Abstract Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated inflammatory condition leading to chronic fibrotic changes with multi-organ involvement. The pathophysiology of this condition remains incompletely understood; however, it is characterized by infiltration by IgG4-positive plasma cells and storiform-pattern fibrosis. Although involvement of the lung parenchyma and pleural space has been well recognized, pulmonary vascular involvement is uncommon. We present a case of precapillary pulmonary hypertension from IgG4-RD in a young female. A 32-year-old female presented with progressive shortness of breath for several months. On examination, she was noted to have hypoxia, tachycardia, anasarca and exophthalmos. Echocardiogram demonstrated McConnell’s sign with an elevated pulmonary artery systolic pressure. Subsequent CT angiography revealed no pulmonary arterial filling defects but showed enlarged pulmonary trunk enlargement, large bilateral pleural effusions with diffuse bilateral ground glass opacities, diffuse lymphadenopathy as well as hepatomegaly. Extensive lab work done as shown in Table 1. Relevant positive work up included normocytic anemia, eosinophilia, elevated beta natriuretic peptide, erythrocyte sedimentation rate (ESR), ferritin, Interleukin -2 (IL-2) and IgG4 levels. She underwent bilateral thoracentesis with transudative effusions. Fine needle aspiration of cervical node showed reactive lymph node with normal flow cytometry and core biopsy of axillary node showed increased number of plasma cells with 4-5 IgG4 positive cells per high-power field. She had an abdominal fat pad biopsy that was negative for amyloidosis. Based on above findings, the patient was treated for IgG4-RD with high-dose glucocorticoids with a prolonged taper, mycophenolate sodium and Rituximab infusion. Right heart catheterization confirmed precapillary pulmonary hypertension with mean pulmonary artery pressure of 58 mmHg, pulmonary vascular resistance of 9 Woods unit, pulmonary capillary wedge pressure of 6 mmHg, cardiac output of 3.76, cardiac index of 2.03 L/min/m2 and pulmonary artery saturation of 52%. The patient was treated with combination therapy of phosphodiesterase 5 inhibitor, endothelin receptor antagonist and activin signal inhibitor with significant improvement of her clinical and hemodynamic status. IgG4-RD is often considered a great mimicker and can be challenging to diagnose and manage. We present a case of acute right sided heart failure secondary to IgG4-RD which underscores the complex pathology and varying clinical manifestations of this disease. Multimodal imaging, tissue biopsy, and a high index of suspicion are essential for diagnosis. Early recognition and strategic treatment plans are critical to prevent irreversible organ damage and improve patient outcomes. This abstract is funded by: None
Walnofer et al. (Fri,) studied this question.