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A 40- year old Finnish man from the capital area was admitted to the hospital emergency because of severe dyspnea following two weeks of bronchitis.
The patient had been previously healthy. He was a smoker but was not diagnosed with any pulmonary disease. He did not have any regular medication.
He had initially symptoms of an upper respiratory tract infection. As the symptoms persisted, bronchitis was suspected and he was prescribed roxithromycin at the health center.
Four days later he was admitted to the hospital emergency because he could hardly walk 100 meters because of severe dyspnea. There was no chest pain. In physical examination, the patient was febrile and his respiratory frequency was 40/min. His blood pressure was 130/78 mmHg, he had sinus tachycardia 144/min, and oxygen saturation was 94% with oxygen mask. In auscultation, rales could be heard from the lungs. ECG is shown in Figure 1. Laboratory analyses demonstrated leucocytosis (23.9 109/l) and high CRP (277 mg/l). During episodes of dyspnea, he became hypotensive and oxygen saturation dropped down to 90% and CPAP treatment was started. Thorax-X-ray is shown in Figure 2.
Clinical diagnosis was pneumonia and sepsis. Intravenous inotropes, cefuroxim and levofloxacin were initiated. However, the patient ‘s condition continued deteriorating twenty four hours after he had arrived at the hospital. Despite noradrenalin infusion, fluid resuscitation, antibiotics and CPAP mask, respiratory difficulties grew and the patient had to be intubated and put to respirator. The next day blood bacterial cultures revealed a pneumococcus resistant to macrolide antibiotics. The patient was still hypotensive, BP dropped to 80/50 mmHg and had sinustachycardia 126/min and CRP remained high (201 mg/l).
Question: What would you have done and why?
A large cardiac silhouette on thorax X-ray and st-elevations in the ECG were suggestive of pericardial affision. Bed-side echocardiography demonstrated a remarkable pericardial effusion, 20-27mm in diastole (Fig. 3). Right atrium collapsed during inspirium. The left ventricular global systolic function was normal, LVEDD was 45mm and left ventricular wall thicknesses 10-11mm. Doppler echocardiography showed marked respiratory variation of the mitral inflow. The clinical findings were compatible with cardiac tamponation.
Pericardiocentesis was done and 350ml of purulent secretion was obtained. After that a drain was inserted. Bacterial staining of the pericardial fluid showed Gram positive cocci and later bacterial culture confirmed that they were pneumococci. The diagnosis was pneumococcal sepsis, pneumonia and purulent pericarditis. The recovery was slow but uneventful. Pericardial drain could not be removed until three weeks later.
Fig. 3 Bed-side echocardiography
Since the patient had been previously healthy, factors predisposing to a fulminant infection were sought. No solid tumors or signs of other malignancies were observed. HIV serology was negative. The main laboratory finding was persistent hypogammaglobulinemia. The serum concentration of IgM was 0,18 g/l (normal range 0,36-2,59g/l), that of IgG was 3,6g/l (normal range 6,8-15g/l) and IgA was 0,17g/l (normal range 0,88-4,84 g/l). The levels of all IgG subclasses were low. The proportion of CD19 B-cells was low and the proportion of CD4/8 T-lymphocytes was 0,4, decreased. After the pneumonic infiltrates had resolved, HRCT (high resolution computed tomography) of the lungs showed still diffuse centrilobular nodularity and mediastinal lymphadenopathy compatible with GLILD, granulomatous lymphocytic interstitial lung disease. Splenomegalia was observed. After further immunological analyzes and missing responses to vaccination, the findings were compatible with a common variable immunodeficiency (CVID). The patient needs lifelong immunoglobulin substitution. Two years and five months after the near fatal infection, the patient is well. The regular medication comprises subcutaneous immunoglobulin substitution and prednisone.
Pericarditis and tamponade
Pericardial tamponade is a medical emergency in which pericardial effusion increases the intrapericardial pressure and leads to hemodynamic collapse. The clinical diagnosis is based on typical presentation (tachycardia, hypotension, marked decrease of systolic pressure during inspirium while diastolic blood pressure remains unchanged) and demonstration of pericardial effusion usually by echocardiography. Immediate pericardial drainage is absolutely indicated. Various infectious or non-infectious agents may cause pericardial effusion. Bacterial pericarditis is a rare and fulminant disease and fatal, if untreated. This condition may be overlooked in a septic patient.
Common variable immunodeficiency (CVID)
CVID is a primary immunodeficiency affecting B-cells. Typically, the serum concentrations of IgG are low and there is a failure to produce antibodies in response to immunization or infection. Also T-cell abnormalities occur. CVID is often complicated by a multisystemic granulomatous disease, usually in the lungs. Splenomegaly and hilar and mediastinal lymphadenopathy are common findings in CVID patients.
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