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.

Validation of salivary cortisol among Filipino adults suspected with Cushing's syndrome

BACKGROUND: Cushing's syndrome is a state of hypercortisolism manifesting non-specific clinical; features where its diagnosis entails biochemical confirmation of cortisol excess. this study aims to validate the efficacy of midnight salivary cortisol as a screening test for Filipino suspected with Cushing's syndrome and determine the cut-off value applicable in the local setting. METHODS: This is a cross-sectional study of Filipinos suspected with endogenous Cushing's syndrome seen at a tertiary hospital. Modification of plasma cortisol measured by RIA was used to measure salivary cortisol. The sensitivity, specificity, positive predictive curve, negative predictive curve and area under the screening tests were estimated and compared using 48 hour low dose dexamethasone suppression test (LDDST) as the reference standard. RESULTS: The determine cut-off value (? 7.0 nmol/L) for salivary cortisol showed a relatively high sensitivity (91.3%) and specificity (89.5%) in detecting cases suspected of Cushing's syndrome. One milligram (1mg) dexamethasone suppression test had the highest sensitivity (100%) but had the lowest specificity (68.4%) as a screening test. The area under the curve of the three diagnostic test appeared to be similar when compared with the low dose dexamethasone suppression test. CONCLUSIONS: Using a cut-off value of 7nmol/L, local utility if late-night salivary cortisol has a high sensitivity and specificity in detecting Cushing's syndrome. It has a similar efficiency with 24-hour urine free cortisol and 1mg dexamethasone suppression test as a screening test for Cushing's syndrome. Salivary cortisol may be considered as a valid initial screening test for Filipinos suspected of cushing's syndrome.

Cortisol secreting adrenal adenoma in a patient with schizophrenia, rheumatic heart disease and myoma uteri: Difficult to find but easy to diagnose

The presence of cortisol - secret ing adenoma concomitantly with rheumatic heart disease, schizophrenia and myoma uteri is rare. This is a case of a 40 year old female with Schizophrenia who gradually developed Cushing's syndrome from an adrenal adenoma. She suffered a cardio-embolic stroke from Rheumatic heart disease which delayed hysterectomy for a bleeding intrauterine myoma. As ide f rom the phys ical f indings of Cushing's syndrome laboratory work up revealed an elevated 24 hour urine free cortisol with loss of normal diurnal cortisol secretion, suppressed 8AM ACTH level and negative suppression after a high dose dexamethasone. The patient underwent laparoscopic adrenalectomy for a 3.8 x 2.4 x 3 cm left adrenocortical adenoma. She required steroid supplementation. Menstrual flow immediately normalized, functional capacity improved and metabolic parameters such as weight, blood pressure and blood sugar were controlled six months after the surgery. Relapse of psychotic symptoms occurred eight months postoperatively because of non-compliance to antipsychotic medications. Cushing's syndrome if untreated can cause significant morbidities such as metabolic, hemodynamic, cardiovascular, bleeding disorder and psychiatric illness. These complications however can also be caused by primary medical illnesses like schizophrenia, rheumatic heart disease and myoma uteri. Treatment of the Cushing's syndrome may resolve some but not all the metabolic and hemodynamic problems and theoretically should also decrease the risk of complications of other primary illnesses concomitantly present. The presence of concomitant primary disease that can cause psychosis, cerebrovascular disease and metrorrhagia should also be investigated in a patient who has Cushing's syndrome. Prompt management of Cushing's syndrome would lessen the risk of complication attributed to schizophrenia, rheumatic heart disease and myoma uteri.