur in the first 5-10years after diagnosis of SLE. Urinalysis is useful in screening for the presence of LN, demonstrating proteinuria or active sediment with acanthocytes. Histologic confirmation of LN, and thus the LN class present, is currently the gold standard for confirming the diagnosis. In addition, knowledge of the LN class is arelevant component of adequate treatment planning in SLE patients with LN. In particular, early diagnosis and rapid response to therapy are of prognostic importance for the preservation of renal function as well as morbidity and mortality of the mostly young patients at the time of initial diagnosis. Thus, the focus of therapy is to achieve complete remission, as well as to avoid active disease phases. Due to acomplex pathogenesis and at the same time avery heterogeneous clinical presentation, with six different histological classes of LN, there are different therapeutic targets. This in turn results in asignificant expansion of the study landscape in the field of LN with an increasing understanding of the signaling pathways and influencing factors, and fortunately in agrowing armamentarium of available targeted therapy options. Simultaneously, new insights into drug therapy to inhibit progression of chronic renal disease are opening up supportive therapy options that can further improve preservation of renal function.

A Case of Pseudo-Pseudo Meigs Syndrome as the Initial Presentation of Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a multigenic disease leading to chronic inflammatory states within organ systems. When associated with ascites, pleural effusions, and elevated CA-125 levels, it is known as pseudo-pseudo Meigs syndrome. It is a clinical diagnosis without findings of benign or malignant ovarian tumors in patients with SLE. We present a patient case of a 42-year-old woman who presented with episodic and progressively worsening ascites, bilateral pleural effusions, and elevated CA-125 levels without any evidence of benign or malignant tumors as the initial manifestation of SLE.

Kikuchi-Fujimoto disease as the initial manifestation of systemic lupus erythematosus complicated with macrophage activation syndrome: two case reports and a review of literature

BackgroundKikuchi-Fujimoto disease (KFD) is a self-limiting and benign disease characterized by cervical lymphadenopathy and fever. Although KFD should be made differentially diagnosed from infectious, autoimmune, and malignant diseases, it sometimes occurs in patients with systemic lupus erythematosus (SLE) and can be complicated with macrophage activation syndrome (MAS). However, it is rare that KFD is the initial manifestation of SLE and to be complicated with MAS.Case presentationA 9.6-year-old girl presented with high-grade fever, double-side cervical lymphadenopathy with mild pain of one week, leukopenia, alopecia, and rash on the cheek. During hospitalization, laboratory investigations showed positive antinuclear antibody (ANA), low complement 3 (C3), and low complement 4 (C4). Imaging investigations showed pleural and pericardial effusion. A 10.3-year-old girl presented with intermittent high-grade fever, double-sided cervical lymphadenopathy with obvious pain of 1-month duration, and discoid lesion on the cheek. During hospitalization, laboratory investigations showed positive ANA, leukopenia, thrombocytopenia, anemia with positive Coombs’ test, low C3, and positive Smith antibodies. Both cases were diagnosed with KFD using lymph node biopsy, simultaneously fulfilling the diagnostic criteria of SLE. Subsequently, the two girls became complicated with MAS, followed by interstitial lung disease and neuropsychiatric lupus, respectively. Both patients benefited from high-dose methylprednisolone pulse therapy combined with intravenous cyclophosphamide.ConclusionsMore attention should be paid to differential diagnosis, especially SLE, in children diagnosed with KFD. In addition, children with SLE who presented with KFD as the initial manifestation seem to have a higher risk of developing MAS and experiencing organ involvement.

Case Report: Recurrent retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the revealing manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

Neuropsychiatric Systemic Lupus Erythematosus in Older Adults: Diagnosis and Management.

Systemic lupus erythematosus (SLE) is a multisystem chronic autoimmune disease with variable clinical manifestations. Neuropsychiatric systemic lupus erythematosus (NPSLE) includes the neurologic syndromes of the central, peripheral and autonomic nervous system and the psychiatric syndromes observed in patients with SLE. Neuropsychiatric systemic lupus erythematosus events may present as an initial manifestation of SLE or may be diagnosed later in the course of the disease. Older adults with NPLSE include those who are ageing with known SLE and those with late-onset SLE. The diagnosis of NPSLE across the lifespan continues to be hampered by the lack of sensitive and specific laboratory and imaging biomarkers. In this review, we discuss the particular complexity of NPSLE diagnosis and management in older adults. We first discuss the epidemiology of late-onset NPSLE, then review principles of diagnosis of NPSLE, highlighting issues that are pertinent to older adults and that make diagnosis and attribution more challenging, such as atypical disease presentation, higher medical comorbidity, and differences in neuroimaging and autoantibody investigations. We also discuss clinical issues that are of particular relevance to older adults that have a high degree of overlap with SLE, including drug-induced lupus, cerebrovascular disease and neurocognitive disorders. Finally, we review the management of NPSLE, mainly moderate to high- dose glucocorticoids and immunosuppressants, again highlighting considerations for older adults, such as increased medication (especially glucocorticoids) adverse effects, ageing-related pharmacokinetic changes that can affect SLE medication management, medication dosing and attention to medical comorbidities affecting brain health.

Case Report: Recurrent retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the revealing manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

Management of Systemic Lupus Erythematosus complicated with Refractory Immune Thrombocytopenic Purpura and Pulmonary Artery Hypertension

The coexistence of immune thrombocytopenia (ITP) and pulmonary artery hypertension (PAH) is rarely observed as the initial manifestation of systemic lupus erythematosus (SLE), often leading to delayed diagnosis and poor outcome. We presented the case of an 18-year-old female of Asian origin with severe ITP and PAH as the initial manifestation of SLE. The patient was successfully treated with a combination of methylprednisolone, mycophenolate mofetil, sildenafil, and vinca alkaloid (vincristine). This case provided the opportunity to increase awareness of an uncommon association between SLE complicated with ITP and PAH and suggest a positive impact of early diagnosis and appropriate treatment on the patient’s outcome. The use of vincristine was considered as per the guideline on refractory ITP before referral for splenectomy.

Lupus eritematoso sistémico juvenil y compromiso hematológico

Introduction: Systemic Lupus Erythematosus (SLE) is a chronic inflammatory autoimmune disease of unknown cause without specificity for a particular organ. Juvenile SLE (jSLE) affects a greater number of organs and systems and presents a more aggressive clinical course than in adults. Hematological alterations could be presented as the only manifestation of SLE, so a detailed and systemic approach should be performed in these patients, once other causes such as infections and neoplasms have been excluded. Objectives: To determine the hematological compromise in patients diagnosed with jSLE in the Pediatric Service of the Faculty of Medical Sciences of the National University of Asuncion; over a period of 5 years. Materials and Methods: Retrospective, observational, descriptive, cross-sectional study. Non-probability sampling of consecutive jSLE cases diagnosed or under follow-up from January 2012 to May 2017. Results: Of the 73 patients, 57 women (78%) and 16 (22%) men were found, with an F:M ratio of 3.5:1. Average age at diagnosis was 11.9 years (SD: 3.65, range between 2 and 17 years). Regarding origin, 39 patients (53%) came from the Capital and Central Department and 34 cases (46%) from rural areas. The mean time between onset of symptoms and clinical diagnosis was 2 months (SD 1.27 months). Symptoms at first consultation were: arthralgias in 38 patients (52%), fever in 14 cases (19.2%), cutaneous-mucosal manifestations in 10 patients (13.7%), neurological manifestations in 5 cases (6.8%), edema in 4 patients (5.5%) and constitutional manifestations in 2 patients (2.7%). Hematologic abnormalities were found in 62 patients (85%), with a single series affected in 25 cases (40.3%), bicytopenia in 33 patients (53.2%), and pancytopenia in 4 patients (6.5%). The most frequent alterations were: anemia in 58 patients (79.4%), lymphopenia in 27 cases (36.9%), thrombocytopenia in 21 patients (28.4%) and leukopenia in 17 cases (23.2%). 60% of patients had more than one affected series. On the subject of anemia (n=58), 16 patients (27.6%) had a direct positive Coombs test and 42 patients (72.4%) had a direct negative Coombs test. Concerning hematimetric indices, 40 patients (69%) presented normochromic normocytic anemia and 18 patients (31%) hypochromic microcytic anemia. Conclusion: Hematological alterations were a frequent finding as a initial manifestation of SLE. Most cases had involvement of two series, with non-hemolytic anemia being the predominant finding. Laboratory tests are of great value when evaluating a patient with suspected autoimmune disease, since the results can confirm the diagnosis, estimate the severity of the disease, assess the prognosis and monitor SLE activity.

Orbital compartment syndrome as the first manifestation of SLE

The occurrence of orbital compartment syndrome is a rare and unusual complication of systemic lupus erythematosus (SLE). Patients will present with symptoms of ocular pain and diplopia and visible signs of proptosis. The condition is considered an ophthalmological emergency because the myositis involving the ocular muscles can cause irrevocable damage to the retina and optic disk, potentially leading to blindness. We report a case of a young African American male who developed orbital myositis with compartment syndrome as his initial manifestation of SLE. The patient underwent an emergent orbital decompression, followed by prompt initiation of immunosuppressant medications. To our knowledge, this is the first report of a case of myositis secondary to SLE complicated by compartment syndrome of the orbit.

Hemorragia alveolar como forma de debut de lupus eritematoso sistémico

Introducción: La hemorragia alveolar difusa (HAD) sigue siendo una complicación pulmonar poco frecuente del lupus eritematoso sistémico (LES), pero potencialmente mortal me parece que así quedaría mejor. Objetivo: presentar las características clínicas, tratamiento y la evolución de dos pacientes con LES con hemorragia alveolar y nefritis lúpica asociada como forma de debut del LES material y método: serie de dos casos de pacientes con diagnóstico de LES con hemorragia alveolar difusa como forma de debut del LES, internadas en el Departamento de Medicina Interna del Hospital Nacional de Itaugua en el periodo 2007-2018. Resultados: de 500 pacientes con LES en seguimiento, 2 mujeres presentaron hemorragia alveolar como forma de debut del LES (0,4%), edad media 22 años ± 4,2., tiempo de enfermedad media de 60 días ± 42,4. Ambas pacientes presentaron disnea, fiebre, hemoptisis, disminución de la Hb, infiltrado pulmonar bilateral en la Radiografía de tórax, nefritis, insuficiencia respiratoria aguda, asistencia respiratoria mecánica (ARM). Solo una paciente se realizó biopsia renal, NL clase 3/C. Así mismo las dos pacientes recibieron pulsos de metilprednisolona 1g/d/3 días, seguidas de ciclofosfamida, con buena respuesta al tratamiento en una paciente y un óbito en la otra paciente. Comentario: Ambas debutaron con HAD, presentaron nefropatía asociada y necesidad de asistencia respiratoria mecánica. El óbito se presentó en la paciente con nefropatía e insuficiencia renal aguda.

Pulmonary Arterial Hypertension and Fulminant Myocarditis as Initial Manifestation of Systemic Lupus Erythematosus

Severe pulmonary arterial hypertension (PAH) is rarely observed as initial manifestation of systemic lupus erythematosus (SLE). Lupus myocarditis may rarely be initial presentation as a fatal complication of SLE, which mainly involved with the left ventricle. Here, we present a case of an incident PAH woman who had a delivery history 4 months previously. During hospitalization, she suffered from extensive myocardial damage, refractory ventricular tachycardia, and cardiogenic shock. Ultimately, she was diagnosed with SLE, fulminant lupus myocarditis, and PAH associated with connective tissue disease. After a large dose of methylprednisolone and PAH target therapy treatment, she recovered successfully and got a good result. To the best of our knowledge, this is the first case report of PAH and fulminant lupus myocarditis as initial manifestation of SLE in a previously healthy postpartum woman.

Acute acalculous cholecystitis in patients with systemic lupus erythematosus: A unique form of disease flare.

Objective We aimed to investigate the clinical features of acute acalculous cholecystitis (AAC) in patients with systemic lupus erythematosus (SLE). Methods SLE patients with AAC hospitalized in the Peking Union Medical College Hospital (PUMCH) from January 2001 to September 2015 were retrospectively analyzed. Their medical records were systematically reviewed. The diagnosis of AAC was based on clinical manifestations and confirmed by radiologic findings including a distended gallbladder with thickened wall, pericholecystic fluid and absence of gallstones. Results Among the 8411 hospitalized SLE patients in PUMCH, 13 (0.15%) were identified to have SLE-AAC. Eleven (84.6%) of them were female, with a mean age of 30.1 ± 8.6 years. AAC was the initial manifestation of SLE in four (30.8%) cases. Eleven (84.6%) patients complained of fever and abdominal pain, four (30.8%) had positive Murphy's sign and six (46.2%) had elevated liver enzymes. The median SLE Disease Activity Index was 8.0 (range 0-20.0) at the time of AAC. Other affected organs in SLE-AAC included kidney (11, 84.6%) and hematologic system (11, 84.6%), followed by mucocutaneous (seven, 53.8%), musculoskeletal (seven, 53.8%) and neuropsychiatric (two, 15.4%) systems. All patients received treatment of glucocorticoids and immunosuppressants but none underwent surgical intervention. During a median follow-up of 28 months (range, 2-320 months), 12 cases (92.4%) responded to treatment with no relapse and one patient (7.6%) died of septic shock. Conclusion Our study suggests that AAC is a relatively uncommon and underestimated gastrointestinal involvement of SLE that is often associated with active disease. For patients with AAC in SLE, treatment with aggressive glucocorticoids could result in a good prognosis.

Heart Failure - A Rare Initial Manifestation of Systemic Lupus Erythematosus

1.1 Context Cardiac involvement is a leading cause of death in systemic lupus erythematosus (SLE). Heart failure (HF) is a potentially life threatening complication of lupus myocarditis which is rarely described as an initial manifestation of SLE. We are presenting a case of HF as initial manifestation of SLE. 1.2 Case description 49 year old male with no past medical history presented with dyspnea and generalized weakness of 6 weeks duration. Physical exam was remarkable for tachycardia, S3 gallop, bibasilar crackles, and 2+ pitting edema. Echocardiogram showed ejection fraction of 35% without regional wall motion abnormalities or pericardial effusion. Cardiac catheterization showed normal coronaries. Serological tests indicated positive ANA, Anti-smooth muscle, SCL-70 and SSA antibodies. Patient was given methyl prednisone 1 g/day for 3 days followed by tapering dose of oral prednisone in addition to intravenous furosemide. He was discharged on prednisone 40 mg, hydroxychloroquine 200mg in addition to B-Blockers and ACE inhibitors. On follow up evaluation after 4 weeks there was complete resolution of his symptoms with improvement in ejection fraction to 45%. 1.3 Conclusion HF due to myocarditis can be a rare initial presenting manifestation of SLE. Identifying the cause is critical as it dictates therapy. Prompt recognition and a combination of supportive treatment and immunosuppression usually results in a favorable outcome.

Gastrointestinal manifestations in systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is an autoimmune disorder characterized by multisystem involvement, including the gastrointestinal (GI) tract. There is a significant variation in the clinical presentation and severity of GI disorders. When GI symptoms present as the initial manifestation of SLE, there is likely to be a delay in the diagnosis. The cause of these GI manifestations in SLE may be the disease, or the side effects of medications, or infections. In this study we investigated the GI manifestations in a group of SLE patients. Our study was conducted on 40 SLE patients and 30 healthy controls to assess the prevalence of GI symptoms in SLE patients. The prevalence of gastrointestinal manifestations in our study was 42.5%. GI manifestations in our SLE patients were: acute abdominal pain (due to pleurisy and peritonitis), 6%; diffuse abdominal pain, 23.5%; epigastric pain, 29%; epigastric pain with vomiting, 23.5%; epigastric pain with chronic constipation, 6%; chronic constipation, 6%; and diffuse abdominal pain with bleeding per rectum, 6%. In our study, we found a higher incidence of Giardia infestation in SLE patients than in healthy controls, and 10% of these patients were asymptomatic. There was more Giardia infestation in patients with GI symptoms as compared with patients with no GI symptoms, with a P value of 0.009. In our study SLE patients with GI symptoms had a peak systolic velocity (cm/s) with a mean of 108.4 ± 32.1 standard deviation (SD) in the celiac Doppler study. Patients without GI symptoms had a peak systolic velocity with a mean of 111.9 ± 37.7 SD, meaning that our patients mostly had no evidence of celiac trunk stenosis, but there was significant difference between SLE patients without GI symptoms and controls, as the mean was higher in SLE patients than in the controls. Also, the celiac end diastolic velocity was higher in both groups of SLE patients with GI symptoms and those without GI symptoms, compared to controls.

Systemic Lupus Erythematosus Presenting as Monocular Elevation Deficiency

Purpose: To report a case of monocular elevation deficiency as the presenting manifestation of systemic lupus erythematosus (SLE). Case summary: A 23-year-old, otherwise healthy female presented with a 3-day history of vertical diplopia and headache. She had a left hypotropia, which worsened in adduction and supra-duction and a profound inferior oblique underaction (-3). Magnetic resonance imaging showed an enhancement around the left superior oblique muscle and multiple infarctions in the left midbrain. On repetitive serological tests, anemia, lymphopenia, and anti-phospholipid antibody were positive. A presumptive diagnosis was a myositis of left superior oblique muscle and hyper-coagulation related with anti-phospholipid antibody. Two months after high-dose steroid treatment, the vertical diplopia was resolved. Five months later, the left hypotropia recurred as a more severe form with the inability to elevate the left eye in all directions. In addition, the infarction associated with vasculitis recurred in the left midbrain. As the treatment with high-dose steroid failed to relieve her ocular symptoms, recession of the left inferior rectus was performed 8 months later. One month after the surgery, she developed multiple lesions of erythematous nodosa with tenderness. Skin biopsy of the lesion in the fingers showed the histological findings consistent with lupus. Conclusions: Eye movement abnormality can be an initial manifestation of SLE, which should be considered as a differential diagnosis especially in young female patients. J Korean Ophthalmol Soc 2016;57(1):161-166