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IgG4-related Disease
Jen Ng, MD
PGY-2
June 18, 2013
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Chief Complaint
•Mr. J is a 55 year old African American male
who presents with abdominal pain and
malaise/fatigue for three weeks.
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
History of Present Illness
•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.
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
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
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Additional History
•Social History:
•20 pack year smoking history. History of alcohol and cocaine
abuse in the past, sober since 2008. 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)
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
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
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
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
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Other Studies
•ECG: normal sinus rhythm with rate of 90
beats per minute and left ventricular
hypertrophy
•Chest X-Ray: no acute cardiopulmonary
processes
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
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.
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Hospital Course
• 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 29.43 (<= 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.
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Post-discharge Clinical Course
• 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.
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Post-discharge Clinical Course
• 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.
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS
Final Diagnosis
IgG4-related Sclerosing Mesenteritis
UNITED STATES
DEPARTMENT OF VETERANS AFFAIRS