Another case of back painAnother 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.

• 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.

• 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.

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:

• 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- 500,000 per year• Vague flank, low back and abdomen pain,

malaise, anorexia, wt loss pyrexia, nausea and vomiting.

• 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

• 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

• 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 2006 19 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 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.

• 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

• 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