Initial Presentation Of Systemic Lupus Erythematosus Research Articles

Severe pulmonary hypertension as the initial manifestation of systemic lupus erythematosus: A case report a 36 years old man

Severe pulmonary hypertension as the first sign of systemic lupus erythematosus (SLE) is uncommon, but has a considerable influence on prognosis compared to other published cases of SLE. However, with the development of new therapeutic alternatives, patient mortality has decreased significantly in recent years. This case, involving a 36-year-old man whose initial presentation of SLE was severe PAH, illustrates the importance of early detection in these individuals to achieve better outcomes.

Cerebritis as an initial presentation of systemic lupus erythematosus

Systemic lupus erythematosus (SLE), an autoimmune disease with multi-system manifestations, has several clinical presentations varying from mild muco-cutaneous involvement to severe multi-organ involvement. We report the case of an 18-year-old girl who presented generalized tonic clonic fits. She gave a history of generalized headaches, recurrent painless oral ulcers, photosensitivity, joint pains and low-grade intermittent fever. Magnetic resonance imaging (MRI) scan of the brain revealed T2WI/FLAIR hyperintense signals in bilateral frontal, parietal and occipital regions and magnetic resonance angiography of the brain demonstrated mild areas of luminal narrowing in M1 and M2 segments of middle cerebral arteries bilaterally, suggestive of lupus cerebritis. Anti-nuclear antibody (ANA) and Anti-DsDNA antibodies were positive. She was diagnosed as SLE and was prescribed corticosteroids, cyclophosphamide and hydroxychloroquine. This case highlights cerebritis as initial presentation of SLE.
 Abbreviations: ANA- Anti-nuclear antibody; CRP- C-reactive protein; FLAIR- Fluid attenuated inversion recovery; SLE- Systemic lupus erythematosus;
 Key words: ANA; Cerebritis; Headache; Systemic Lupus Erythematosus
 Citation: Ashfaq F, Butt NI, Rasheed MB, Ghoauri MSA, Anser A. Cerebritis as initial presentation of systemic lupus erythematosus. Anaesth. pain intensive care 2023;27(4):604−608; DOI: 10.35975/apic.v27i4.2214
 Received: May 23, 2023; Reviewed: May 28, 2023; Accepted: July 08, 2023

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.

Posterior segment involvement in systemic lupus erythematosus – A series from South India

To report the spectrum of posterior segment manifestations and visual outcomes in a large series of patients with systemic lupus erythematosus (SLE).Retrospective study at a tertiary referral eye center in south India between 2016 and 2022.Charts of 109 patients diagnosed to have SLE were retrieved from our medical database. Only nine cases of SLE (8.25%) had posterior segment involvement. The male: female ratio was 1:8. The mean age was 28 years. Unilaterality was the most common presentation in eight cases (88.89%). Lupus nephritis was the most common systemic presentation in five cases (55.56%). Antiphospholipid antibodies (APLA) positivity was seen in two cases (22.22%). Ocular manifestations included microangiopathy (cotton wool spots) in one case, occlusive retinal vasculitis with cotton wool spots in four cases (five eyes), optic disc edema with combined venous and arterial occlusion (one case), central retinal vein occlusion with cotton wool spots and hemorrhages (one case), macular edema (four cases), posterior scleritis with optic disc edema and exudative retinal detachment in the posterior pole (one case), and tubercular choroidal granuloma (one case). Treatment included systemic steroids, hydroxychloroquine sulfate (HCQS), and immunosuppression in all cases, blood thinners in two cases, and laser photocoagulation in four cases. HCQS-related retinal toxicity was not seen in any of the 109 cases. Ocular manifestation was the initial presentation of SLE in one case. Visual outcome was poor in three cases.Presence of posterior segment findings in cases with SLE may suggest a severe systemic disease. Early detection and aggressive treatment result in better visual outcomes. Ophthalmologists could play a vital role in guiding systemic therapy.

Cardiac tamponade in a male as an initial presentation of SLE. An interesting case report

A relapsing and remitting illness that may affect any organ system, systemic lupus erythematosus (SLE) is a chronic autoimmune inflammatory condition. Autoantibodies and immune complex development are its defining features. The heart is one of the organs that it might impact. In patients with SLE, the immune system’s response to dying cells is aberrant. The diagnosis of SLE is made on the basis of distinctive clinical findings in the skin, joints, kidneys, and central nervous system, as well as serological indicators like antinuclear antibodies (ANA), specifically antibodies to dsDNA. Nonsteroidal anti-inflammatory medicines (NSAIDs), corticosteroids, immunosuppressive medications, and biological agents may be used to treat lupus, depending on the symptoms, findings of blood tests, and the specific organs affected. Here we are reporting a rare case of a male with pericardial tamponade as a first symptom of SLE.

Unusual Case Presentation of Systemic Lupus Erythematosus in a Young Woman

Systemic Lupus Erythematosus (SLE) is a chronic multisystem autoimmune disease. Serositis occurs in 16% of SLE patients, and while cardiac tamponade and acute peritonitis with ascites can occur during the course of the disease, they are rare as the first presentation. A 25-year-old woman presented to the emergency department in Tishreen Hospital with complaints of dyspnea, fever, chills, and chest and abdominal pain. Two months prior, she suffered from musculoskeletal pain, fatigue, anorexia, weight loss of about 15 kg, severe hair loss, and recurrent oral aphthous. On clinical examination, the patient was pale and tired with dyspnea and pitting edema (grade 3–4). Pericardiocentesis was emergently performed because there were signs of cardiac tamponade. Three days later, the patient developed an acute surgical abdomen due to acute peritonitis and ascites. Later, the patient was diagnosed with SLE after excluding malignant and infectious diseases. Consequently, methylprednisolone pulses, azathioprine, and hydroxychloroquine 200 mg/day were introduced immediately. The clinical status of the patient dramatically improved, and three months later, the patient was symptom-free with normal laboratory tests. In conclusion, although cardiac tamponade and acute surgical abdomen because of acute peritonitis and ascites as the initial presentation of SLE are very rare, they can occur coincidently.

CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Endocapillary hypercellularity levels are associated with early complete remission in children with class IV lupus nephritis as the initial presentation of SLE

BackgroundEndocapillary hypercellularity (ECHC) is commonly seen in class IV lupus nephritis (LN), the most common and severe LN in children. Factors influencing early complete remission (CR) in pediatric class IV LN have been poorly described. We investigated the relationship between ECHC levels and early CR in pediatric class IV LN.MethodsPatients with newly, simultaneously diagnosed systemic lupus erythematosus (SLE) and class IV LN by renal biopsy from 2012 to 2021 were studied. In this retrospective study, two pathologists who were blind to clinical information reviewed all pathological data retrospectively and classified glomerular lesions according to the revised criteria of the International Society of Nephrology and the Renal Pathology Society (ISN/RPS). The demographics, baseline clinical characteristics, laboratory parameters, renal histopathological findings, treatment regimen and CR at 6 months after immunosuppressive therapy were analyzed. ECHC was categorized as: > 50% (group A), 25–50% (group B) and < 25% (group C). CR was defined as absence of clinical symptoms, 24-hour urinary protein < 0.15 g, and normal levels of serum creatinine and albumin.ResultsSixty-four patients were identified: 23, 15 and 26 in groups A, B and C, respectively. Group A had significantly higher levels of D-dimer, urine protein, and SLE disease activity index (SLEDAI) than groups B and C. Group C had a markedly higher estimated glomerular filtration rate (eGFR) than groups A and B. A substantially greater proportion of patients in group A had glomerular microthrombi and basement membrane thickening than in groups B and C. At 6 months post treatment, CR was achieved in 19 (82.6%), 5 (33.3%) and 11 (42.3%) in groups A, B and C, respectively (p < 0.05, group A vs groups B and C). Multiple logistic regression analysis revealed that ECHC and urine protein levels were significantly associated with CR.ConclusionECHC and urine protein levels may be valuable biomarkers for predicting early CR in pediatric class IV LN.

Early Intervention and Resolution of Pediatric Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus: A Pediatric Case Report

Early Intervention and Resolution of Pediatric Intestinal Pseudo-Obstruction in Systemic Lupus Erythematosus: A Pediatric Case Report

Pancreatic pseudocyst as initial presentation of a 22-year-old male lupus patient

Systemic Lupus Erythematosus (SLE) related acute pancreatitis is an unusual feature of SLE that lead to serious complication like pancreatic pseudocyst. Acute pancreatitis or pancreatic pseudocyst as the initial presentation of SLE is rarely been reported. A 22-year-old gentleman presented with fever, abdominal pain with distension and facial puffiness. Examination revealed anaemia, Alopecia, tachycardia, oedema, Blood pressure of 110/70 mm of Hg, abdominal tenderness, abdominal distension and crackles. Urine examination showed active sediments with proteinuria of 3.8g/day. ANA and Ani-dsDNA were positive. Serum amylase and Lipase were 192U/L and 658U/L respectively. A computed tomography scan showed a Pancreatic pseudocyst of size 55 42 38 mm. Kidney biopsy revealed diffuse proliferative glomerulonephritis with full house immune complexes. Follow-up ultrasonography showed resolution of Pseudocyst. Conclusion: High index of suspicion is required to diagnose SLE related pancreatitis as presenting features of SLE may mask it. This might ensure early diagnosis and treatment to prevent complications of the disease.

ORGANIZING PNEUMONIA: A RARE MANIFESTATION OF EARLY-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS

SESSION TITLE: Medical Student/Resident Diffuse Lung Disease SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Systemic Lupus Erythematosus (SLE) affects lungs (30-50%) with pleurisy being the commonest manifestation. Interstitial Pneumonia (IP) related to SLE was found be less reported about 10% and Organizing Pneumonia (OP) type of IP was noted to be very rare (1). Here, we present a rare case of Organizing pneumonia manifesting in SLE CASE PRESENTATION: A 30-year-old female with history of left breast carcinoma status post bilateral mastectomy, adjuvant chemotherapy and radiation therapy completed in March 2019, sickle cell trait and SLE, first presented in January 2020 with dyspnea and cough. CT chest revealed bilateral lower lobe predominant consolidative opacities with air bronchogram and multifocal sub-pleural ground glass opacities throughout both the lungs [Fig 1A], she was treated for pneumonia. On next admission, she also complained of generalized myalgias/arthralgias and unresolving skin rashes on extremities and neck with topical steroids. No other system involvement except for proteinuria. ANA, ds-DNA and lupus anticoagulant were positive with elevated CRP. CT chest slightly worsened compared to previous CT [Fig 1B]. Bronchoscopy revealed normal gross appearance. RLL trans-bronchial biopsy pathology showed mild chronic interstitial inflammation, intra-alveolar macrophages and focal secondary organizing pneumonia with no specific features. She was started on Plaquenil and long-term low dose steroids that controlled her symptoms DISCUSSION: SLE commonly manifests as lung pathology varying from pleurisy (50%), lung parenchymal disease (10-15%) and pulmonary vascular hypertension (1,2). SLE induced IP commonly presents as Chronic IP (63.6%). The most common HRCT finding is unclassifiable and Nonspecific IP (NSIP) is shown to be the most common histopathology (1). Previous articles reported that pulmonary manifestations especially ILD and serositis usually present as a late manifestation or in SLE patients diagnosed at an older age (1,3,4) with no gender predominance. OP has a well-known association with connective tissue disorders like Rheumatoid Arthritis and Myositis but there are only a handful of cases of SLE reported in association with Bronchio-Obliterans Organizing Pneumonia (BOOP), and commonly seen in older women(1). In contrast our patient was young 30-year-old female who had isolated Organizing pneumonia identified as an early manifestation / initial presentation of SLE. Also, it is possible that OP resulted following a predisposing pulmonary insult in SLE – pneumonia and chest radiotherapy. It was noted from metanalysis that NSIP with OP was a favorable prognosis in patients with SLE compared to other histopathology (2,5) CONCLUSIONS: Though rare, Organizing Pneumonia can occur at any age of onset/ duration of SLE and early diagnosis and treatment with steroids and immunosuppression decreases morbidity and mortality from lung manifestations in SLE, given its favorable prognosis Reference #1: 1. Toyoda Y, Koyama K, Kawano H, Nishimura H, Kagawa K, Morizumi S, et al. Clinical features of interstitial pneumonia associated with systemic lupus erythematosus. Respir Investig. 2019;57(5):435–43. Reference #2: 2. Enomoto N, Egashira R, Tabata K, Hashisako M, Kitani M, Waseda Y, et al. Analysis of systemic lupus erythematosus-related interstitial pneumonia: a retrospective multicentre study. Sci Rep. 2019;9(1):1–11.v Reference #3: 5. Tselios K, Urowitz MB. Cardiovascular and Pulmonary Manifestations of Systemic Lupus Erythematosus. Curr Rheumatol Rev. 2017;13(3):206–18. DISCLOSURES: No relevant relationships by Rathnavali Katragadda, source=Web Response No relevant relationships by Vinod Khatri, source=Web Response No relevant relationships by Leela Krishna Vamsee Miriyala, source=Web Response No relevant relationships by Shanti Pittampalli, source=Web Response

Lupus erythematosus tumidus (LET) with autoimmune thyroid dysfunction (AITD) as the first presentation of systemic lupus erythematosus: A case report and review of the literature

IntroductionLupus erythematosus tumidus (LET) is a rare cutaneous manifestation especially as a first presentation of systemic lupus erythematosus (SLE). Autoimmune thyroid dysfunction (AITD) may be associated with SLE but rarely at initial presentation, and its diagnosis may be delayed. Case reportA 29 year old male presented to Tishreen Hospital in Damascus with a three-year history of recurrent cellulitis-like lesions on the face, and more recently, he developed similar lesions on the trunk and the chest, in addition to the development of peripheral and scrotal edema, constipation, xeroderma, hair loss, musculoskeletal pain and depression. Laboratory investigations revealed: leukopenia, anaemia, elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). Immunological tests identified the positive anti-nuclear antibody (ANA), anti-double stranded DNA (anti-dsDNA), anti Ro/SSA, anti La/SSB antibodies. Additionally, there was consumed complement C3, elevated thyroid stimulating hormone (TSH), thyroid hormones decreased free T3 and T4 and anti-thyroid peroxidase (anti-TPO) antibody was positive. Skin biopsy from the cheek plaque suggested the presence of LET and revealed slight hyperkeratosis; actinic elastosis, telangiectasia and edema of the papillary dermis; deep dermis perivascular and periadnexal inflammatory infiltrates with karyorrhexis of the lymphocytes and dermis edema between strands of collagen. The patient fulfilled the SLE classification criteria and consequently, methylprednisolone, azathioprine, hydroxychloroquine, levothyroxine were introduced with dramatic improvement. ConclusionLET is a rare cutaneous lupus-specific lesion that may be associated with SLE. AITD, hypothyroidism in particular, could be an initial presentation of SLE. Increased awareness and early diagnosis of such clinical presentations may improve patient outcomes.

Intracranial hypertension as an initial clinical manifestation of Systemic Lupus Erythematosus

Abstract Background Systemic Lupus Erythematosus (SLE) is an auto-immune systemic disorder that may affect multiple organ systems including central nervous system. Although involvement of the nervous system and eye is not uncommon in SLE, the presence of intracranial hypertension (IH) with papilledema in SLE a rare occurrence and it requires high level of suspicion to be diagnosed, especially when the combination of IH and papilledema is the initial presentation of SLE. Case presentation A 21-year-old female presented with a month long history of progressively deteriorating bilateral frontotemporal headache of throbbing quality. In neurological examination the only finding was bilateral papilledema with mild peripheral visual field Constriction. A brain Magnetic Resonance Imaging (MRI), MR angiography and venography showed no abnormal findings. Cerebrospinal fluid (CSF) analysis revealed a high opening pressure (320 mm H2O) with normal CSF indices. She also had normocytic, normochromic anemia, mild thrombocytopenia, increased quantitative CRP, ESR and LE cell was also reported. Upon further work-up, it was revealed that she had positive ANA and Anti-ds-DNA, decreased level of complement C3 and C4. She, with SLE, was treated with corticosteroid which resulted in significant improvement in patient's manifestation of IH. Conclusions The association of IH and SLE has already been established and one should be aware of this coexistence and in IH cases, SLE should also be considered as an underlying cause and subsequently appropriate diagnostic tests should be performed.

An atypical life-threatening presentation, with delayed diagnosis, in a 22-year-old female

Acute lupus pneumonitis (ALP) is a rare life-threatening complication in systemic lupus Erythematosus (SLE) that may present with nonspecific respiratory symptoms. Typically, ALP presents following the formal diagnosis of SLE. Here we present the first case in North America, to our knowledge, of acute lupus pneumonitis as the initial presentation of SLE, with no prior respiratory symptoms and only one ACR (American College of Rheumatology) criteria fulfilled for SLE diagnosis.

Neuropsychiatric Systemic Lupus Erythematosus in Children.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the presence of antinuclear antibodies and other autoantibodies, as well as a clinical course that is characterized by flares and remissions. The clinical presentation is diverse, ranging from a mild disease characterized by rash and arthritis to a severe life-threatening disease involving multiple organs. Approximately 25% of children with SLE have neuropsychiatric manifestations of SLE, which are a major cause of morbidity and mortality. Neuropsychiatric symptoms may be the initial presentation of SLE in children. The mortality rate is relatively low, but morbidity may be significant and permanent damage can occur. This article discusses the importance, known pathophysiologic mechanisms, clinical approach, and evidence-based therapeutic options for the diagnosis and management of neuropsychiatric lupus erythematosus in children and adolescents.

DIGITAL GANGRENE AS THE INITIAL PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS WITHOUT SECONDARY ANTIPHOSPHOLIPID SYNDROME: CASE REPORT

This is a case of a 28-year-old female presented with an extensive cutaneous gangrene of her fingers and toes with history of a livedo reticularis rash in both lower limbs. Her work-up showed positive serology for Systemic Lupus Erythematosus (SLE). There were no other symptoms suggestive of SLE. Interestingly, the patient adequately responded to rituximab therapy. Such a case of digital gangrene as the initial presentation of SLE without secondary antiphospholipid syndrome has rarely been reported in the literature.

Sometimes There Is More Than One Puzzle on the Table: Pneumococcal Bacteremia as a New Systemic Lupus Erythematosus Presentation.

Infection is common and a leading cause of death in patients with systemic lupus erythematosus (SLE). SLE is associated with a diverse spectrum of immune impairments including humoral defects and hypocomplementemia that contribute to a lupus patient's increased susceptibility to infection with encapsulated bacteria. Nonetheless, there are only few reports of severe invasive bacterial infection as the initial presentation of SLE in the literature. Here, we report a rare case of SLE presenting with pneumococcal bacteremia. Based on the high resolution chest computed tomography and the result of blood cultures, the bacteremia was assumed to be secondary to pneumococcal pneumonia.

Acute longitudinal myelitis as the first presentation in child with systemic lupus erythematosus concurrent with positive antiphospholipid antibody

Systemic lupus erythematosus (SLE) is a multi-system auto-immune disorder that is characterized by widespread immune deregulation, formation of auto–antibodies, and immune complexes, resulting in inflammation and potential damage to variety of organs. 25-95% it is complicated by neurological or neuropsychiatric symptoms, which is referred to as neuropsychiatric SLE (NPSLE). NPSLE contain both central and peripheral nervous systems, which includes transverse myelitis. We report our experience of concurrent manifestation of transverse myelitis as an initial presentation of SLE, which suggests the common immune-mediated mechanisms of diseases. We here report the case of a 7-year-old girl with SLE who first presented with features of TM. The patient developed ascending weakness starting from low extremities, experienced difficulty in voiding. An initial diagnosis of TM was made on the basis of clinical findings and MRI spine, which displayed T2 weighted high signal intensities at thoracic level. She partially respond to intravenous immunoglobulin therapy, and serological analysis revealed the presence of anti-dsDNA, anti nuclear antibody with decreased level of complements. The diagnosis was revised to acute transverse myelitis resulting from SLE. Additional methylprednisolone pulse therapy led to rapid clinical improvement. This was followed by oral prednisolone and cyclophosphamide pulse therapy. The crossreactivity of auto-antibodies and increased susceptibility to infection owing to immunologic changes associated with lupus may form the basis of the association. Systemic Lupus Erytheromyitis should consider as an etiology of transverse myelitis. Aggressive treatment may alter the course and lead to a better outcome.

Interplay of Infections, Autoimmunity, and Immunosuppression in Systemic Lupus Erythematosus

Infectious agents are considered to be crucial environmental factor in the etiopathogenesis of systemic lupus erythematosus (SLE). Infections may serve as initial trigger to the development of autoimmunity and carry an overall greater risk of morbidity and mortality than the general population. Initial presentation of SLE can mimic infections, and in turn infections can mimic disease flares in established SLE. Infections due to predisposition by commonly used immunosuppressive therapies are a significant cause of morbidity and mortality. In this review, viral, bacterial, fungal, and parasitic infections that contribute to the etiology of SLE, potentially mimic or precipitate flares, create diagnostic dilemmas, complicate treatment, or protect against disease, are discussed. Infection risks of current immunosuppressive therapies used in the treatment of SLE are outlined. Strategies to prevent infection, including vaccines, prophylactic antibiotic therapies, toll-like receptor antagonism, and antioxidant treatment that may decrease disease burden and improve quality of life in lupus patients will be discussed.

Acquired Von Willebrand's Syndrome in Systemic Lupus Erythematosus.

Acquired von Willebrand syndrome (AVWS) is an uncommon, underdiagnosed, and heterogeneous disease which is increasingly recognized as a cause of bleeding diatheses. Systemic lupus erythematosus (SLE) is an infrequent cause of AVWS. Herein, we report a case of AVWS diagnosed during the initial presentation of SLE in a previously healthy young man with no family history of bleeding diathesis who presented with worsening epistaxis, gastrointestinal bleeding, and anasarca. He was found to have severe anemia and prolonged activated partial thromboplastin time (aPTT) with severely decreased levels of von Willebrand factor (VWF) measurements in addition to markedly decreased factor VIII levels. Further evaluation revealed nephrotic syndrome and interstitial lung disease due to SLE. He initially received combination therapy with intravenous immunoglobulin (IVIG) and von Willebrand factor/factor VIII concentrates without significant improvement. Treatment with steroids, cyclophosphamide, and rituximab was followed by clinical improvement evidenced by cessation of bleeding. The short follow-up did not allow us to definitely prove the therapeutic effect of immunosuppressive treatment on AVWS in SLE patients. This case adds to the literature supporting the relationship between AVWS and SLE and highlights the importance of combination therapy in the treatment of severe AVWS as well as the role of IVIG, cyclophosphamide, and rituximab in AVWS associated with SLE.