An 18-year-old boy immigrant from Guinea was admitted to our hospital because of fever and abdominal pain starting about three weeks before. His past medical history was unremarkable. Physical examination revealed diminished lung breath sounds consistent with pleural effusion and hepatomegaly. Lab tests showed abnormal blood count (PLT 741 x 103/mm3), high erythrocyte sedimentation rate (ESR:87 mm/h), C-reactive protein (CRP:10.22 mg/dl), and gamma globulins (25.3%, n.v. 11.1-18.8%), while procalcitonin (PCT) was 0.9 ng/ml (n.v. < 0.5 ng/ml). All these findings suggested an inflammatory/infectious disease.
Ultrasound examination showed fluid in the abdominal cavity together with bladder wall thickening (Figure 1), hepatomegaly, and pleural and pericardial effusion. Computed tomography confirmed all these findings (Figure 2).
Within few days the patient experienced worsening fatigue and dyspnea. Significant jugular venous distension and hepatomegaly were present on physical examination. Blood pressure was 100/60 mm Hg without evidence of pulsus paradoxus. However, echocardiography showed a large pericardial effusion (>5 cm) with significant respiratory variations (i.e., >25%) of mitral inflow and inferior vena cava plethora (Figure 3). All these findings were considered consistent with an incipient cardiac tamponade and the patient was treated with an urgent echo-guided subxiphoid pericardiocentesis (1500 mL of serohematic fluid), without complications. A pericardial drainage catheter was left in situ up to 48 h in order to promote adherence of pericardial layers and prevent further accumulation of fluid. The analysis of pericardial fluid was unremarkable (e.g., serology negative for Mycobacterium tuberculosis). The patient was empirically treated with Ibuprofen (600 mg tid) and Doxycycline (100 mg bid), until further microbiological tests (faeces and urine) revealed an Ancylostoma duodenale/Necator americanus infection. The patient was thus successfully treated with mebendazole (100 mg bid). His clinical conditions progressively improved, despite the persistence of abdominal discomfort. A new echocardiogram after 6 weeks showed an evolution to constrictive pericarditis (Figures 4 and 5).
At this point, due to the lack of a definite aetiology for the polyserositis with effusive-constrictive pericarditis we suspected an IgG4-related disease. Our diagnostic hypothesis was confirmed by elevated serum IgG4 concentrations (3.050 g/L, n.v. 0.011-1040). The patient was thus treated with prednisolone at a dose of 75 mg per day for 2 weeks. The echocardiogram showed a progressive resolution of the pericardial disorder without constrictive physiology. The prednisolone was tapered to 25 mg per day and the patient was discharged. Last follow-up echocardiogram (three weeks later) showed an almost complete resolution of the pericardial disease with mild residual thickening of both layers and residual deposits of fibrin (Figure 6).