|Year : 2022 | Volume
| Issue : 1 | Page : 89-93
Systemic lupus erythematosus presenting as unprovoked brachio-cephalic vein thrombosis: A case report
Gautam Jesrani, Samiksha Gupta, Shivani Gupta, Tagru Raju, Monica Gupta
Department of General Medicine, Government Medical College and Hospital, Chandigarh, India
|Date of Submission||19-Sep-2021|
|Date of Acceptance||27-Sep-2021|
|Date of Web Publication||23-May-2022|
Dr. Gautam Jesrani
Department of General Medicine, Government Medical College and Hospital, Sector 32, Chandigarh - 160 030
Source of Support: None, Conflict of Interest: None
Systemic lupus erythematosus (SLE) is an autoimmune disease with multisystem involvement, and majorly affects the female population of the childbearing age. Vascular thrombosis is a known complication in the natural disease course, but the thrombotic event as the initial presentation of SLE is rarely depicted, especially for the thoracic vasculature. Herein, we are narrating a case of a 38-year-old gentleman, who previously suffered from myocardial infarction, due to left coronary artery occlusion and this time presented with left brachiocephalic vein thrombosis. On evaluation, SLE was diagnosed, without antiphospholipid syndrome, and a standard treatment of anticoagulant with hydroxychloroquine was prescribed for this. This report illustrates that an unusual vascular location can be involved in SLE and reemphasizes that a thorough immunological work-up should be performed promptly in the absence of any identified etiology.
Keywords: Anti-phospholipid syndrome, brachiocephalic vein thrombosis, coronary artery occlusion, systemic lupus erythematosus, venous thrombosis
|How to cite this article:|
Jesrani G, Gupta S, Gupta S, Raju T, Gupta M. Systemic lupus erythematosus presenting as unprovoked brachio-cephalic vein thrombosis: A case report. J NTR Univ Health Sci 2022;11:89-93
|How to cite this URL:|
Jesrani G, Gupta S, Gupta S, Raju T, Gupta M. Systemic lupus erythematosus presenting as unprovoked brachio-cephalic vein thrombosis: A case report. J NTR Univ Health Sci [serial online] 2022 [cited 2023 Feb 4];11:89-93. Available from: https://www.jdrntruhs.org/text.asp?2022/11/1/89/345799
| Introduction|| |
Systemic lupus erythematosus (SLE) is a multisystem disorder involving autoimmune infirmity, predominantly affects the female population of age 15–44 years. Several cardiovascular, renal, musculoskeletal, and nervous system–related complications have been stated, which can range from minimal or silent to grave or life-threatening implications. In the cardiovascular system, arterial and/or venous thrombosis have been extensively described for SLE, and the incidence is found to be 29.1 per 1000 patient-years. This complication is more in the first year of primary disease diagnosis, and a leading cause of mortality (26.5%) among these patients.,
| Case Presentation|| |
A 38-year-old male presented to our emergency department with the chief complaint of left- sided neck swelling from the last 5 h, which was first noticed by him early in the morning. Previously, the patient was also experiencing a feeling of heaviness in his left upper limb from the past 2 days, which was gradual in onset and not impairing his daily routine activity. The patient did not encounter limb numbness or edema, and there was no chest pain or palpitation associated with these symptoms. He was managing his own personal business and there was no history of substance addiction, chronic debilitating illness, or past trauma. Two years back, ST-segment elevation myocardial infarction was diagnosed in this patient, and he underwent percutaneous intervention for this in a different health center. Old records stated that a drug-eluting stent was placed in his left coronary artery, and dual antiplatelet drugs were prescribed afterward. The patient was lost to follow-up after 6 months and left the advised medications. Other than normal complete blood count, renal function tests, and coagulation profile, only lipid profile was available in his previous record, which was also inconclusive (total cholesterol 178 mg/dL, normal 120–200; triglyceride levels 124 mg/dL, normal 10–150; high density lipoprotein 39 mg/dL, normal 40–60; low density lipoprotein 89 mg/dL, normal <130; very low density lipoprotein 26 mg/dL, normal <30).
On presentation, the patient had no focal deficit, and his blood pressure was 122/80 mm Hg, capillary glucose was 87 mg/dL, and his oxygen saturation was 97%. The patient had an average built and neck examination on the left side demonstrated cord-like noncompressible structure with absent jugular pulsations. There was also local swelling with no signs of inflammation, and the right side of the neck was free of any pathology. Neurological examination, including the left upper limb, did not reveal any abnormality, and his cardiovascular and respiratory systems were within the normal limits.
Normal electrocardiography was recorded on the initial presentation, and the chest X-ray [Figure 1] demonstrated bilateral clear lung fields but blunting of both costophrenic angles. About 20 mL of pleural fluid was tapped from the right side, which demonstrated a leukocyte count of 20 cells/mm3 (40% neutrophils and 60% lymphocytes), protein levels of 3.1 gm/dL, adenosine deaminase value of 8 IU/L (Normal <10), and glucose levels of 68 mg/dL. No growth of any organism was detected on pleural fluid culture, and cartridge-based nucleic acid amplification test (CBNAAT) for tuberculosis was negative. In routine investigations, total leukocyte counts and hemoglobin levels were unremarkable, but the platelet count was 138 × 109 (Normal 150–450). His renal function tests were stable, coagulation profile (including prothrombin and activated partial thromboplastin time) had no abnormality, and lipid profile was also within the reference range this time. Urinalysis had no proteinuria or presence of pus cells, and thyroid function tests were inconclusive. Noncontrast computed tomography (CT) of the head had no pathology, and the nerve conduction velocity test was normal. The patient underwent Doppler scan of the neck, which raised the possibility of internal jugular vein (IJV) thrombosis and required CT angiography evaluation. The scan identified hypodense thrombus in the left brachiocephalic trunk, with an extension to the left IJV and subclavian vein [Figure 2]. There were no pulmonary infiltrates and bilateral lower limb doppler excluded the possibility of deep vein thrombosis. So, low molecular weight heparin 60 mg twice a day subcutaneously was started, which was bridged to oral warfarin from the second day of therapy.
|Figure 1: Chest X-ray demonstrating bilateral clear lung fields and blunting of the both costo-phrenic angles (yellow arrow)|
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|Figure 2: Computed tomographic angiography demonstrating hypodense, nonenhancing lesion in the left brachiocephalic (a), internal jugular (b), and subclavian (c) vein, suggestive of thrombus (green arrow)|
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For the etiology identification, serum fibrinogen levels were estimated, but had a negative result, and his anti-cardiolipin and lupus anticoagulant antibody analysis was within the normal range. Ham's test for paroxysmal nocturnal hemoglobinuria was inconclusive, and similar results were observed for factor V Leiden mutation, vitamin B12, and folic acid levels. In the absence of any identifiable etiology, anti-nuclear antibody (ANA) levels were measured and fortunately had a positive result (3+ intensity) with diffuse homogenous pattern (by immunofluorescence). In this continuation, antidouble strand deoxyribonucleic acid (ds-DNA) antibody levels were found to be 89 IU/dL (Positive >75); rheumatoid factor and anti-cyclic citrullinated peptide antibody levels were negative; C3 (89 mg/dL, normal 90–180) and C4 (12 mg/dL, normal 10–40) levels were unremarkable, and C-reactive protein levels were invariably raised (18 mg/L, normal 0–5). Thus, on the basis of clinical (exudative pleural fluid) and immunology criteria (ANA and ds-DNA), the patient was diagnosed with SLE and hydroxychloroquine (HCQ) 200 mg twice a day was added to his ongoing treatment. The symptoms of his left upper limb heaviness and neck swelling improved to some extent, and he was discharged on HCQ of the same dose, aspirin 75 mg/day, atorvastatin 20 mg/day, and warfarin 5 mg/day. International normalized ratio (INR) was maintained around 3, and the patient was advised for strict treatment adherence and regular follow-up.
| Discussion|| |
SLE is considered as an independent risk factor for arterial or venous thrombosis, and cardiovascular accidents, including thrombotic events, are the first complications of the disease “flare.”, About 50% of SLE patients have concurrent antiphospholipid syndrome (APS), which is characterized by thrombosis of different vascular territories and recurrent pregnancy losses. Chronic inflammation, leading to increased fibrinogen levels, endothelial damage and accelerated atherosclerosis, and hypercoagulable state are two crucial steps in the pathogenesis of SLE-related thrombosis. Ischemic heart disease, cerebrovascular infarcts, ischemic peripheral vascular disease, deep vein thrombosis, pulmonary thrombo-embolism, and retinal vein occlusion are some commonly outlined thrombotic events in SLE.
The diagnosis of SLE is based on diverse clinical and laboratory parameters, according to the guidelines of the European League against rheumatism/American College of Rheumatology 2019. On the basis of positive ANA and ds-DNA antibody titers, and exudative pleural effusion (serositis), our patient fulfilled this criterion and was diagnosed with SLE in search of the etiology of left brachiocephalic vein thrombosis. Similar to this unusual case, several other reports have been demonstrated arterial or venous thrombosis as the initial presentation of lupus, and some peculiar studies are included in [Table 1].,,,,,,,,,, Thus, the appearance of SLE does not always include the customarily described symptoms, and thrombotic episodes may be the initial affliction among the atypical presentation.
|Table 1: Important Studies Describing Systemic Lupus Erythematosus With Thrombotic Events|
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Before labeling SLE as the culprit etiology for thrombosis, it is mandatory to rule out other possibilities. In this, exclusion of APS is paramount as there is a significant association between these two diseases. APS is diagnosed when both laboratory i.e. presence of anticardiolipin antibody (IgG and/or IgM), lupus anticoagulant (LAC) and anti-beta2 glycoprotein-1 antibody (IgG and/or IgM), and clinical (vascular thrombosis and pregnancy morbidity) criteria are fulfilled. It is important to note that these antibodies may be present in SLE patients, without fulfilling the APS criteria and can lead to a hypercoagulable state. About 42% of LAC positive and 40% of anti-cardiolipin antibody-positive individuals with SLE have thrombotic events, and only 10%–18% of SLE patients have thrombosis in the absence of these antibodies. Along with this, estimation of protein C, protein S deficiencies, factor V Leiden mutation, vitamin B12 (an indirect marker of plasma homocysteine levels), B6 or folic acid deficiency is also crucial for ruling out the other possible differentials. Chronic renal failure, diabetes mellitus, dyslipidemia, hypertension, and hypothyroidism can be associated with SLE and promote a thrombotic state.
The treatment of SLE with thrombosis is relatively complex and depends on the presence or absence of APS and related antibodies, and underlying chronic conditions. HCQ, which is a standard remedy for SLE treatment, has also antithrombotic, platelet inhibitory, and cholesterol-reducing properties. In APS with SLE patients, a combination of aspirin (81–100 mg) with HCQ is used for primary prophylaxis, and anticoagulants should be added for thrombotic episodes. The target INR recommendation for SLE with APS is 3–4, but for SLE alone is 2–3. As followed in our case, recurring events should be treated with anticoagulants to achieve a target INR of 3–4.
| Conclusion|| |
To conclude, an extremely variable clinical presentation of SLE can include arterial or venous thrombosis, and any vascular territory can be affected. Unusual and unprovoked events should be evaluated with ANA and ds-DNA analysis, which has considerable sensitivity and specificity in the diagnosis establishment. Further, imaging studies like CT or magnetic resonance angiography are useful for the estimation of the thrombus extent. HCQ, which is a standard therapy for SLE, has antithrombotic properties, and anticoagulant drugs should be added for definite treatment of the thrombotic events.
Declaration of patient consent
A written consent is present, duly signed by the patient. The authors obtained the consent after explaining that no identity will be revealed. and the case information, including pictures will be used for education purpose only. He was also explained that journal publication will not contain any material or picture, disclosing his identity. The patient gave positive consent, and the authors certify that written consent is present, procured for publication.
A proper written consent is present, which was obtained from the patient for the use of the data related to this case.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Litvinov RI, Nabiullina RM, Zubairova LD, Shakurova MA, Andrianova IA, Weisel JW. Lytic susceptibility, structure, and mechanical properties of fibrin in systemic lupus erythematosus. Front Immunol 2019;10:1626.
Bazzan M, Vaccarino A, Marletto F. Systemic lupus erythematosus and thrombosis. Thromb J 2015;13:16.
Cooley I, Derebail VK, Gibson KL, Álvarez C, Poulton CJ, Blazek LN, et al
. Association of lupus nephritis histopathologic classification with venous thromboembolism-modification by age at biopsy. Kidney Int Rep 2021;6:1653-60.
Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al
. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-9.
Bertoli AM, Alarcón GS, Calvo-Alén J, Fernández M, Vilá LM, Reveille JD. Systemic lupus erythematosus in a multiethnic US cohort: Clinical features, course, and outcome in patients with late-onset disease. Arthritis Rheum 2006;54:1580-7.
Al-Homood IA. Thrombosis in systemic lupus erythematosus: A review article. ISRN Rheumatol 2012;2012:428269.
Montes de Oca MA, Babron MC, Blétry O, Broyer M, Courtecuisse V, Fontaine JL, et al
. Thrombosis in systemic lupus erythematosus: A French collaborative study. Arch Dis Child 1991;66:713-7.
Mintz G, Acevedo-Vázquez E, Gutiérrez-Espinosa G, Avelar-Garnica F. Renal vein thrombosis and inferior vena cava thrombosis in systemic lupus erythematosus. Frequency and risk factors. Arthritis Rheum 1984;27:539-44.
Mantovani Cardoso E, Hundal J, Feterman D, Magaldi J. Concomitant new diagnosis of systemic lupus erythematosus and COVID-19 with possible antiphospholipid syndrome. Just a coincidence? A case report and review of intertwining pathophysiology. Clin Rheumatol 2020;39:2811-5.
Nabavizadeh SH, Farahbakhsh N, Fazel A, Houshmand H, Anushiravani A. Pulmonary embolism in an adolescent girl with negative ACLA systemic lupus erythematosus (SLE): A case report. Electron Physician 2016;8:1874-9.
da Silva AN, Ferreira LD, Monaco CG, Silva CE, Gil MA, Peixoto LB, et al
. Intracardiac thrombosis and mitral prosthesis dysfunction in systemic lupus erythematosus. A case report. Rev Port Cardiol 2003;22:213-9.
Ng AH, Ng DK. A lady with systemic lupus erythematosus complicated with central venous sinus thrombosis: A case report. J Clin Rheumatol Immunol 2020;20:42-6.
Silver AS, Shao CY, Ginzler EM. Aortitis and aortic thrombus in systemic lupus erythematosus. Lupus 2006;15:541-3.
Baidoun F, Issa R, Ali R, Al-Turk B. Acute unilateral blindness from superior ophthalmic vein thrombosis: A rare presentation of nephrotic syndrome from class IV lupus nephritis in the absence of antiphospholipid or anticardiolipin syndrome. Case Rep Hematol 2015;2015:413975. doi: 10.1155/2015/413975.
Asherson RA, Morgan SH, Harris EN, Gharavi AE, Krausz T, Hughes GR. Arterial occlusion causing large bowel infarction--A reflection of clotting diathesis in SLE. Clin Rheumatol 1986;5:102-6.
Sambhav K, Shakir O, Chalam KV. Bilateral isolated concurrent superior ophthalmic vein thrombosis in systemic lupus erythematosus. Int Med Case Rep J 2015; 8:181-3.
Tsugawa K, Tanaka H, Kudo M, Nakahata T, Ito E. Renal artery thrombosis in a pediatric case of systemic lupus erythematosus without antiphospholipid antibodies. Pediatr Nephrol 2005;20:1648-50.
Savelli SL, Roubey RAS, Kitzmiller KJ, Zhou D, Nagaraja HN, Mulvihill E, et al
. Opposite profiles of complement in antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) among patients with antiphospholipid antibodies (aPL). Front Immunol 2019;10:885. doi: 10.3389/fimmu. 2019.00885.
[Figure 1], [Figure 2]