Abstract Background/Aims IgG4 disease is a rare fibro-inflammatory multi-organ immune disorder which more commonly affects middle-aged to elderly patients, with a male predominance. ACR/EULAR classification criteria allow diagnosis to be made according to clinical, pathological, serological and radiographic changes if unable to obtain a histological sample. Histopathology shows lymphoplasmacytic infiltration, obliterative phlebitis and storiform pattern of fibrosis. It is typically responsive to glucocorticoids and B-cell depleting therapies. Methods A gentleman in his seventies was referred to respiratory clinic following an abnormal chest X-ray demonstrating a new large right upper lobe mass with a bulky hilum. He had reported exertional dyspnoea, mucopurulent sputum and night sweats over months. He was an ex-smoker (60 pack year history), had worked as a welder, and had asbestos exposure. His background included stroke, diabetes, chronic kidney disease and osteoporosis. In 2011, he was diagnosed with IgG4-mediated cholangiopathy and pancreatitis on the basis of MRI, endoscopic ultrasound and CT findings. IgG4 was elevated at 2.76 with normal tumour markers. He responded well to prednisolone but was intolerant of azathioprine. This year, when he presented with respiratory symptoms, his disease has been in remission. Results Routine bloods, including eosinophils, and Hepatitis B and C screen were unremarkable. Pulmonary function tests were obstructive. He was diagnosed with COPD and started on inhalers. He received oral amoxicillin to treat superadded infection. CT thorax-abdomen-pelvis showed right upper lobe (RUL) dense consolidation with a solid component, moderate emphysema and an ill-defined sclerosis of right 6th rib of uncertain significance. Repeat CT scan demonstration of improvement in RUL consolidation. He was discussed in the respiratory MDT. Bronchoalveolar lavage fungal, mycobacterium and bacterial cultures were negative. Brushings and washings demonstrated evidence of lymphocytic chronic inflammatory infiltrates without malignant cells. CT-PET scan revealed moderate FDG uptake at the RUL consolidation suggestive of infection or malignancy with reactive hilar lymph nodes. It showed avid FDG uptake at bilateral parotid glands, mild FDG uptake at the infra-renal abdominal aorta and moderate FDG uptake along the common iliac arteries. He had a raised dsDNA (18 IU/mL), but otherwise unremarkable ANCA, complement levels and autoimmune screen. Ultrasound of salivary and parotid glands showed benign multifocal Warthin’s tumour. The vascular radiologist felt periarterial inflammation/fibrotic changes represented early retroperitoneal fibrosis. He was referred to rheumatology and discussed in the IgG4 MDT; IgG4 disease was strongly suspected as per EULAR criteria. He was initially treated with mycophenolate and prednisolone before being switched to rituximab. Subsequent CT thorax demonstrated resolved consolidation with residual scarring. Conclusion IgG4 disease can be challenging to diagnose and manage. It can affect different organs over a long time course. Disclosure C.G. Mellor: None. N. Lane: None. J. Vila: None.
Mellor et al. (Wed,) studied this question.