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Another case of back pain. 70 yo white female c 15 yr hx of seronegative polyarthritis c non-erosive, symmetrical mcp swelling and ulnar deviation. Also hx of DDD of spine with sciatica and response to LESI and oral antiinflams.
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70 yo white female c 15 yr hx of seronegative polyarthritis c non-erosive, symmetrical mcp swelling and ulnar deviation. Also hx of DDD of spine with sciatica and response to LESI and oral antiinflams.
2003 presented with epigastric pain and had a cholecystectomy after ultrasound showed stones. • ESR’s ranged from 30 to 46 between 2002 and 2004 with variable synovitis. • Rx of polyarthritis with NSAIDS.
2004 found to have a positive ppd and was given INH - developed hepatitis. June 2007 new complaint of left flank pain; Physical reveled a mass at left of uterus. CT showed hydronephrosis of left kidney and mass at left side of the bladder.
July 2007 under went bilat salpingo-hysterectomy and revision of the left ureter and biopsy of mass. • Dx retroperitoneal fibrosis; no cancer. • Path:
3 weeks post op: ESR 115 and CRP 15 • Pt returned with one day hx of right flank pain, malaise • CT showed stent in left ureter and hydronephrosis of right side • Creatinine 2.1; urinalysis normal
1948 Ormond’s disease, periureteritis fibrosa, periureteritis plastica periureteritis, sclerosing retroperitoneal granuloma, fibrous retroperitonitis. • 1:200,000- 500,000 per year • Vague flank, low back and abdomen pain, malaise, anorexia, wt loss pyrexia, nausea and vomiting.
Elevated ESR and CRP • Fibrotic encasement of ureters causes obstructive uropathy and renal insufficiency • Chronic periaortitis, perianeurysmal fibrosis
Idiopathic (2/3) and secondary (1/3) • Secondary: drugs - methylsergide, bromocriptine, beta blockers, methyldopa, hydralazine, analgesics. • Malignancy: carcinoid, lymphoma sarcoma
Infections - TB, Histo, actinomycosis • Radiation Rx - testicular seminoma, colon, pancreatic CA. • Surgery:lymphadenecomy colectomy, AAA repair.
Pathology - hard white plaque around the aorta and iliac vessels and ureters. • Micro - sclerosis and infiltration of mononuclear cells • Antibodies to fibroblasts, IgG4 producing plasma cells.
Early stage - low back, flank, abd pain - dull, girdle distribution in 90%; weight loss, malaise, anorexia, testicular pain, claudication, edema, thrombophlebitis, intestinal ischemia
Late stage - ureteral obstruction, flank pain, uremia • Lab - elevated ESR and CRP. ANA is abnormal in 60%. Leukocytosis and eosinophilia are frequent. Urinalysis is normal. • Monoclonal or polyclonal dysproteinemias.
Presentation is usually obstructive uropathy. • Ultrasound and CT can suggest the Dx. • MRI can be used, but if GFR is impaired, giving Gadolinium can cause nephrogenic systemic fibrosis.
Treatment - relief of obstruction. • Corticosteroids 90% respond to 60 mg/ day for 6 weeks, then taper to 10 mg for 6-18 months. • Tamoxifen- used in desmoid tumors. Unclear mechanism. AIM 2006 19 pts rx’d with 20 mg bid and 15 responded in 2.5 weeks. One recurred and responded to retreatment.
Methotrexate (20 mg/week), mycophenolate mofetil, azathioprine have been used. • Prognosis: 10 - 30% recur; mortality is less than 10% over many years if not associated with a malignancy.
Pt was placed on 60 pred and her flank pain was gone in 24 hours • At week one ESR was 10 and CRP 0.5. And creatinine fell from 2.1 to 1.6. Back pain was improved. BP 180/95 and sugar 160 • Pred dropped to 40mg qd; rifampin added; after 5 days tamoxifen 20mg bid