Cardiac tamponade as a rare manifestation of systemic lupus erythematosus: A report on four cases in the Philippine General Hospital

SYNOPSIS: Cardiac tamponade among systemic lupus erythematosus (SLE) patients is an unusual event. The pericardial effusion may be a consequence of uremia, infections in the pericardium, or the lupus pericarditis itself. We present four atypical cases of cardiac tamponade from pericarditis of connective tissue disease (CTD), all of which were treated with drainage and immunosuppressants. Due to the rarity of this combination, management was a challenge.CLINICAL PRESENTATION: Four females each sought consult for dyspnea associated with typical manifestations of connective tissue disease such as arthritis, characteristic rashes, serositis, typical laboratory features, and a positive ANA and/or anti-dsDNA. The first three cases fulfilled the criteria for SLE, while the fourth fulfilled the criteria for SLE-dermatomyositis overlap syndrome. Echocardiography was done due to suspicion of pericardial involvement and revealed massive pericardial effusion in tamponade physiology in all cases.DIAGNOSIS: Cardiac tamponade from serositis due to connective tissue disease [SLE (case 1 to 3) or SLE-dermatomyositis overlap (case 4). Other common etiologies of tamponade such as bacterial, tuberculous, malignant, and uremic pericardial effusion were ruled out by clinical and laboratory tools, including Gram stain and culture, cytology, PCR, and biochemical testing. The pericardial fluid of the first case tested positive for lupus erythematosus (LE) cells, indicative of lupus serositis.TREATMENT AND OUTCOME: All patients underwent pericardial drainage via tube pericardiostomy. They received high dose glucocorticoids after infectious etiologies for the pericardial effusion were ruled out. The fourth case with the overlap syndrome, however, required more immunosuppressants using azathioprine and methotrexate. Resolution of pericardial effusion was noted with this approach. Three of four were discharged improved, however, the third case suffered from worsening nephritis and pulmonary hemorrhage leading to her demise.SIGNIFICANCE AND RECOMMENDATIONS: Four cases of cardiac tamponade as a manifestation of connective tissue disease were presented. Literature underlines the rarity of this condition anytime during the course of SLE. Despite this, SLE should be considered as one of the differential diagnosis of cardiac tamponade, especially in patients who manifest with multi-systemic findings. Likewise, massive pericardial effusion should be considered in patients with a connective tissue disease presenting with subtle evidence of pericardial involvement. It requires timely identification and treatment with high dose steroids, after other causes such as infections have been excluded. Immediate drainage through pericardiocentesis or pericardiostomy in combination with immunosuppressants may be life-saving.

A case of Churg-Strauss syndrome presenting with foot drop

BACKGROUND: Churg-Strauss syndrome (CSS), or eosinophilic granulomatosis with polyangiitis, is a rare syndrome that affects small- to medium-sized arteries and veins. Criteria for the diagnosis include: asthma (wheezing, expiratory rhonchi), eosinophilia of more than 10% in peripheral blood, paranasal sinusitis, pulmonary infiltrates (may be transient), histological proof of vasculitis with extravascular eosinophils, and mononeuritis multiplex or polyneuropathy. The worldwide incidence of CSS is approximately 2.5 cases per 100,000 adults per year and its incidence in the United States is one to three cases per 100,000 adults per year.1 In the Philippines, the exact incidence is unknown with very few published case reports about it.SETTING: University of the Philippines-Philippine General Hospital (UP-PGH), a tertiary training hospital in Manila, PhilippinesTHE CASE: A 40-year-old Filipino male with a history of adult onset asthma and recurrent sinusitis manifesting with inability to dorsiflex the left ankle (foot drop), various dermatologic lesions, and arthralgia. Complete blood count showed hypereosinophilia. Electromyography revealed asymmetric moderate to severe sensory and motor denervation of limbs compatible with polyneuropathy. Skin biopsy revealed lymphocytic vasculitis. P-ANCA was positive. During his incumbent hospitalization, the skin lesions, arthralgia and neurologic manifestations improved on administration of high dose steroids. Pregabalin was used to control pain secondary to the mononeuritis multiplex.SIGNIFICANCE: To report a rare case of ChurgStrauss syndrome presenting as foot drop. This case highlights the importance of considering ChurgStrauss syndrome among adult patients presenting with neurologic complaint (inability to dorsiflex the left ankle/foot drop) and various dermatologic lesions.