Cardiac Tamponade as the Initial Presentation of Coexistent Systemic Lupus Erythematosus and Addison’s Disease: A Case Report and Literature Review

Abstract

Cardiac tamponade is a rare presentation of systemic lupus erythematosus (SLE), occurring in less than 1% of cases. Its coexistence with primary adrenal insufficiency (Addison’s disease) represents an even rarer clinical scenario. We present the case of a 28-year-old female who presented with cardiac tamponade as the initial presentation of concurrent SLE and Addison’s disease. Laboratory investigations revealed positive antinuclear and anti-dsDNA antibodies, low complement levels, hyponatremia, and hyperkalemia. Adrenal function tests confirmed primary adrenal insufficiency. Echocardiography identified a large pericardial effusion with cardiac tamponade, necessitating emergent pericardiocentesis. The patient responded well to high-dose glucocorticoids, hydroxychloroquine, and mineralocorticoid replacement therapy. This case highlights the rare and challenging presentation of cardiac tamponade as the initial presentation of the coexistent SLE and Addison’s disease. Prompt recognition and management of this complex scenario are crucial. Clinicians should maintain a high index of suspicion for underlying autoimmune disorders in patients presenting with cardiac tamponade.

Background

Cardiac tamponade is a life-threatening condition characterized by the accumulation of fluid in the pericardial space, exerting pressure on the heart, and impeding its normal function. Although cardiac tamponade can have multiple causes, it rarely manifests as an initial presentation in systemic autoimmune diseases such as systemic lupus erythematosus (SLE) and primary adrenal insufficiency (PAI) or Addison’s disease.

SLE is a chronic, multisystem autoimmune inflammatory disorder with a strong female predominance, characterized by a highly variable clinical presentation affecting virtually every organ system. Initial symptoms often include arthralgia, myalgia, and frank arthritis, particularly involving the smaller joints of the hands, wrists, and knees. Classic features also include a malar rash, renal impairment, neurological manifestations such as seizures, and general malaise. Cardiac involvement is common, affecting more than 50% of patients.^1,2 Although pericarditis and pericardial effusion are the most common cardiac manifestations, cardiac tamponade, especially as the initial presentation of the disease, is extremely uncommon.^3,4 Cardiac tamponade is a rare and potentially lethal complication, estimated to occur in less than 1% of patients with SLE. ⁵

Primary adrenal insufficiency is most commonly caused by autoimmune processes, either as isolated autoimmune adrenalitis or as part of an autoimmune polyglandular syndrome. It is characterized by a low cortisol and aldosterone production rate, and a high plasma ACTH concentration that clinically manifests as hyperpigmentation. ⁶ Simultaneous occurrence of SLE and primary adrenal insufficiency in the presentation of cardiac tamponade is a rare clinical scenario that poses significant diagnostic and therapeutic challenges.

In this report, we present a unique and rare clinical scenario in which cardiac tamponade serves as the harbinger for concurrent diagnosis of SLE and PAI. This case report aims to shed light on this rare presentation, discuss the diagnostic challenges encountered, explore the interplay between SLE and Addison’s disease in the context of cardiac tamponade, and review relevant literature to provide insights into the management and prognosis of such complex cases. Through this discussion, we hope to contribute to the body of knowledge on the rare coexistence of these conditions and highlight the importance of considering a broad differential diagnosis in patients presenting with cardiac tamponade.

Case Presentation

A 28-year-old female presented to the emergency department with a 2-day history of sharp pleuritic chest pain, exacerbated by deep breaths and lying down, associated with shortness of breath. The patient also reported experiencing generalized fatigue, vomiting, significant unintentional weight loss, and episodes of skin darkening over the previous few months. She denied any recent infections, trauma, or family history of autoimmune diseases. The patient had a medical history of intermittent joint pain, morning stiffness, nonscarring alopecia, serositis, and anemia. She had complications during her third trimester of pregnancy in 2022, including premature rupture of membranes and preeclampsia

On examination, the patient appeared pale and exhibited signs of respiratory distress, characterized by dyspnea. Vital signs revealed hypotension with a blood pressure of 90/60 mmHg, tachycardia with a heart rate of 110 beats per minute, a respiratory rate of 22 breaths per minute, and oxygen saturation of 92% on room air. Cardiovascular examination showed distant heart sounds without murmurs, rubs, or gallops. Cutaneous findings included hyperpigmentation on the face, hands, and elbows. The musculoskeletal assessment revealed no joint swelling, deformities, or rashes.

Laboratory tests revealed hyponatremia, hyperkalemia, and anemia. Autoimmune screening showed positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA) antibodies, and low complement levels as shown in Table 1, suggestive of systemic lupus erythematosus (SLE). Adrenal function tests indicated elevated adrenocorticotropic hormone (ACTH) levels with low early morning cortisol levels, consistent with primary adrenal insufficiency (Addison’s disease). Echocardiography and x-ray revealed a large pericardial effusion with evidence of cardiac tamponade as shown in Figure 1, characterized by right atrial and right ventricular diastolic collapse. Immediate pericardiocentesis was performed to relieve tamponade and stabilize the patient’s hemodynamics. Analysis of the pericardial fluid obtained during the procedure revealed low sugar levels, elevated LDH levels, and a normal white blood cell count. Cytological examination of the fluid was negative for malignant cells, ruling out malignancy as the cause of effusion.

Table 1.

Patient’s Laboratory Values.

Test	Value	Reference
RBC	4.44	4.1 – 5.5 K/ul
HGB	10.8	12 – 16 g/dL
HCT	34.1	37 – 48 %
MCV	76.8	80 – 100 fL
WBC	4.28	4 – 11 K/ul
Lymphocytes	1.23	1 – 4.8 K/ul
Neutrophils	2.66	1.8 – 7.7 K/ul
Platelets	199	140 – 450 K/ul
aPTT	49.8	25 – 35 s
PT	17.2	11 – 15 s
INR	1.23	0.85 – 1.2
Urea	11.43	6 – 20 mg/dL
Creatinine	0.56	0.5 – 0.9 mg/dL
Sodium	134.8	135 – 145 mmol/L
Potassium	3.5	3.5 – 5 mmol/L
Calcium	7.94	8.6 – 10 mmol/L
CRP	59.5	0 – 5 mg/dL
ESR	120	0 – 10 mg/dL
Cortisol	2	4 – 22 ug/dL
ACTH	250	6 – 50 pg/dL
C3 complement	69	90 – 180 mg/dL
C4 complement	11.2	10 – 40 mg/dL
ANA	1:1280	U/ml
Anti-ds-DNA	23	>18 U/ml positive
Anti-B2-GP1 IgM	37.2	>18 U/ml positive
Anti-B2-GP1 IgG	7.34	>18 U/ml positive
aCL IgM	18	>18 U/ml positive
aCL IgG	14	>18 U/ml positive

RBC, Red Blood Cells; HGB, Hemoglobin; HCT, Hematocrit; MCV, Mean Corpuscular Volume; WBC, White Blood Cells; PT, Prothrombin Time; INR, International Normalized Ratio; CRP, C-Reactive Protein; ESR, Erythrocyte Sedimentation Rate; ACTH, Adrenocorticotropic Hormone; ANA, Antinuclear Antibody.

Figure 1.

Chest X-ray showing a large pericardial effusion.

The patient was admitted to the intensive care unit (ICU) for close monitoring. She was started on high-dose intravenous corticosteroids for both SLE and Addison’s disease along with hydroxychloroquine for SLE management. The initiation of fludrocortisone treatment for Addison’s disease was also undertaken to address mineralocorticoid deficiency.

Using the European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) 2019 criteria for Systemic Lupus Erythematosus (SLE), the diagnosis was confirmed based on an entry criterion of a positive antinuclear antibody (ANA) titer >1:80. The patient achieved a total score of 18, exceeding the diagnostic threshold of 10. The breakdown included non-scarring alopecia (2 points), pericardial effusion (5 points), low complement (C3) levels (3 points), positive anti-double-stranded DNA antibodies (6 points), and anti-beta-2 glycoprotein antibodies (2 points).

During the patient’s hospital stay, her condition stabilized with the initiation of targeted treatment. She was closely monitored for any signs of disease flare-ups or complications. Upon discharge, the patient was instructed to continue the prescribed medications, including corticosteroids and hydroxychloroquine, and to follow up regularly with her rheumatologist and endocrinologist. The importance of adherence to the treatment regimen, lifestyle modifications, and prompt reporting of any new symptoms were emphasized to the patient. Regular monitoring of disease activity, treatment response, and potential side effects were planned as part of the long-term management strategy.

Discussion

Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder that can result in various cardiovascular manifestations, including valvular heart disease, myocarditis, pericarditis, and conduction defects. Pericardial effusion occurs in approximately 50% of SLE patients, while cardiac tamponade is exceedingly rare. ⁷ In a cohort of 395 patients with adult-onset SLE, cardiac tamponade was reported in only 10 cases, with four presenting as the initial manifestation, highlighting its rarity. ⁸

Cardiac tamponade in SLE patients has been associated with female gender, anemia, renal involvement, pleuritis, elevated ESR, and decreased C4 levels. ⁹ In our patient, several of these risk factors were present: female gender, anemia, and elevated ESR.

PAI typically manifests with cardiovascular symptoms such as hypotension, syncope, and arrhythmias, with cardiac tamponade being a rare presentation. ¹⁰

The pathogenesis of tamponade in SLE and PAI likely involves autoimmune-mediated pericardial inflammation, leading to excessive fluid accumulation. The coexistence of SLE and PAI is rare and diagnostically challenging. Both autoimmune diseases predispose patients to pericardial effusions, highlighting the importance of autoimmune screening and imaging.^2,11 In our case, both conditions likely contributed to the development of cardiac tamponade

The pathogenesis of cardiac tamponade in autoimmune PAI appears to be primarily due to autoimmune-mediated pericardial inflammation. ¹² Most cases reported in the literature have been observed in patients diagnosed with autoimmune polyglandular syndrome type II (APS 2), a rare endocrine disorder characterized by PAI, type 1 diabetes mellitus, and/or autoimmune primary hypothyroidism. Therefore, in cases of pericardial effusions, it is important to investigate thyroid function tests to identify the presence of hypothyroidism or to consider APS 2 as a potential underlying cause.10 Adrenal insufficiency further compounds this by impairing the body’s stress response due to glucocorticoid deficiency, leading to decreased vascular resistance and reduced cardiac stroke volume. Additionally, aldosterone deficiency causes intravascular volume depletion and lowers intracardiac filling pressures, increasing the risk of right atrial and ventricular collapse even with minimal pericardial effusion.^11-16

Cardiac tamponade is a critical condition that arises from the accumulation of fluid within the pericardial space and ultimately compromises cardiac function.1 This results in a reduction in right ventricular filling and left ventricular cardiac output, consequently resulting in hypotension, potentially culminating in cardiac arrest. Pericardial effusion typically develops as a result of progressive accumulation of fluid around the pericardium, which can be caused by various infectious and non-infectious factors. Infections can be triggered by various viral, bacterial, fungal, and parasitic agents. Viral pericarditis is generally considered a mild condition that can be managed with symptomatic treatment using nonsteroidal antiinflammatory drugs. However, in some cases, effusion can become large and result in cardiac tamponade, necessitating pericardiocentesis. On the other hand, bacterial agents can cause purulent pericarditis through direct bacterial invasion, whereas non-purulent effusion can occur as a result of immune-mediated mechanisms, such as autoimmune diseases like SLE.^2,17

Diagnosing cardiac tamponade in the context of SLE and PAI can be challenging because of the nonspecific nature of the symptoms and the rarity of this presentation. However, prompt recognition and treatment are crucial for preventing hemodynamic compromise and potentially fatal outcomes. Cardiac tamponade typically presents with symptoms, such as dyspnea, chest pain, and hemodynamic instability. Physical examination may reveal signs of Beck’s triad, consisting of hypotension, elevated jugular venous pressure, and muffled heart sounds. In addition, pulsus paradoxus, which is a decrease in systolic blood pressure during inspiration, may be observed. Echocardiography is the diagnostic modality of choice for evaluating pericardial effusion and confirming a diagnosis of cardiac tamponade. It allows for the visualization of pericardial effusion, assessment of its size and hemodynamic consequences, and guidance for pericardiocentesis if needed. ¹⁸

In cases like ours, where an underlying medical condition is initially unclear, a patient presenting with chest pain and shortness of breath requires careful consideration of a wide range of differential diagnoses, a comprehensive approach that includes a detailed medical history, thorough physical examination, and targeted laboratory investigations is essential to refine the differential and pinpoint the underlying cause. Autoimmune screening and imaging studies were performed to elucidate underlying conditions. Analysis of the pericardial fluid indicated exudative effusion with potential infective, inflammatory, or malignant origins. However, microbiological and cytological results ruled out infectious or malignant effusion. To exclude the presence of malignancy, a breast examination and subsequent CT scan were performed. The diagnosis of SLE was supported by positive anti-dsDNA and antinuclear antibody titers in addition to other factors, which are key criteria according to the American College of Rheumatology guidelines. In this case, the patient fulfilled four of the 11 criteria for a definite diagnosis of SLE.

The management of cardiac tamponade involves supportive measures and definitive treatment. Supportive measures include maintaining hemodynamic stability through fluid resuscitation and the use of vasopressors if necessary. However, the definitive treatment for cardiac tamponade is pericardiocentesis, which involves removal of accumulated fluid from the pericardial space. Pericardiocentesis can be performed using a subxiphoid or parasternal approach, under echocardiographic or fluoroscopic guidance. In cases where pericardiocentesis is not feasible or there is recurrent accumulation of fluid, a surgical procedure called a pericardial window may be performed to create a permanent communication between the pericardial space and the pleural cavity, allowing for continuous drainage of the fluid. ¹⁹

In the context of SLE, the treatment of cardiac tamponade involves addressing the underlying autoimmune diseases. Glucocorticoids and immunosuppressive agents, such as cyclophosphamide or mycophenolate mofetil, may be prescribed to control the inflammatory response and prevent further cardiac involvement. ²⁰ In cases of primary adrenal insufficiency, hormone replacement therapy with glucocorticoids and mineralocorticoids is essential to restore normal adrenal function and to prevent adrenal crisis. Management of these conditions requires a multidisciplinary approach involving rheumatologists, endocrinologists, and cardiologists to optimize treatment strategies and ensure comprehensive care. ¹⁰

Conclusion

In conclusion, this case highlights the rare and challenging coexistence of systemic lupus erythematosus (SLE) and primary adrenal insufficiency (PAI) presenting as cardiac tamponade. The autoimmune processes in both conditions likely contributed to the development of pericardial effusion, emphasizing the need for heightened clinical awareness when diagnosing and managing such cases. Early recognition, prompt intervention, and a multidisciplinary approach are essential to optimize patient outcomes. This case underscores the importance of considering autoimmune etiologies in pericardial diseases and provides a basis for further research into this uncommon clinical scenario.