IgG4-related Disease Jen Ng, MD PGY-2 June 18, 2013 U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Mr. J is a 55 year old African American male who presents with abdominal pain and malaise/fatigue for three weeks. Chief Complaint U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Three weeks prior to admission, the patient developed progressively worsening, intermittent, “crampy” abdominal pain in the epigastrum and lower quadrants, varying in intensity, not related to food, and without associated change in bowel habits. He also noted worsening fatigue/malaise and anorexia over this time period. Three days prior to admission, he had 1 self-resolving episode of epistaxis. On morning of admission, he noted 1 episode of black stool, which alarmed him, and he therefore presented to the emergency department. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Additional History Past Medical History: Coronary artery disease with 2 bare metal stents placed in 2008 Left leg deep venous thrombosis in 2004 Factor VIII hyperactivity Hypertension Hyperlipidemia Melena 3/2012 with upper endoscopy/colonoscopy/capsule endoscopy significant only for mild sigmoid diverticulosis and internal hemorrhoids Iron-deficiency Anemia Past Surgical History: None U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Additional History Social History: 20 pack year smoking history. History of alcohol and cocaine abuse in the past, sober since Active marijuana use. Lives in shelter Family History: None Allergies: Morphine and codeine - rash Medications: Aspirin 81mg daily Metoprolol tartrate 25mg daily Simvastatin 40mg at bedtime Lisinopril 5mg daily Ferrous sulfate 325mg twice a day (not compliant) U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Physical Examination General: chronically ill appearing man, lying down in no apparent distress Vital Signs: T: 97.2 BP: 115/68 HR: 114 (improved to 95 with 1L saline) RR: 20 and O2 sat: 100% on room air Abdomen: soft, not distended, mildly tender to palpation in lower quadrants, without rebound or guarding, normoactive bowel sounds Remainder of physical exam including rectal exam was normal U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Laboratory Findings CBC: hemoglobin 11.1 (baseline), MCV 73 Remainder of CBC was within normal limits Basic Metabolic panel: within normal limits Hepatic panel: within normal limits PT 14.2, INR 1.35, PTT within normal limits HIV negative Venous lactate within normal limits Urinalysis within normal limits U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Other Studies ECG: normal sinus rhythm with rate of 90 beats per minute and left ventricular hypertrophy Chest X-Ray: no acute cardiopulmonary processes U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS Findings: A 4 x 6 x 6cm spiculated mass is centered within the mid abdominal mesentery, inferior to the level of the superior mesenteric artery and contains large central calcifications. It encases and markedly narrows the superior mesenteric artery. No bowel wall thickening. Impression: Findings are most consistent with sclerosing mesenteritis with associated retroperitoneal fibrosis. Other considerations include carcinoid tumor or lymphoma. Marked superior mesenteric artery narrowing without evidence of bowel ischemia.

Hospital Day 1: –Surgery was consulted for biopsy of mesenteric mass to assist with diagnosis –Serial abdominal exams remained stable. –The patient’s pain was treated with tramadol 50mg every 4-6 hours as needed. –The patient’s hemoglobin and vitals remained stable. Hospital Day 2: –ESR and CRP were elevated at 33 (0-15) and (<= 3), respectively Hospital Day 3-4: –24 hour urinary excretion of 5-hydroxyindoleacetic acid was collected, results within normal limits. –Biopsy was deferred for outpatient setting and the patient was discharged. Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

The patient followed up with surgery clinic and he received surgical biopsy confirming the diagnosis of sclerosing mesenteritis. He then saw his primary care provider that same month, who performed age-appropriate cancer screening (as his condition can be associated with a paraneoplastic syndrome) including PSA, all of which were within normal limits. Given that his abdominal pain had resolved and he had no other symptoms, treatment was deferred. He was lost to follow up during the Hurricane Sandy period and then later re-presented to medicine clinic, again with abdominal pain. At this time, he was noted to have had a rise in his creatinine from 0.8 to 1.9. Post-discharge Clinical Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Renal ultrasound and repeat CT scan of his abdomen/pelvis were significant for increasing size of the mesenteric mass complicated by severe left hydronephrosis and bowel wall edema in setting of likely mesenteric venous congestion. The patient was admitted and he received a left percutaneous nephrostomy tube placement with stabilization of his renal function. After his discharge, he returned to his primary care provider for follow up. Immuno-staining from his biopsy showed borderline increase in IgG4 positive plasma cells, ALK-1 stain negative. His IgG4 level was 123 (4-86). He was started on prednisone 40mg and tamoxifen 10mg twice a day, now only on prednisone taper. Repeat CT scan of his abdomen/pelvis showed stable size of mass. Post-discharge Clinical Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

IgG4-related Sclerosing Mesenteritis Final Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS