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.

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Presentation transcript:

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.

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 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 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: 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 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 per year Vague flank, low back and abdomen pain, malaise, anorexia, wt loss pyrexia, nausea and vomiting Ormond’s disease, periureteritis fibrosa, periureteritis plastica periureteritis, sclerosing retroperitoneal granuloma, fibrous retroperitonitis. 1:200, ,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 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 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. 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. 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. 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. 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 pts rx’d with 20 mg bid and 15 responded in 2.5 weeks. One recurred and responded to retreatment. 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 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: % recur; mortality is less than 10% over many years if not associated with a malignancy. Methotrexate (20 mg/week), mycophenolate mofetil, azathioprine have been used. Prognosis: % 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 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