IgG4-related disease is a fibroinflammatory systemic condition characterized by tumefactive lesions, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and elevated serum IgG4 concentrations. It has been described in virtually every organ system. Autoimmunity and infectious agents are potential immunologic triggers in IgG4-related disease. Herein, we describe a peculiar case of effusive-constrictive pericarditis in an 18-year-old boy with polyserositis and concomitant
IgG4-related disease is a systemic fibroinflammatory disease with tissue infiltration of IgG4-positive plasma cells. The histopathological features show similarities across organs; however clinical features may vary considerably. Cardiac manifestations may include pericarditis, often in a constrictive form.
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 × 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
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
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
IgG4-related disease is an increasingly recognized systemic immune-mediated condition. Its features include tumor-like swelling of involved organs, a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, and a variable degree of fibrosis that has a characteristic “storiform” pattern [
Since there is still no definitive serological test to diagnose IgG4-related disease, a combination of histological, immunophenotypic, clinical, radiographic, and laboratory investigations has been proposed to perform the diagnosis [
In a recent large cohort study the histopathological evaluations were more frequently performed in the most easily accessible organ such major salivary glands, kidney, lacrimal glands, and lung; in contrast, only a minority of patients underwent pancreas or retroperitoneal/periaortic biopsy because these deeper structures are much more difficult to access [
The same authors showed elevated serum levels of IgG4 in more than 95% of Japanese patients with IgG4-related disease [
IgG4-related disease is now a well, although rare, recognized cause of pericardial disease (i.e., constrictive pericarditis) with several reports over the last years [
We would like to draw attention to certain key aspects of our report, first of all the clinical course of the pericardial disease in our patient. Pericardial constriction due to IgG4-related disease, once clinically manifested, often requires biopsy followed by surgical pericardiectomy. In our patient we were planning these procedures due to the worsening of pericardial pathology; however, we observed an impressive and prompt response to glucocorticoids with an almost complete resolution of the pericardial constriction after 5 weeks, as documented by the instrumental echocardiographic follow-up.
The second aspect is related to the pathophysiology of IgG4-related disease in our patient. The pathogenesis of IgG4-related disease is poorly understood. Autoimmunity and infectious agents are potential immunologic triggers in IgG-4-related disease [
We conclude by recalling that IgG4-related disease, even if rare, should be considered in the diagnostic workout of patients with polyserositis and effusive-constrictive pericarditis and that there are strict relationships between parasitic infections and elevated serum IgG4 levels. To the best of our knowledge this is the first reported case of constrictive pericarditis in a patient with concomitant elevated serum IgG4 levels and
The authors declare that they have no conflicts of interest.
Giuseppe D. Sanna and Roberto Manetti contributed equally.
The authors wish to thank Dr. Anna Giovanna Naitana, Dr. Elisabetta Maria Porqueddu, and Dr. Giacomo Flore for their support in the clinical management of the patient.