Case reports: 1. IGG4 Related Fibrosis: A Treatable Disease. Four Cases in a District General Hospital

Abstract

Background: IgG4 related fibrosclerosis is rare multi-system disease, characterised by IgG4+ plasma cells and T-lymphocytic infiltration of affected organs. Patients present to various specialties with a wide range of symptoms depending on the organ involved. Patients may present to rheumatologists with raised inflammatory markers or symptoms mimicking a connective tissue disease such as Sjögren's syndrome. Diagnosing and recognising this condition is important since the majority of patients respond to corticosteroids. Methods: We present 4 cases presenting to a district general hospital. Results: Case 1 A 57-year-old female presented with epigastric pain, weight loss and loin pain. Abdominal CT revealed a mass encasing the aorta and inferior vena cava, an enlarged pancreas and thickening of the sigmoid colon. Renal ultra-sound revealed bilateral hydronephrosis. Biopsy of the para-aortic mass confirmed IgG4 related fibrosis which had led to retroperitoneal fibrosis and hydronephrosis. She was commenced on prednisolone. Her disease is currently well controlled with Mycophenolate mofetil and low dose prednisolone. Case 2 A 61-year-old female was seen in Ophthalmology with bilateral proptosis. Past medical history included recurrent cervical and submandibular lymphadenopathy. MRI showed enlarged lacrimal glands and enlargement of the lateral, inferior and medial rectus muscles. She complained of sicca symptoms. Orbital biopsy demonstrated substantial IgG4+ plasma cells. Submandibular gland biopsy demonstrated chronic sialadenitis and IgG4+ cells. She was commenced on prednisolone with improvement in her symptoms. She was given a course of Rituximab therapy and remains on low dose prednisolone. Repeat orbital MRI has demonstrated improvement. Case 3 A 64-year-old lady was referred to ophthalmology with left orbital swelling and diplopia. Initial biopsy demonstrated a chronic inflammatory process. She was diagnosed with a periorbital pseudotumour and commenced on steroids and underwent deep orbital radiotherapy. MRI of the orbit demonstrated a soft tissue lesion in the superonasal aspect of the left orbit. Her symptoms deteriorated and she underwent debulking. Histology confirmed plasma cells expressing IgG4 and localised amyloidosis of the orbit. She remains asymptomatic without treatment and has declined further investigations. Case 4 A 59-year-old man complained of enlarging lymph nodes in the left side of his neck and groin. Ultra-sound revealed multiple lymph nodes. Left submandibular lymph node biopsy demonstrated substantial IgG4+ plasma cells. He was commenced on steroid therapy tapered over 4 weeks. He currently remains asypmtomatic and is not requiring treatment. The literature on this condition is sparse and will be reviewed. Conclusions: IgG4 related fibrosis is a newly recognised disease entity. Recognition is important as this condition responds to corticosteroid therapy. Disclosure statement: The authors have declared no conflicts of interest.