Myocarditis and Central Retinal Vein Occlusion as Initial Presenting Symptoms of Systemic Lupus Erythematosus, A Case ReportAuthorsMehrdad Jafari Fesharakia, MD aNegar Raissi Dehkordi, MD b, Zahra Zakeri, MDc, Emadoddin Hosseinjani, MDd,, Nastaran Raissi Dehkordi, MD eaAssistant Professor of CardiologyDepartment of Cardiology, School of MedicineCardiovascular Research CenterShahid Labbafinezhad HospitalEmail: mehrjfmd@yahoo.combCardiovascular Research CenterShahid Labbafinezhad HospitalShahid Beheshti University of Medical SciencesEmail: n.raissi@sbmu.ac.irc Professor of RheumatologyDepartment of Adult Rheumatology, School of MedicineShahid Labbafinezhad HospitalShahid Beheshti University of Medical SciencesEmail: zah_zakeri@yahoo.comdAssistant Professor of CardiologyDepartment of Cardiology, School of MedicineCardiovascular Research CenterShahid Labbafinezhad HospitalEmail: HosseinjaniH@mums.ac.ire bCardiovascular Research CenterShahid Labbafinezhad HospitalShahid Beheshti University of Medical SciencesEmail: raissi@sbmu.ac.irAddress for correspondenceNastaran Raissi Dehkordi, MDDepartment of Cardiology, School of Medicine, Cardiovascular Research CenterShahid Labbafinezhad HospitalShahid Beheshti University of Medical Sciencesraissi@sbmu.ac.irphone number: +989128077485Key Clinical MessageSystemic lupus erythematosus may present with atypical symptoms, including central retinal vein occlusion, myocarditis, and deep vein thrombosis, highlighting the need for a comprehensive diagnostic approach. Early diagnosis and prompt treatment help mitigate disease morbidity and mortality, and emphasize the importance of heightened clinical suspicion in complex presentations.AbstractBackground: This article presents the case of a patient diagnosed with systemic lupus erythematosus (SLE) who exhibited a rare and atypical combination of initial symptoms, including acute visual loss, myocarditis, and deep vein thrombosis. This case underscores the significance of reporting such manifestations, contributing to a deeper understanding of the diverse clinical presentations of SLE.Case Presentation: A 35-year-old patient with a history of pulmonary embolism (PTE) and deep vein thrombosis (DVT) presented with acute visual loss, myocarditis, and DVT as the initial symptoms of SLE. The patient’s medical history also included epilepsy treated with Sodium Valproate. Physical examination revealed a malar rash, unilateral lower extremity edema, and complete vision loss in the right eye. Laboratory investigations showed elevated cardiac troponin levels and markedly increased D-dimer levels. Doppler sonography confirmed the diagnosis of DVT, revealing echogenic non-compressible popliteal veins and altered femoral vein diameters. Echocardiography demonstrated mild to moderate systolic dysfunction and severe hypokinesia in specific cardiac walls.Conclusions: This case highlights the rare and unusual nature of the patient’s presentation, combining central retinal vein occlusion, myocarditis, and DVT as initial symptoms of SLE. It emphasizes the importance of considering SLE in patients with seemingly unrelated symptoms and the need for prompt recognition and management. Early intervention in multi-organ system involvement of SLE can significantly enhance patient outcomes.KeywordsSystemic lupus erythematosus; Deep Vein Thrombosis; Lupus Myocarditis; Central Retinal Vein OcclusionIntroductionSystemic Lupus Erythematosus (SLE) is a chronic autoimmune disease characterized by immune complex deposition and autoantibody production, occurring predominantly in reproductive-age female patients (1). Multiple organ systems may be involved in patients with SLE, including skin, musculoskeletal system, kidneys, heart, and lungs (2). While ocular involvement may be a common manifestation of SLE, retinal vein occlusion is an uncommon occurrence in severe vaso-occlusive retinopathy related to SLE. This condition can appear as occlusion in major vessels, such as the central retinal vessels and the cilioretinal artery, or as extensive microembolization in small vessels. The most severe form of retinopathy in SLE can result in extensive damage to the retina and significant visual impairment (3) SLE can also lead to myocarditis, an inflammation of the heart muscle caused by immune complex deposition. Myocarditis in SLE can range from asymptomatic to severe heart failure, depending on the extent of inflammation and damage to the heart muscle (4). Deep vein thrombosis (DVT) is another known complication of SLE, caused by an increased risk of blood clots associated with the disease (5).This case report highlights the unique presentation of systemic lupus erythematosus (SLE) in a patient who presented with central retinal vein occlusion, deep vein thrombosis, and myocarditis as the initial symptoms. While each of these manifestations has been previously reported in SLE patients, the occurrence of all three simultaneously is extremely rare. This case highlights the importance of considering the possibility of SLE in patients presenting with seemingly unrelated symptoms, as prompt recognition and treatment can prevent further complications and improve outcomes. It also underscores the need for clinicians to be aware of the wide spectrum of multi-organ system involvement in SLE and to consider the possibility of the disease in the differential diagnosis of patients presenting with unusual symptoms.Case History/ExaminationA 35-year-old patient presented to the emergency department complaining of blurred vision starting two days ago, which had turned into complete visual loss in the right eye the next morning, in addition to swelling in the right leg, and a history of flu-like symptoms last week. The patient had a history of PTE and DVT following leg trauma two years ago and a history of epilepsy with unknown etiology which was under treatment with Sodium Valproate. According to the family history, the patient’s sister had passed away at the age of 20 due to SLE-related complications. However, it is unclear whether her morbidity was caused by antiphospholipid syndrome (APS), lupus nephritis, or other factors.Physical exam findings included a malar rash across the nose and cheeks, and unilateral edema of the right lower extremity. Ophthalmologic examination of visual acuity showed no light perception in the right eye, suggestive of occlusion in the central retinal vein. Additional physical exam findings were negative for oral or nasal ulcers, joint pain and swelling, pleural or pericardial rub, and lymphadenopathy.MethodsInitial blood investigations showed a white blood cell count of 7.8 × 10^3/μl with 59% neutrophil predominance and 38% lymphocytes, hemoglobin of 6.6 g/dl, and platelet count of 110 × 10^3/μl. The patient’s creatinine levels were within normal range (1.1 mg/dL) and his urine analysis did not show proteinuria. Cardiac troponin level was 8.1 ng/ml and D-dimer level was significantly increased (8000), while ECG showed normal sinus rate and rhythm with no ST or T changes (Figure 1). Doppler sonography of the right lower extremity showed echogenic non-compressible popliteal vein with increased diameter, suggestive of DVT. Follow-up venous Doppler sonography confirmed the initial diagnosis, and showed decreased diameter in common femoral vein (CFV) and distal part of superficial femoral vein (SFV), suggestive of acute DVT in distal SFV and popliteal vein, and chronic DVT in CFV and proximal SFV. Echocardiography revealed mild to moderate systolic dysfunction, (EF:40-45%), severe hypokinesia in the base mid-inferior wall, posterior wall, and inferoseptal wall, in addition to mild diastolic dysfunction, and moderate mitral regurgitation.Considering the patient’s family history of SLE (in the patient’s sister), and DVT in the lower limb, comprehensive rheumatologic lab exams were requested for the patient. Complement levels including C3, C4, and complement CH50 were within normal range. Antinuclear antibody (ANA) was positive in two separate tests, in addition to two positive (>1/160) titers of anti-double-strand DNA (anti-dsDNA). Anti-Cardiolipin Ab (IgG) (ELISA), Anti Cardiolipin Ab (IgM) (ELISA), B2 Glycoprotein Ab (lgG) were negative, while Anti-beta2-glycoprotein I Ab (IgM and IgG) levels were increased. P.ANCA (Anti-MPO), C.ANCA (Anti-PR3) were negative. A summary of the patient’s rheumatologic lab results is shown in Table 1. Coagulation tests for Protein C, S, Antithrombin III, and Factor V Leiden were within normal limits.Due to elevated Troponin levels, the patient was admitted to the cardiac care unit with a possible diagnosis of acute coronary syndrome and DVT. A coronary angiogram was obtained, which showed normal coronary arteries. Based on the patient’s family history of SLE, presenting symptoms, and three positive laboratory findings (ANA, anti-dsDNA, Anti-beta2-glycoprotein I Ab), a diagnosis of SLE was reached. Additionally, the diagnosis of APS was suspected due to an episode of thrombosis and positive Anti-beta2-glycoprotein I Abs. After ruling out coronary artery disease, a diagnosis of lupus myocarditis was reached, on the basis of clinical findings, laboratory tests (elevated levels of cardiac troponin and anti-dsDNA) and imaging tests (echocardiogram).Treatment was initiated with high-dose corticosteroids, anticoagulant agents, and hydroxychloroquine. Workup for ACS and DVT was initiated, including Warfarin 5 mg once daily for anticoagulation, and the patient’s INR level was controlled in a range of 3.0-4.0 due to recurring episodes of DVT. An MRI was requested, which did not show any signs of vasculitis and ruled out the need to administer cytotoxic therapies. Regular consultations with cardiology, rheumatology, ophthalmology, and nephrology specialists were conducted to follow the patient’s condition.Conclusion and ResultsThe patient’s visual acuity remained unchanged, and he was discharged with instructions for continued medical treatment and weekly follow-up visits with instructions to report any vision deterioration.DiscussionMultiple organ systems may be involved in SLE, including cutaneous, musculoskeletal, renal, cardiac, hematopoietic, and ocular (6). Affecting up to one third of the patients, ocular manifestations are a common finding in SLE patients. Although the most common ocular finding is keratoconjunctivitis sicca, more serious complications such as optic neuritis and occlusive vasculitis may occur, leading to potential visual loss (3,7). Cardiac manifestations may include pericarditis, myocarditis, Libman-Sacks endocarditis, and coronary artery disease (8). Although lupus myocarditis remains a rare manifestation of SLE and occurs in 5-10% of patients (8,9), the potentially life-threatening complications of lupus myocarditis, including heart failure, arrhythmias, and sudden cardiac death (10) underscore the need for prompt diagnosis and management. Although ocular and cardiac manifestation may be prevalent in SLE patients, a combination of central retinal vein occlusion, myocarditis, and DVT manifesting as the initial symptoms in the same patient is an extremely rare finding, and to the best of our knowledge, has not been reported in the literature. Although this case report presents a rare and unusual presentation of SLE, there are several limitations that need to be acknowledged. Firstly, this is a single case report an d may not be representative of the general patient population. Secondly, a diagnosis of APS requires positive laboratory criteria on two occasions taken at least 12 weeks apart, and thus long-term follow-up is required, in which the patient is asked to stop taking anticoagulation medication for a week, and is tested for APS laboratory criteria in order for us to reach a definite diagnosis of APS. Thirdly, the patient’s incomplete family history might include confounding information, which is unfortunately not available.In conclusion, the primary take-away lessons from this case report are the need for thorough and comprehensive evaluation of patients presenting with atypical symptoms, and the importance of interdisciplinary collaboration in diagnosing and managing complex medical cases.Consent for publicationSigned informed consent was obtained from the patient regarding the use of patient information for the purposes of writing a case report publication.Competing interestsThe authors declare that they have no competing interests.FundingThis work did not receive any funding from private or public sources.AcknowledgementsNot applicable.FundingThis research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.DisclosureThe authors declare that there are no conflicts of interest.Author ContributionsM.J.F: Conceptualized the study with N.R.D., wrote the manuscript and provided final approval.Ne.R.D: Participated in drafting and writing the manuscript and collected patient data.Z.Z: Critically revised the manuscript.E.H.: Critically revised the manuscript.Na.R.D: Co-conceived the study, participated in drafting and writing the manuscript and critically revised the content.All authors provided final approval of the study.Figure LegendFigure 1. ECG obtained upon admission showed normal sinus rate and rhythm.References1. Moulton VR, Suarez-Fueyo A, Meidan E, Li H, Mizui M, Tsokos GC. Pathogenesis of Human Systemic Lupus Erythematosus: A Cellular Perspective. Trends Mol Med. 2017 Jul;23(7):615–35.2. Fanouriakis A, Tziolos N, Bertsias G, Boumpas DT. Update οn the diagnosis and management of systemic lupus erythematosus. Ann Rheum Dis. 2021 Jan;80(1):14–25.3. Silpa-archa S, Lee JJ, Foster CS. Ocular manifestations in systemic lupus erythematosus. Br J Ophthalmol. 2016 Jan;100(1):135–41.4. Raval JJ, Ruiz CR, Heywood J, Weiner JJ. SLE strikes the heart! A rare presentation of SLE myocarditis presenting as cardiogenic shock. BMC Cardiovasc Disord. 2021 Jun 13;21(1):294.5. Al-Homood IA. Thrombosis in systemic lupus erythematosus: a review article. ISRN Rheumatol. 2012 Jul 30;2012:428269.6. García-Carrasco M, Pinto CM, Poblano JCS, Morales IE, Cervera R, Anaya JM. Systemic lupus erythematosus. El Rosario University Press; 2013.7. Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: a review of the literature. Autoimmune Dis. 2012 Jul 2;2012:290898.8. Wang TKM, Chan N, Khayata M, Flanagan P, Grimm RA, Griffin BP, et al. Abstract 13131: Cardiovascular Manifestations and Outcomes in Systemic Lupus Erythematosus: The Cleveland Clinic Contemporary Experience. Circulation. 2020 Nov 17;142(Suppl_3):A13131–A13131.9. Al-Nokhatha SA, Khogali HI, Al Shehhi MA, Jassim IT. Myocarditis as a lupus challenge: two case reports. J Med Case Rep. 2019 Nov 20;13(1):343.10. Al-Akchar M, Shams P, Kiel J. Acute Myocarditis. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2022.
