1. IgG4 related disease as a cause of isolated retroperitoneal fibrosis with no other organ involvement; Case report
- Author
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Priyanka Cackamvalli, Rubab Fatima Malik, Almurtada Razok, and Muhammad Zahid
- Subjects
medicine.medical_specialty ,Histopathology ,Case Report ,Disease ,Malignancy ,Retroperitoneal fibrosis ,Gastroenterology ,03 medical and health sciences ,IgG4 related disease ,0302 clinical medicine ,Rheumatology ,Fibrosis ,Internal medicine ,parasitic diseases ,medicine ,Autoimmune disease ,business.industry ,fungi ,General Medicine ,medicine.disease ,030220 oncology & carcinogenesis ,030211 gastroenterology & hepatology ,Surgery ,IgG4-related disease ,medicine.symptom ,business ,Rare disease - Abstract
Introduction and importance Retroperitoneal fibrosis (RPF) is a rare disease characterized by the replacement of normal tissue with fibrosis and inflammation. We present the case of a 40-years-old gentleman with RPF whose presenting complaints were bilateral flank pain and weight loss and was found to have IgG4 related disease. To the best of our knowledge, IgG4-related disease with isolated retroperitoneal involvement is a very rare occurrence. Case presentation The diagnosis of IgG4-Related retroperitoneal fibrosis was made based on clinical, radiological and histopathological criteria. Imaging revealed isolated retroperitoneal involvement and the patient was started on oral steroids with a good clinical response after ten days. Repeated imaging months later showed significant regression in the fibrosis. Clinical discussion RPF can occur due to many etiologies and is categorized to idiopathic and secondary. Factors associated with secondary RPF include medications, autoimmune disease, malignancy and IgG4 related disease. Almost all the reported cases of IgG4 related disease had evidence of multifocal involvement such as the pancreas, aorta and kidneys. Our patient was diagnosed with isolated RPF due to IgG4 disease. He met all the suggested diagnostic criteria, was started on oral steroids and had an excellent clinical outcome. Conclusion IgG4 related disease can present with isolated retroperitoneal fibrosis without involvement of other organ systems. The diagnosis should be based on specific criteria. Treatment with corticosteroids can lead to remission both clinically and radiographically., Highlights • Retroperitoneal fibrosis can present in a variety of clinical pictures. • IgG4 related disease can present with isolated retroperitoneal fibrosis. • It is diagnosed based on clinical, radiological and pathological criteria. • Treatment with corticosteroids can lead to clinical and radiological remission.
- Published
- 2021
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