SYSTEMIC LUPUS ERYTHEMATOSUS PRESENTING WITH MYOCARDITIS AND RECURRENT AND DIFFICULT TO MANAGE ACUTE PULMONARY EDEMA

Ciências da Saúde, Volume 29 – Edição 148/JUL 2025 /

REGISTRO DOI: 10.69849/revistaft/fa10202507252010

Flávio Renan Paula da Costa Alcântara1
Gabriela Cruz de Oliveira Alcântara1
Lucas Braga de Melo1
Rayssa Martins Souza Hass2
Gleide Elane Braga Ferreira3
Bárbara Seabra Carneiro3

SUMMARY

Systemic lupus erythematosus (SLE) is an extremely complex autoimmune disease characterized by severe immune dysregulation. Alterations in several intra- and extracellular signal transduction pathways, as well as signatures, primarily of interferon, culminate in dysfunction of the innate and, primarily, adaptive immune system. The deposition of immune complexes in various organs and tissues is responsible for their functional and structural impairment. Such impairments are the genesis of the diverse and systemic clinical picture that characterizes this pathology, and also make inducing remission a challenging goal. Cardiac involvement, as in other tissues, is diverse and can affect the endocardial and valvular compartment, the epicardial compartment with pericarditis or even coronary artery disease, and, most common of all, the myocardium related to pump dysfunction in severe cases. This is a 28-year-old female patient recently diagnosed with SLE with predominantly cutaneous and joint involvement, without adequate immunosuppressive therapy. She was admitted complaining of progressive dyspnea for a week and transferred to the ICU after developing acute respiratory failure due to APE and requiring invasive mechanical ventilation. She was reintubated twice for new episodes of APE, despite receiving renal replacement therapy (RRT). After treatment with vasoactive drugs (dobutamine), diuretics, methylprednisolone 125 mg/day, and human immunoglobulin 2 g/kg, she was discharged. Acute pulmonary edema is a serious and uncommon complication in patients with SLE. Accurate and timely diagnosis combined with appropriate immunosuppressive therapy can lead to complete resolution of acute myocardial dysfunction, avoiding unfavorable outcomes, including long-term organic sequelae that directly impact patient morbidity and mortality.

Keywords: Systemic lupus erythematosus; myocarditis; acute pulmonary edema;

INTRODUCTION

SLE is a chronic, multisystem autoimmune disease characterized by a complex interplay of genetic, epigenetic, environmental, and hormonal factors, leading to a complex network of immune dysfunction. The disease most frequently affects women, usually between the ages of 16 and 55. 1-3

The diagnosis of SLE is based on clinical and laboratory findings and after excluding other diagnoses, especially infectious etiologies that often mimic it. According to ACR/EULAR 2019, the classification criteria require the presence of a positive antinuclear antibody (ANA) ≥1:80 as an entry criterion. Based on this, the diagnosis is suggested by a minimum score of 10 points, combining clinical criteria distributed across domains such as mucocutaneous, articular, renal, neurological, hematological, and laboratory criteria, including serological. Each criterion has a specific score, and only the highest score in each domain is considered.7-8

This pathology, as mentioned above, has a wide range of manifestations, ranging from mild cases with joint and skin involvement to more complex cases such as renal, hematologic, cardiac, or central nervous system involvement. Cardiac involvement occurs in the form of valvular, pericardial, myocardial, or coronary artery disease.1-3 Acute pulmonary edema (APE) associated with lupus myocarditis is a rare and potentially fatal condition.

CASE REPORT

This is a 28-year-old female patient, recently diagnosed with SLE and primary cutaneous and joint involvement, who developed progressive dyspnea for a week, associated with ventilator-dependent chest pain and dry cough, and was transferred to the intensive care unit (ICU) of a tertiary hospital after acute respiratory failure due to APE and the need for invasive mechanical ventilation. During hospitalization, the diagnosis of SLE was reiterated according to the following criterias: homogeneous nuclear ANA 1:160, arthritis, subacute skin lesions, serositis, decreased C3 and C4, and proteinuria >500mg in 24 hours. On physical examination, the patient was in poor general condition, with tachycardia, hypertension, anasarca, bilateral decreased breath sounds without adventitious sounds, cardiac auscultation without alterations, globular abdomen and no visceromegaly. Echocardiogram showing ejection fraction of 34%, increased left cavities, with eccentric left ventricular hypertrophy (LVH), bilateral pleural effusion and mild pericardial effusion. Chest tomography showed consolidative opacities associated with air bronchograms with adjacent ground-glass attenuation, scattered throughout the lungs, notably in the lower lobes; mild bilateral pleural and pericardial effusion, in addition to a high cardiothoracic ratio. Viral serologies, blood cultures and urine cultures were negative. She was hospitalized in the ICU for 19 days, being intubated a total of three times due to PEA, despite receiving renal replacement therapy (RRT). After treatment with dobutamine, diuretic therapy, methylprednisolone 125 mg/day, and human immunoglobulin 2 g/kg for 5 days, she was discharged to the ward with clinical improvement. Immunosuppression with methylprednisolone was not performed initially due to the presentation of a positive IGRA and due to the severity and rapidity of the cardiac dysfunction, it was decided to perform an immunoglobulin infusion and, 30 days after the institution of treatment for tuberculosis infection, the patient was treated with the National Institutes of Helth (NIH) protocol for remission induction with cyclophosphamide.

Image 1: Chest X-ray at the time of diagnosis of APE, showing a significantly enlarged cardiac silhouette, in addition to diffuse infiltration of the lung parenchyma. Imagem 2: Chest X-ray after discharge from the ICU, showing improvement in pulmonary congestion. Imagem 3: Chest X-ray 6 months after admission, showing a significant reduction in cardiac silhouette.

DISCUSSION

SLE can manifest with cardiac involvement in 30% to 89% of patients. Its pathogenesis is not fully understood; however, the formation and deposition of immune complexes and complement activation are mechanisms that may be involved. Clinical cardiac repercussions in SLE can occur in various forms, including pericarditis, myocarditis, Libman-Sacks endocarditis, pulmonary arterial hypertension, and coronary artery disease. Coronary artery disease associated with accelerated atherosclerosis is the form most associated with long-term deficits. These deficits result from the chronic inflammatory process and corticosteroid use. Lupus myocarditis, although more common, is usually asymptomatic, with the symptomatic form being described in only 5% to 10% of cases. This condition is therefore severely underreported. Although rare, there are reports of cases of severe acute cardiac dysfunction as the initial manifestation of the disease. 1,3-6. Acute pulmonary edema associated with myocarditis is a serious and rare clinical condition, whose favorable outcome depends on early recognition and appropriate treatment. Although there is little information in the literature on protocols for the management of patients with this condition in the context of SLE, the immunosuppressive therapeutic approach combined with conventional heart failure treatment has shown positive results, as illustrated in the clinical case presented. 4,5

CONCLUSION

Acute pulmonary edema is a serious and uncommon complication in patients with SLE, but it becomes more prevalent in patients with lupus myocarditis. Accurate and timely diagnosis, along with appropriate immunosuppressive therapy, aims to achieve clinical resolution of myocardial dysfunction and minimize adverse outcomes.

REFERENCES
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  7. Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1982; 25: 1271–7
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1Resident of Hospital Universitário Getúlio Vargas – UFAM;

2Resident of Rheumatology do Hospital Universitário Getúlio Vargas – UFAM

3Preceptor of Hospital Universitário Getúlio Vargas – UFAM

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