A Sluggish Mannose-binding protein-associated serine protease's Way To Be Successful

Visual acuity was decreased to finger-counting at 4?ft distance. Deep tendon reflexes were bilaterally decreased and stiffness of the small joints of the hands with inability to make a full fist noted. Cardiac auscultation revealed normal S1 and S2 with an early diastolic pericardial knock but no rub or murmur. There was mild jugular venous distension but no Kussmaul's sign or pulsus paradoxus were noted. He was found not to have any other significant abnormal findings on chest auscultation. There were no palpable visceromegaly, no signs of fluid overload, no lymphadenopathy, no focal neurological deficits or signs of meningeal irritation. Initial laboratory data on admission to ER revealed severe thrombocytopenia (platelet count of 25?000/��L), microcytic, hypochromic anaemia and acute renal failure. Initial chest X-ray (CXR) showed cardiomegaly. CT head was unremarkable. Electrocardiogram showed a normal sinus rhythm, normal voltage with no electrical alternans or evidence of ischaemia (figure 1). Figure?1 Initial electrocardiogram with normal sinus rhythm, normal voltage with no electrical alternans or evidence of ischaemia. Considering his MG132 clinical presentation and initial laboratory findings suggestive of the possibility of an immune-mediated disease process, basic autoimmune work up was sent (table 1). He was started on steroids immediately on the same day of admission. On day 2 as there was no clinical improvement; haematology and rheumatology services were consulted. Peripheral blood smear showed evidence of microangiopathic haemolytic anaemia with 1+ schistocytes. Despite considering a SLE, CREST syndrome and Scleroderma as main differential diagnosis, the possibility of thrombotic thrombocytopenic purpura (TTP) could not be ruled out. Subsequently, the patient was started on IVIG and plasmapharesis later on day 2. Table?1 Laboratory values Also on day 2 of hospitalisation, two-dimensional (2D) echocardiogram (requested originally due to evidence of cardiomegaly on CXR, diastolic click and elevated JVD) revealed 3�C4?cm of pericardial effusion with echocardiographic evidence of late diastolic right ventricular collapse and early pericardial tamponade (video 1). As the patient became haemodynamically unstable, emergent pericardiocentesis with right heart catheterisation was performed and 800?mL of exudative pericardial fluid was drained with only transient clinical improvement. On right heart catheterisation, the venous waveform prior to drainage of effusion showed an amputated ��y�� descent indicating cardiac tamponade, while following the drainage, a steep ��y�� descent along with a steep ��x�� descent appeared. This finding was suggestive of presence of effusive constrictive pericarditis (figure 2).