IgG4-Related Retroperitoneal Fibrosis in a Patient With B-Cell Lymphoproliferative Disorder
Rida Inam1, Asim Mehmood1, Attia Mahmood2
1Internal Medicine, Shifa College of Medicine, Islamabad, PAK.
Abstract:
We report an interesting case of a 25-year-old male patient who presented with a complaint of pain in the abdomen for six months, which was not associated with any other symptom, the patient was diagnosed with IgG4-related retroperitoneal fibrosis (RF) via endoscopic ultrasound (EUS)-guided biopsy. He was prescribed steroids and proton pump inhibitors. Due to the limited presentation and rarity of RF, diagnosis of this disease requires extensive diligence and care. In this case report, we underscore the importance of considering the differential diagnosis of RF or Ormond's disease when a patient presents with vague symptoms of pain in the abdomen. According to our knowledge, this is the first case of IgG4-related RF in a patient with B-cell lymphoproliferative disorder reported from Pakistan.
Insights
This case report highlights IgG4-related retroperitoneal fibrosis (RF), a rare condition diagnosed via endoscopic ultrasound (EUS)-guided biopsy. Early consideration of RF is crucial for patients with unexplained abdominal pain.
Area of Science:
- Gastroenterology and Immunology
- Pathology
Background:
- IgG4-related retroperitoneal fibrosis (RF) is a rare condition characterized by chronic inflammation and fibrosis in the retroperitoneum.
- Diagnosis can be challenging due to vague presenting symptoms and the need for specialized imaging and biopsy techniques.
Observation:
- A 25-year-old male presented with six months of unexplained abdominal pain.
- Endoscopic ultrasound (EUS)-guided biopsy confirmed IgG4-related retroperitoneal fibrosis (RF).
Findings:
- The patient was diagnosed with IgG4-related RF, a rare entity, particularly in association with B-cell lymphoproliferative disorder.
- This represents the first reported case of IgG4-related RF in a patient with B-cell lymphoproliferative disorder from Pakistan.
Implications:
- This case underscores the importance of considering IgG4-related RF (Ormond's disease) in the differential diagnosis of patients with persistent, vague abdominal pain.
- Increased awareness and diagnostic diligence are essential for timely identification and management of this rare condition.
