|Year : 2021 | Volume
| Issue : 2 | Page : 82-85
Recurrent cardiac tamponade with stevens–Johnson's syndrome – A rare deadly combo
Iragavarapu Tammiraju1, B Srinivas2, K Nirupama3, K Pruthvi Naresh2
1 Department of Cardiology, ASRAM Medical College, Eluru, Andhra Pradesh, India
2 Department of General Medicine, ASRAM Medical College, Eluru, Andhra Pradesh, India
3 Department of Dermatology, ASRAM Medical College, Eluru, Andhra Pradesh, India
|Date of Submission||04-Apr-2020|
|Date of Decision||22-May-2020|
|Date of Acceptance||31-May-2020|
|Date of Web Publication||03-May-2021|
Dr. Iragavarapu Tammiraju
Associate Professor, Department of Cardiology, Asram Medical College, Eluru - 534 005, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Pericardial effusion is an accumulation of fluid in the pericardial sac. It is caused by exudative or transudative collections. Large effusions can lead to a life-threatening condition called tamponade if not intervened properly. Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis are life-threatening cutaneous drug reactions in which the epidermis is separated from the dermis. The diagnosis mainly relies on the clinical signs and histopathology of skin lesions. Immunological mechanisms, reactive drug metabolites, or interactions between these two are proposed. Here, we report a case of recurrent cardiac tamponade with SJS secondary to empirical antituberculous therapy.
Keywords: Empirical drugs, recurrent effusion, Stevens–Johnson
|How to cite this article:|
Tammiraju I, Srinivas B, Nirupama K, Naresh K P. Recurrent cardiac tamponade with stevens–Johnson's syndrome – A rare deadly combo. J Indian coll cardiol 2021;11:82-5
|How to cite this URL:|
Tammiraju I, Srinivas B, Nirupama K, Naresh K P. Recurrent cardiac tamponade with stevens–Johnson's syndrome – A rare deadly combo. J Indian coll cardiol [serial online] 2021 [cited 2021 Jun 13];11:82-5. Available from: https://www.joicc.org/text.asp?2021/11/2/89/315260
| Introduction|| |
Pericardial effusion is an accumulation of fluid in the pericardial sac. It is caused by exudative or transudative collections. Large effusions can lead to a life-threatening condition called tamponade if not intervened properly. Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening cutaneous drug reactions in which the epidermis is separated from the dermis. The diagnosis mainly relies on the clinical signs and histopathology of skin lesions. Immunological mechanisms, reactive drug metabolites, or interactions between these two are proposed.
| Case Report|| |
Here, we report the case of a 55-year-old female, who is a known hypertensive, hypothyroidism came with complaint of shortness of breath, bilateral pedal edema, and cough associated with fever. She is diagnosed to have pericardial effusion. Despite diuretic use, pericardial effusion remained, and pericardiocentesis was done outside, and the patient was started on empirical antitubercular treatment. Two weeks after starting antitubercular medication, the patient developed multiple macular erythematous lesions over the face [Figure 1]a, proximal upper limbs, and anterior and posterior trunk [Figure 1]b with oral ulcers and dyspnea. The patient presented to us in this situation. On examination, she had pulsus paradoxus. Electrocardiogram showed electrical alternans. Repeat echocardiogram showed large pericardial effusion with tamponade [Figure 2]. Repeat pericardiocentesis was done. Investigative profile revealed the presence of antinuclear antibodies and autoimmune etiology. Dermatologist consultation was taken for skin lesions, and skin biopsy was done, which confirmed SJS secondary to antitubercular treatment. Hence, antituberculous therapy (ATT) was stopped. The patient was started on steroids for SJS and autoimmune pericarditis, and the lesions disappeared slowly [Figure 3]. One month after the second pericardiocentesis, when repeat echocardiogram was done, it still showed mild pericardial effusion without tamponade [Figure 4]. Repeat albumin was 3.0 g/dl, and after the correction of hypoalbuminemia, pericardial effusion was decreased. The investigative profile of the patient was mentioned in [Table 1].
|Figure 1: (a) Multiple erythematous lesions on the face of the patient suggestive of Stevens–Johnson syndrome. (b) Multiple erythematous lesions on the back of the patient suggestive of Stevens–Johnson syndrome|
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|Figure 2: Subcostal view showing pericardial effusion (white arrow) with tamponade|
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|Figure 4: Subcostal view showing minimal pericardial effusion (white arrow) after pericardiocentesis|
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| Discussion|| |
Pericardial effusion is an accumulation of fluid in the pericardial sac. It can be due to varied etiology (infectious, neoplastic, autoimmune, metabolic, and drug-related). Many cases still remain idiopathic in developed countries (up to 50%), whereas other common causes include cancer (10%–25%), pericarditis, infectious causes (15%–30%), iatrogenic causes (15%–20%), and connective tissue diseases (5%–15%). Tuberculosis (TB) is the dominant cause in developing countries (60%), where TB is endemic.
Pericardial effusion may be classified based on its onset (acute, subacute, and chronic >3 months), distribution (circumferential/loculated), hemodynamic factors (cardiac tamponade and effusive-constrictive), composition (exudates and transudate), and especially by its size as mild, moderate, and large (>20 mm) based on a simple echocardiographic assessment. Hydropericardium, a noninflammatory transudative pericardial effusion, may occur not only in heart failure but also in advanced hypoalbuminemia, such as in cirrhosis and nephrotic, when Starling forces promote the accumulation of plasma ultrafiltrate across the pericardium as well as other membranes (e.g., pleura and peritoneum). There is a case report of pericardial effusion with hypothyroidism in Down's syndrome patient. In our case, the recurrent effusion can be due to hypoalbuminemia and hypothyroidism.
The diagnosis of cardiac tamponade is essentially a clinical diagnosis requiring echocardiographic confirmation of the initial diagnostic suspicion. In most patients, cardiac tamponade should be diagnosed by a clinical examination that shows elevated systemic venous pressure, tachycardia, dyspnea, and paradoxical arterial pulse. The management should be tailored to the single patient according to his/her preferences and evolution. The management depends on the hemodynamic impact, size, presence of inflammation (i.e., pericarditis), associated medical conditions, and the etiology whenever possible. Recurrences are also common, and pericardiectomy or less invasive options (i.e., pericardial window) should be considered with recurrent cardiac tamponade or symptomatic pericardial effusion (either circumferential or loculated). Pericardiocentesis is the first line of management, and pericardiectomy is generally the last option to be carefully considered and offered in well-experienced surgical centers since it is a long and demanding cardiac surgery operation. A pericardial window is often an alternative option to be considered; anti-inflammatory therapies, including colchicine, may be not efficacious in the absence of inflammation and pericarditis. In our case, the first episode of pericardial effusion could be autoimmune or hypothyroidism, and recurrent pericardial effusion is due to hypoalbuminemia and hypothyroidism aggravating the underlying autoimmune pericarditis.
Relatively, 5.3% of hospital admissions were associated with adverse drug reactions (ADRs). The maculopapular eruption is a common adverse cutaneous drug reaction. SJS and TEN are life-threatening cutaneous drug reactions in which the epidermis is separated from the dermis. In SJS, there is <10% body surface area involvement whereas in TEN more than 30% and 10%–30% overlap cases have been reported. SJS is an immune complex-mediated hypersensitivity complex that typically involves the skin and the mucous membranes. It is associated with fever, inflammation of the buccal mucosa, and severe purulent conjunctivitis. The incidence of this disorder is unknown, but it is thought to be very low. The etiology is multiple, including drugs, infectious agents, and idiopathic causes. Immunological mechanisms, reactive drug metabolites, or interactions between these two are proposed. Interactions between CD95 L and Fas (CD 95) are directly involved in the epidermal necrolysis., Granulysin is also considered as a key mediator for disseminated keratinocyte death in SJS/TEN. The diagnosis of SJS is considered in patients with the following clinical features: drug exposure 1–4 weeks before the symptom onset; acute onset febrile illness and malaise; erythematous macules, targetoid lesions, or diffuse erythema; and necrosis of the epidermis.
Cutaneous ADRs (CADRs) are one of the commonly observed significant adverse effects of the first-line antitubercular therapy and are reported in 5.7% of tubercular patients. CADR associated with antitubercular treatment includes morbilliform rash, erythema multiforme syndrome, urticaria, lichenoid eruption, and other more serious ones such as SJ syndrome and exfoliative dermatitis. SJ syndrome is a rare but potentially fatal complication of antitubercular therapy. In a review of 9111 hospitalized patients of pulmonary TB, 25 or 0.27% developed SJ syndrome. Rifampicin® is widely used in the treatment of TB and has been associated with a variety of adverse effects. A relatively uncommon side effect is cutaneous vasculitis. The development of urticaria or exanthem within 1–2 h of an initial pyrazinamide dose has been reported, and pyrazinamide-induced erythema multiform was confirmed by rechallenge in another study. In a case report, erythema multiform has been reported in one patient following pyrazinamide administration for cutaneous TB related to a pleural fistula. Given the association between HIV infection and a hypersensitivity reaction, all patients who developed SJS should screen for HIV. The mortality rate mainly depends on the age and health of the patient, and rates can range from 30% to 100%. Death is commonly due to infectious complications. Moreover, since the use of drug is the most important cause, the identification and removal of the causative medication is of paramount importance to halt the progression of the conditions and to prevent a recurrence. In our case, the patient developed these symptoms after starting empirical ATT, and among ATT drugs, rifampicin and pyrazinamide are known to cause SJS.
| Conclusion|| |
SJS and aggravated constrictive pericarditis are possible adverse events of anti-TB therapy. Till date, no treatment has been identified to be capable of halting the progression of skin detachment. The combination of recurrent pericardial effusion and SJS is very rare and has a cause-and-effect relationship. Starting of empirical ATT in a case of pericardiocentesis is not 100% safe. Proper evaluation and planning of management is required in these rare circumstances. Supportive management is crucial to improve the patient's condition, probably more than specific immunomodulating treatments. Despite all therapeutic efforts, mortality is high and increases with disease severity, patients' age, and underlying medical conditions.
Pericardial effusion in a patient with SJS is rare. Hence, we report this case.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]