IgG4 related pericardium and lung disease in pediatric patient complicated with fatal massive hemoptysis: a case report and review of literature

Background: IgG4-related disease (IgG4-RD) is a progressive and sometimes fatal disease that rarely affects pediatric age group. It may affect the orbits, lacrimal and salivary glands, pancreas, kidneys, peritoneum and other organs. Lung and pleura are not commonly reported in IgG4-RD. We here present a rare case of pediatric IgG4-RD with rare involvement of pericardium, pleura and lungs.

Case presentation: A 13-year-old girl presented with intrathoracic IgG4-RD with pleuropericardial involvement. She showed initial improvement on prednisolone. Azathioprine and then mycophenolate failed to control relapses during steroid tapering. Her last relapse was treated by rituximab however, the patient developed acute fatal massive hemoptysis.

Conclusions: Pediatric IgG4-RD is a rare entity with pericardio-pulmonary affection as the rare of the rare. Usual treatment of prednisolone and steroid sparing agents should be used, with rituximab used as a rescue therapy, but fatal complications may occur.

Comments：

IgG4-RD is indeed a rare disease, and it is even more unusual to see pericardio-pulmonary involvement in a pediatric patient. It is important to be aware of such cases to better understand the spectrum of the disease.

It is encouraging to hear that the patient initially responded to prednisolone, which is the first-line treatment for IgG4-RD. However, it is unfortunate that the relapses could not be controlled with steroid-sparing agents such as azathioprine and mycophenolate. The use of rituximab as a rescue therapy is a reasonable option, but as you noted, it is not without risks.

The occurrence of fatal massive hemoptysis is a rare but known complication of rituximab, and it underscores the importance of carefully considering the risks and benefits of any treatment. It is important to weigh the potential benefits of controlling the disease activity against the risks of adverse effects of the treatment.

Overall, this case highlights the need for close monitoring of pediatric patients with IgG4-RD, especially those with unusual or severe presentations, and the importance of individualized treatment plans.

 

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