CASE SERIES OF AORTITIS PANCREATITIS AND THYROIDITIS LEADING TO ONE DIAGNOSIS

Document Type

Conference Proceeding

Publication Date

6-27-2024

Publication Title

J Gen Intern Med

Keywords

creatinine, immunoglobulin G, immunoglobulin G4, prednisone, steroid, triacylglycerol lipase, abdominal aorta, abdominal pain, acute abdomen, acute pancreatitis, adult, aortitis, arteritis, autoimmune pancreatitis, case report, case study, clinical article, complication, conference abstract, diagnosis, diarrhea, drug therapy, epigastric pain, female, fibroblast, histopathology, human, human tissue, hydronephrosis, immunoglobulin G4 related disease, intestine infarction, intravenous drug administration, kidney infarction, lung biopsy, male, middle aged, Mikulicz disease, oral drug administration, orbit disease, pancreatitis, renal artery, retroperitoneal fibrosis, right groin, sclerosing cholangitis, secondary prevention, submandibular gland, thyroid disease, thyroiditis, vasculitis

Abstract

CASE: Case 1 - 48-year-old male patient with a complaint of 4 days of abdominal pain with associated diarrhea.Patient was afebrile, hemodynamically stable and lab work including lipase and LFTs were unremarkable except creatinine 1.6 with GFR 54. CT abdomen pelvis showed findings suggestive of acute/subacute infarcts within the anterior mid left kidney and anterior right kidney. There were also patchy areas of ischemia in different regions of gastrointestinal tract. Non-specific fat stranding in the distal aortocaval region and extending into the proximal right iliac region suggestive of inflammatory process versus retroperitoneal fibrosis or inflammatory aortitis/arteritis.CTA showed severe stenosis with wall thickening of the right renal artery, left renal artery, distal abdominal aorta, SMA and IMA regions.Rheumatology evaluated the patient, upon work-up, IgG levels were elevated. Patient was started on oral steroids for possible IgG4 related vasculitis which led to drastic improvement in symptoms. Case 2- 69 years old female patient with recently diagnosed thyroid disease presented with complains of acute epigastric pain over the last few hours. Acute abdominal series showed large mass-like opacity at left med/lower lung zone. CT abdomen and pelvis with IV contrast showed acute interstitial pancreatitis. IgG 4 levels were elevated, patient's pancreatitis likely secondary to autoimmune pancreatitis. Patient started on prednisone 40 mg once daily with improvement in 2 days after initiation. Plan was made to to continue for 4-6 weeks followed by taper. Further thyroid and lung biopsies demonstrated abundance of plasma cells and storiform fibroblasts which consolidated the diagnosis of IgG4 related autoimmune diease with multi system involvement. IMPACT/DISCUSSION: Myriad of ways IgG4 disease can present and adequate diagnosis for early treatment to reduce morbidity. In patients with idiopathic pancreatitis and aortitis, IgG4 should be suspected so that early treatment can be intiated to avoid complications like renal/intestinal infarcts, retroperitoneal fibrosis which can involve ureters and cause hydronephrosis CONCLUSION: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibroinflammatory condition that is capable of affecting multiple organs. Common forms of presentation include pancreatitis, sclerosing cholangitis, sclerosing sialdenitis in lacrimal, parotid and submandibular glands (Mikulicz disease), orbital disease, retroperitoneal fibrosis chronic aortitis often involving ureters and causing hydronephrosis. Histopathology findings are important to the diagnosis of IgG4-RD, such findings are never diagnostic alone of IgG4-RD. Pathology findings must always be interpreted in the context of clinical, serologic, and radiologic data. Early recognisition is important given early initiation of steroid based treatment if it is missed in the setting of alternative diagnosis and hence the treatments in cases like acute pancreatitis and renal infarcts.

Volume

39

First Page

S278

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