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An Unusual Cause of Abdominal Pain

An Unusual Cause of Abdominal Pain. Jeffrey H. Phillips, M.D., F.A.C.P. Internal Medicine Clinical Update March 24, 2004. History of illness. 63 year old African American man presents June, 2003 with 3 weeks of nausea and periumbilical abdominal discomfort

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An Unusual Cause of Abdominal Pain

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  1. An Unusual Cause of Abdominal Pain Jeffrey H. Phillips, M.D., F.A.C.P. Internal Medicine Clinical Update March 24, 2004

  2. History of illness • 63 year old African American man presents June, 2003 with 3 weeks of nausea and periumbilical abdominal discomfort • PMH: mild intermittent asthma; BPH; tonsillectomy • MEDS: prn albuterol ALLERGIES: None • FH: Father with Alzheimer’s. Brother with DM. • SH: retired insurance exec.; nonsmoker x 40 years; no alcohol x 2 years; one coffee per day; exercises regularly • ROS: weight down 10 pounds; otherwise neg.

  3. Physical exam • VS: BP 130/70, HR 70, RR 14, T 98.3 • No jaundice or rash • Sclera anicteric; no oral ulcers; no adenopathy • Normal cardiopulmonary exam • Abdomen soft; mildly tender in the epigastric and periumbilical areas; bowel sounds normal • No hernias; 1+ prostate; stool guaiac negative

  4. Diagnostic work-up (June’03) • WBC 6100, Hb 11.8, platelets 411,000; BUN/Cr 20/1.1, LFTs, lipase, PSA normal; globulin slightly elevated at 4.5 • UGI: moderate GE reflux; otherwise negative • Sonogram: normal liver and pancreas; hypoechoic tissue encasing abdominal aorta; no adenopathy • CT abdomen: 4 x 5 cm periaortic mass from SMA origin down to iliac arteries; compatible with matted nodes, probable lymphoma; no aorta aneurysm seen • CT directed needle biopsies: abnormal but nondiagnostic

  5. CT – June, 2003

  6. Diagnostic work-up (July ’03) • Hospitalized in July for open biopsy • Path consistent with idiopathic retroperitoneal fibrosis (special stains negative for lymphoma) • Pertinent labs: • ESR 141 • CRP 5.77 • ANA, cANCA normal • H/H 8.3/26.3; platelets 631,000 • BUN/Cr 24/1.9

  7. Who ya' gonna call...?

  8. Who ya’ gonna call…? • Surgery? • Gastroenterology? • Rheumatology? • Oncology? • Urology? • Chief of Medicine?

  9. Idiopathic retroperitoneal fibrosis • First described by French urologist Albarran in 1905 • Established as clinical entity by Ormond in 1948 with description of two cases • Characterized by the extensive development of inflammatory fibrotic tissue in the retroperitoneum • Often leading to compression and obstruction of ureters and adjacent organs

  10. Idiopathic retroperitoneal fibrosis • Annual incidence: 0.2 to 0.5 per 100,000 • Average age 50; M:F ratio 2:1 • No ethnic differences • Pathology: dense plaque in the RP, starting at aortic bifurcation, enveloping aorta, IVC, ureters • Bilateral in two thirds of cases • May involve gonadal, celiac, SM, renal arteries

  11. Pathogenesis • Cause unknown; probably autoimmune response to an insoluble lipid called ceroid leaking through thinned arterial walls (circulating ceroid Abs in >90% patients) • Increased production of cytokines (interleukins and Th2) mediates inflammation/fibrosis • Early stage: plaque highly vascular with deposition of collagen and infiltrates of polyclonal B and CD4 T cells • Inflammatory tissue is gradually replaced by fibrosis • Most clinical features secondary to mass effect of fibrosis on adjacent structures

  12. Clinical features • 80% present with dull, poorly localized, noncolicky pain in back, flank, or abdomen • Weight loss, nausea, malaise less frequent • Increasing urinary obstruction with decreased volume or flank pain • Impingement of the IVC or aorta may produce lower extremity edema, thrombophlebitis, claudication, or intestinal ischemia

  13. Diagnosis • Elevated ESR in 80 to 90% • Mild normochromic, normocytic anemia • Elevated BUN/Cr • Imaging depends on presentation: IVP vs. sonogram vs. CT abdomen; MRI may image best • Biopsy necessary to exclude secondary causes • May coexist with other immune-mediated diseases: • PBC, RA, SLE, PAN, GN, ankylosing spondylitis, fibrosing mediastinitis, Hashimoto’s thyroiditis, Wegener’s

  14. Treatment - Surgical • Indicated in cases with severe ureteral or other organ involvement (ureterolysis) • Cystoscopically placed ureteral stents may obviate need for open surgery • Open biopsy also necessary to exclude lymphoma or metastaic cancer as cause of fibrotic process

  15. Treatment - Medical • Corticosteroids • First used in 1958 • Suppress inflammatory response • Best if started in early stages • Typical regimen is prednisone 40 to 60 mg daily tapered to 10 mg daily over 3 to 6 months and then continued several years at low dose • Pulse dosing or alternate day dosing are options

  16. Steroid Therapy for Idiopathic Retroperitoneal Fibrosis: Dose and DurationKardar, et al. Journal of Urology (2002) 168: 550-555 • Prospective study of 12 patients with IPF over 10 year period treated with alternate day steroids • 60 mg q.o.d. x 2 months, 40 mg q.o.d. x 2 weeks, 20 mg q.o.d. x 2 weeks, 10 mg q.o.d. x 2 weeks, and then 5 mg daily for 2 years • Follow-up of 26 to 132 months (median 63) • Only 2 patients failed (1 non-responder and 1 with recurrent symptoms and elevated ESR)

  17. Treatment - Medical • Immunosuppressive agents • Azathioprine • Cyclophosphamide • Methotrexate • Mycophenolate mofetil • Typically given initially with steroids • May serve as a steroid sparing agent • May be first-line when surgery not an option

  18. Immunosuppressive Therapy for Idiopathic Retroperitoneal Fibrosis: A Retrospective Analysis of 26 CasesMarcolongo, et al. AJM (2004) 116: 194-197 • 26 patients with IRF and ureteral obstruction over a 12 year period treated with ureteral stents or nephrostomy • All patients received prednisone (1 to 1.5 mg/kg for 3 weeks, then tapered over 6 months) and • Azathioprine 2.5 mg/kg/d x 6 months then reduced to 1.5 mg/kg x 6 months or • Cyclophosphamide 2 mg/kg/d x 3months and tapered and discontinued within 6 months • Treatment failure rate: 1 per 100 patient-years • This study showed increased safety with azathioprine

  19. Tamoxifen • Effective in pelvic desmoid tumors • Thought to increase production of transforming growth factor-B, an immunosuppressive cytokine that modulates fibroblast activity • Also inhibits protein kinase C, an obligatory mediator of cell proliferation • Relatively safe though may increase risk of thromboembolism and ovarian cancer • Several small series show effectiveness

  20. Prognosis • Resolution is rare without treatment • Over time, mass less likely to respond to medical therapy (less inflammation and more fibrosis) • Renal insufficiency resolves or significantly improves with treatment • Ureteral obstruction may recur in up to 50% treated with surgery alone, compared to 10% treated with steroids, and 1% with steroids plus immunosuppressive therapy

  21. CT imagesJune, 2003 September, 2003

  22. CT imagesMarch 19, 2004

  23. Conclusions • 80% present with dull, poorly localized pain in back, flank, or abdomen; ESR elevated in 80% to 90% • CT or MRI, and biopsy help exclude secondary causes • Surgery often necessary for ureteral involvement • Steroids with or without immunosuppresive therapy is mainstay of treatment and offers best prognosis • Long-term follow-up is necessary

  24. Early diagnosis and prompt treatment preserve organ function

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