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My Interesting Case My Interesting Case Janet M. Roscoe, MDCM, FRCPC, FACP Janet M. Roscoe, MDCM, FRCPC, FACP University of Toronto University of Toronto The Scarborough Hospital The Scarborough Hospital

My Interesting Case

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Page 1: My Interesting Case

My Interesting CaseMy Interesting Case

Janet M. Roscoe, MDCM, FRCPC, FACPJanet M. Roscoe, MDCM, FRCPC, FACP

University of TorontoUniversity of Toronto

The Scarborough HospitalThe Scarborough Hospital

Page 2: My Interesting Case

Submandibular Gland Swelling and Submandibular Gland Swelling and Acute Renal FailureAcute Renal Failure

• 47 Year old Male47 Year old Male• Well until late October 2005Well until late October 2005• Travelled to Cancun, Mexico, 2Travelled to Cancun, Mexico, 2ndnd week of October week of October• Developed bilateral, nontender swelling in both Developed bilateral, nontender swelling in both

submandibular areas, late in October, 2005submandibular areas, late in October, 2005• Saw ENT specialist-Dx “inflammatory process in the Saw ENT specialist-Dx “inflammatory process in the

submandibular gland”submandibular gland”• 2 courses of antibiotics gave no improvement2 courses of antibiotics gave no improvement

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Submandibular Gland Swelling & Submandibular Gland Swelling & ARFARF

• Continued to feel well but submandibular Continued to feel well but submandibular swelling continued until March 2006swelling continued until March 2006

• Developed symptoms of “sinus congestion” Developed symptoms of “sinus congestion” plus epistaxis and feeling of “fever”plus epistaxis and feeling of “fever”

• Tx by family physician with decongestantTx by family physician with decongestant• Denied deafness or ear pain but he felt his ears Denied deafness or ear pain but he felt his ears

weren’t “popping” back as quickly as usual when weren’t “popping” back as quickly as usual when flyingflying

• Chest X-ray done & reported as normalChest X-ray done & reported as normal

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SMG Swelling and ARFSMG Swelling and ARF

• Referred by ENT to Rheumatology for Referred by ENT to Rheumatology for assessment for possible Sjogren’s syndromeassessment for possible Sjogren’s syndrome

• BP noted to be elevated (February 2006)BP noted to be elevated (February 2006)• Patient started on calcium channel blocker and a Patient started on calcium channel blocker and a

beta blockerbeta blocker• Serum creatinine elevated at 180, 200Serum creatinine elevated at 180, 200

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SMG Swelling & ARFSMG Swelling & ARF

• Referred to NephrologyReferred to Nephrology• Seen in consultation on May 3Seen in consultation on May 3rdrd, 2006, 2006• Further Hx: Patient has been followed by a Further Hx: Patient has been followed by a

cardiologist downtown for a “bulge” in his aortacardiologist downtown for a “bulge” in his aorta• Presumably an aortic aneurysm, stable for ten Presumably an aortic aneurysm, stable for ten

years (measuring 44 mm)years (measuring 44 mm)• Has been told it might be a congenital Has been told it might be a congenital

abnormalityabnormality

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SMG & ARF HX. ContinuedSMG & ARF HX. Continued

• Headaches since starting BP medsHeadaches since starting BP meds• ““Tearing” of eyes but no redness or painTearing” of eyes but no redness or pain• No change visual acuityNo change visual acuity• No joint pain or ankle swellingNo joint pain or ankle swelling• Recent increased shortness of breath(attributes it Recent increased shortness of breath(attributes it

to his past hx of asthma as a child)to his past hx of asthma as a child)• SOB was significant to cause his family MD to SOB was significant to cause his family MD to

order the chest X-ray in Marchorder the chest X-ray in March

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SMG & ARF Hx continuedSMG & ARF Hx continued

• Increased urine frequency since March 2006 Increased urine frequency since March 2006 • now voiding q2hours day and nightnow voiding q2hours day and night• Urine colour is pale, never red or brownUrine colour is pale, never red or brown• No dysuria, no flank or abdominal painNo dysuria, no flank or abdominal pain• Family hx-father died age 59 of liver cancerFamily hx-father died age 59 of liver cancer• 2/3 brothers had premature CAD with CABG2/3 brothers had premature CAD with CABG

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SMG & ARF Physical ExamSMG & ARF Physical Exam

• BP 136/88BP 136/88• Bilateral hard submandibular swellingsBilateral hard submandibular swellings• Palpable liver 4 cm below costal margin, firm, Palpable liver 4 cm below costal margin, firm,

smoothsmooth• Dx Impression: Systemic vasculitis, probably Dx Impression: Systemic vasculitis, probably

Wegener’s granulomatosisWegener’s granulomatosis• Plan-Vasculitis w/uPlan-Vasculitis w/u

– Ultrasound and renal biopsyUltrasound and renal biopsy

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SMG & ARF Lab ResultsSMG & ARF Lab Results

• Nuclear Renal Scan-May 5Nuclear Renal Scan-May 5thth, 2006, 2006– Decreased blood supply; 25% function in left kidneyDecreased blood supply; 25% function in left kidney– Good function of right kidney (75%) but holdup of Good function of right kidney (75%) but holdup of

activity in upper pole calyxactivity in upper pole calyx• Abdominal Ultrasound May 9Abdominal Ultrasound May 9thth, 2006, 2006

– Abdominal Aorta unremarkableAbdominal Aorta unremarkable– Fatty infiltration of liver-no focal lesionsFatty infiltration of liver-no focal lesions– Right kidney large-13.4 cm longRight kidney large-13.4 cm long– Mild to moderate right hydronephrosisMild to moderate right hydronephrosis

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SMG & ARF Abdominal US SMG & ARF Abdominal US ContinuedContinued

• Right renal pelvis mildly distended along with proximal Right renal pelvis mildly distended along with proximal portion of the ureter, debris or solid material located in portion of the ureter, debris or solid material located in the proximal portion of the right ureterthe proximal portion of the right ureter

• Left kidney enlarged, 13.6 cmLeft kidney enlarged, 13.6 cm• Mild hydronephrosis and distention of proximal Mild hydronephrosis and distention of proximal

portion of the left ureter up to 2.2 cm and it also portion of the left ureter up to 2.2 cm and it also contains either debris or solid materialcontains either debris or solid material

• No cortical thinningNo cortical thinning• Prostate mildly enlargedProstate mildly enlarged• Recommend exclusion of solid lesion such as Recommend exclusion of solid lesion such as

transitional cell carcinomatransitional cell carcinoma

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SMG & ARF Lab Results ContinuedSMG & ARF Lab Results Continued

• Vasculitis workup including c and p ANCA, anti Vasculitis workup including c and p ANCA, anti DNA antibody, complements, angiotensin DNA antibody, complements, angiotensin converting enzyme all negativeconverting enzyme all negative

• But ESR is 121But ESR is 121• Urine protein and microalbumin/creatinine ratio Urine protein and microalbumin/creatinine ratio

normalnormal• Urinalysis negative for bloodUrinalysis negative for blood

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SMS & ARFSMS & ARF

• Urgent referral to urologist delayed because patient had Urgent referral to urologist delayed because patient had to travel out of townto travel out of town

• Seen by urologist, May 24Seen by urologist, May 24thth and cystoscopy, PSA, repeat and cystoscopy, PSA, repeat imaging plannedimaging planned

• Creatinine 174, PSA normalCreatinine 174, PSA normal• Flexible cystoscopy June 13Flexible cystoscopy June 13thth, 2006 showed mild , 2006 showed mild

prostatic enlargement with inflammation consistent prostatic enlargement with inflammation consistent with prostatitiswith prostatitis

• CT (noncontrast) ordered for further workup of other CT (noncontrast) ordered for further workup of other causes of hydronephrosis including retroperitoneal causes of hydronephrosis including retroperitoneal fibrosisfibrosis

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SMG & ARF: CT Scan June 30, 2006SMG & ARF: CT Scan June 30, 2006

• CT ResultCT Result• Kidneys inhomogenous and largeKidneys inhomogenous and large• Mild to moderate bilateral hydronephrosisMild to moderate bilateral hydronephrosis• Marked enlargement and thickening of the Marked enlargement and thickening of the

proximal ureters. proximal ureters. • Ureters are high in density in the proximal thirdUreters are high in density in the proximal third

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SMG & ARF CT ScanSMG & ARF CT Scan

• Cause for findings uncertain but could include Cause for findings uncertain but could include bilateral transitional cell carcinomas or blood bilateral transitional cell carcinomas or blood clots. Infectious debris cannot be excluded. clots. Infectious debris cannot be excluded.

• Inflammatory processes in the ureters could Inflammatory processes in the ureters could include ureteritis cystica or leukaplakia. include ureteritis cystica or leukaplakia.

• No discrete renal masses noted. No discrete renal masses noted. • No evidence of retroperitoneal, pelvic or No evidence of retroperitoneal, pelvic or

inguinal lymphadenopathy. inguinal lymphadenopathy. • Vascular structures are within normal limitsVascular structures are within normal limits

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SMG & ARFSMG & ARF

• Returns to my office on July 10Returns to my office on July 10thth, 2006, 2006• Now metallic taste in his mouth, severe fatigue, pruritisNow metallic taste in his mouth, severe fatigue, pruritis• Symptoms suggestive of uremiaSymptoms suggestive of uremia• Tenderness in left flankTenderness in left flank• Blood work July 11, 2006-creatinine 411, hemoglobin Blood work July 11, 2006-creatinine 411, hemoglobin

110, potassium 5.1 and bicarb 16110, potassium 5.1 and bicarb 16• Sinus X-ray-Mucosal swelling of left maxillary sinus Sinus X-ray-Mucosal swelling of left maxillary sinus

with suggestion of retention cystwith suggestion of retention cyst• Chest Xray-normalChest Xray-normal

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SMG & ARFSMG & ARF

• Admitted to SGH, July 12Admitted to SGH, July 12thth, 2006, 2006• Retrograde pyelography-bilateral hydronephrosisRetrograde pyelography-bilateral hydronephrosis• Ureters both show diffuse irregularityUreters both show diffuse irregularity• Bilateral stents insertedBilateral stents inserted

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SMG & ARF SMG & ARF

• Biopsy submandibular mass, July 12, 2006Biopsy submandibular mass, July 12, 2006• Chronic inflammationChronic inflammation• Numerous plasma cellsNumerous plasma cells• LymphocytesLymphocytes• Scattered lymphoid follicleScattered lymphoid follicle• Germinal cell formationGerminal cell formation• Marked fibrosis and parenchymal atrophyMarked fibrosis and parenchymal atrophy• Dx- chronic sialadenitis with marked fibrosisDx- chronic sialadenitis with marked fibrosis

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MRI Abdomen and PelvisMRI Abdomen and Pelvis

• July 13, 2006July 13, 2006• Extensive infiltration of the renal hila bilaterallyExtensive infiltration of the renal hila bilaterally• Extending into the renal pelvis bilaterally and along the Extending into the renal pelvis bilaterally and along the

uretersureters• Infiltrative process extends into the perirenal fat on the Infiltrative process extends into the perirenal fat on the

right sideright side• Infiltration or thickening of the left portal veinInfiltration or thickening of the left portal vein• Subtle infiltration of the pelvic fat surrounding the internal Subtle infiltration of the pelvic fat surrounding the internal

iliac arteries and veins and extending to the bladder baseiliac arteries and veins and extending to the bladder base• No adenopathyNo adenopathy• Pericardial effusion-maximum thickness of 1.5 cmPericardial effusion-maximum thickness of 1.5 cm• Provisional diagnosis retroperitoneal fibrosis but seen by Provisional diagnosis retroperitoneal fibrosis but seen by

Infectious Disease-Consider TB, histoplasmosis, and Infectious Disease-Consider TB, histoplasmosis, and BrucellosisBrucellosis

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Patient RadiologyPatient Radiology

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Patient RadiographyPatient Radiography

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Patient RadiographyPatient Radiography

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Patient RadiologyPatient Radiology

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Patient RadiologyPatient Radiology

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Patient RadiologyPatient Radiology

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Patient RadiologyPatient Radiology

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Diagnosis:Diagnosis:

• Dr. Tabo SikenataDr. Tabo Sikenata

• Did a literature Review and made the diagnosisDid a literature Review and made the diagnosis

• Multifocal Idiopathic FibrosclerosisMultifocal Idiopathic Fibrosclerosis

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Idiopathic RPFIdiopathic RPF

• The Journal of Rheumatology, 2006; 33: 358-361 The Journal of Rheumatology, 2006; 33: 358-361 (February)(February)

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Multifocal Idiopathic Fibrosclerosis Multifocal Idiopathic Fibrosclerosis Case 1:Case 1:

• 69 year old male69 year old male• 30 year history of chronic intermittent abdominal pain, 30 year history of chronic intermittent abdominal pain,

pseudoobstruction, cholangiolar fibrosis, right renal masses pseudoobstruction, cholangiolar fibrosis, right renal masses with hydronephorisiswith hydronephorisis

• Biopsies showed marked fibrosis with chronic Biopsies showed marked fibrosis with chronic inflammation typical of retroperitoneal fibrosisinflammation typical of retroperitoneal fibrosis

• Bilateral edema of eyes and diplopia, Left submandibular Bilateral edema of eyes and diplopia, Left submandibular gland swellinggland swelling

• Dx multifocal idiopathic fibrosclerosisDx multifocal idiopathic fibrosclerosis• Tx with prednisone and cylcosporineTx with prednisone and cylcosporine• Relapsed when cyclosporine withdrawn and remitted when Relapsed when cyclosporine withdrawn and remitted when

cyclosporine reintroducedcyclosporine reintroduced

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Multifocal Idiopathic Fibrosis Case 2Multifocal Idiopathic Fibrosis Case 2

• 74 year old male74 year old male• 5 year history of left upper quadrant pain5 year history of left upper quadrant pain• Gangrenous gallbladder-abdominal CT shows enlarged Gangrenous gallbladder-abdominal CT shows enlarged

pancreas with localized mass.pancreas with localized mass.• Pre-aortic mass 3 years laterPre-aortic mass 3 years later• Biopsy dense fibrosis with mild patchy chronic Biopsy dense fibrosis with mild patchy chronic

inflammation typical of idiopathic retroperitoneal fibrosis. inflammation typical of idiopathic retroperitoneal fibrosis. Mass encases ureter at up junctionMass encases ureter at up junction

• Develops bilateral enlargement of submandibular glandsDevelops bilateral enlargement of submandibular glands• Bx shows chronic sclerosing sialadenitisBx shows chronic sclerosing sialadenitis• Responds to prednisone and cyclosporineResponds to prednisone and cyclosporine

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Multifocal Idiopathic Fibrosclerosis Multifocal Idiopathic Fibrosclerosis Literature ReviewLiterature Review

• www.the lancet.com Vol. 367 January 21, 2006 www.the lancet.com Vol. 367 January 21, 2006 Retroperitoneal Fibrosis, Vaglio, Salvarani and Retroperitoneal Fibrosis, Vaglio, Salvarani and BuzioBuzio

• Range of diseases characterised by the presence Range of diseases characterised by the presence of a fibro-inflammatory tissueof a fibro-inflammatory tissue

• Usually surrounding abdominal aorta and the Usually surrounding abdominal aorta and the iliac arteries and extending into the iliac arteries and extending into the retroperitoneum to envelop neighbouring retroperitoneum to envelop neighbouring structures-eg the uretersstructures-eg the ureters

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Multifocal Idiopathic FibrosclerosisMultifocal Idiopathic Fibrosclerosis

• First described by French urologist in 1905First described by French urologist in 1905• Next described in English by Ormond who described two Next described in English by Ormond who described two

cases in 1948cases in 1948• Incidence 1:200,000-1:500,000Incidence 1:200,000-1:500,000• Prevalence 1-2 per 100,000Prevalence 1-2 per 100,000• Usual presentation-vague, poorly localized pain over flank, Usual presentation-vague, poorly localized pain over flank,

low back and abdomen or nonspecific systemic complaints-low back and abdomen or nonspecific systemic complaints-malaise, anorexia, weight loss, moderate pyrexia, nausea malaise, anorexia, weight loss, moderate pyrexia, nausea and vomitingand vomiting

• Acute phase reactants are often elevatedAcute phase reactants are often elevated• Obstructive uropathy and renal insufficiency may be Obstructive uropathy and renal insufficiency may be

present secondary to fibrous encasement of the ureterpresent secondary to fibrous encasement of the ureter

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Multifocal Idiopathic Multifocal Idiopathic Fibrosclerosisis: Major Causes of 2ry Fibrosclerosisis: Major Causes of 2ry

RPF (UpToDate, 2007)RPF (UpToDate, 2007)

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Multifocal Idiopathic FibrosclerosisMultifocal Idiopathic Fibrosclerosis

• Idiopathic RPFIdiopathic RPF• Umbrella term-chronic periaortitis Umbrella term-chronic periaortitis • Idiopathic retroperitoneal fibrosisIdiopathic retroperitoneal fibrosis• Inflammatory abdominal aortic aneurysmInflammatory abdominal aortic aneurysm• Perianeurysmal retroperitoneal fibrosisPerianeurysmal retroperitoneal fibrosis

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Multifocal Idiopathic Fibrosclerosis: Multifocal Idiopathic Fibrosclerosis: Schematic: www.thelancet.com 367 Schematic: www.thelancet.com 367

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Multifocal Idiopathic FibrosclerosisMultifocal Idiopathic Fibrosclerosis

• IRPF aorta not dilated, surrounding fibro-inflammatory tissue IRPF aorta not dilated, surrounding fibro-inflammatory tissue might or might not encase adjacent structuresmight or might not encase adjacent structures

• Inflammatory abdominal aortic aneurysm-tissue develops around Inflammatory abdominal aortic aneurysm-tissue develops around a dilated aorta but does not cause obstructionsa dilated aorta but does not cause obstructions

• Perianeurysmal rpf involves an inflammatory aneurysm-Perianeurysmal rpf involves an inflammatory aneurysm-surrounding tissue of which entraps adjacent organssurrounding tissue of which entraps adjacent organs

• Distinguish non-aneurysmal from aneurysmal formsDistinguish non-aneurysmal from aneurysmal forms• In the last 20 years introduction of medical therapy mainly based In the last 20 years introduction of medical therapy mainly based

on corticosteroids, and imaging techniques such as CT and MRI on corticosteroids, and imaging techniques such as CT and MRI has greatly improved patient outcomehas greatly improved patient outcome

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Idiopathic RPS EpidemiologyIdiopathic RPS Epidemiology

• Incidence 1:200,000 to 1:500,000Incidence 1:200,000 to 1:500,000• Males affected 2-3x more often than womenMales affected 2-3x more often than women• No ethnic predispositionNo ethnic predisposition• Mean age at presentation is 50-60 yearsMean age at presentation is 50-60 years• Can occur in children and older adultsCan occur in children and older adults• No familial clusteringNo familial clustering• Occasional reports in sibs or twinsOccasional reports in sibs or twins

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Idiopathic RPS: DiagnosisIdiopathic RPS: Diagnosis

• No standard diagnostic criteriaNo standard diagnostic criteria• CT or MRI scans are modalities of choice for diagnosis CT or MRI scans are modalities of choice for diagnosis

and followupand followup• Soft tissue mass surrounding the abdominal aorta and Soft tissue mass surrounding the abdominal aorta and

iliac arteries with possible encasement of neighbouring iliac arteries with possible encasement of neighbouring structures such as ureters and inferior vena cava structures such as ureters and inferior vena cava suggests diagnosis of RPFsuggests diagnosis of RPF

• Histological exam of RP tissue usually needed when Histological exam of RP tissue usually needed when mass shows atypical locations-pelvic, peripancreatic or mass shows atypical locations-pelvic, peripancreatic or if malignancy or infectious disease is suspectedif malignancy or infectious disease is suspected

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Idiopathic RPFIdiopathic RPF

• Pathogenous theory by Mitchinson and Parums-chronic Pathogenous theory by Mitchinson and Parums-chronic periaortitis is a range of diseases including idiopathic periaortitis is a range of diseases including idiopathic retroperitoneal fibrosisretroperitoneal fibrosis

• Characterized by advanced aortic atherosclerosis, Characterized by advanced aortic atherosclerosis, medial thinning, pronounced adventitial and perioaortic medial thinning, pronounced adventitial and perioaortic inflammation and fibrosisinflammation and fibrosis

• Local inflammatory reaction to oxidized low-density Local inflammatory reaction to oxidized low-density lipoproteins (LDL) and ceroid (a lipooproteic polymer lipoproteins (LDL) and ceroid (a lipooproteic polymer resulting from LDL oxidation within plaque resulting from LDL oxidation within plaque macrophages)macrophages)

• Aortic media breached or thinned-oxidized lipids Aortic media breached or thinned-oxidized lipids presented to T and B lymphocytespresented to T and B lymphocytes

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Idiopathic RPFIdiopathic RPF

• Self perpetuating inflammatory response occursSelf perpetuating inflammatory response occurs• Occurrence of constitutional symptoms, raised acute Occurrence of constitutional symptoms, raised acute

phase reactants, positive antibodies and associated phase reactants, positive antibodies and associated autoimmune disease involving other organs suggest autoimmune disease involving other organs suggest iRPF may be a manifestation of systemic autoimmune iRPF may be a manifestation of systemic autoimmune disease and not an exaggerated local reaction to disease and not an exaggerated local reaction to atherosclerosisatherosclerosis

• May be an association with HLA-DRB1*03, an allele May be an association with HLA-DRB1*03, an allele linked to various autoimmune diseases suggested, as linked to various autoimmune diseases suggested, as type 1 diabetes, myasthenia gravis and systemic lupus type 1 diabetes, myasthenia gravis and systemic lupus erythematosiserythematosis

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Idiopathic RPFIdiopathic RPF

• Malignant disease associated RPF results from Malignant disease associated RPF results from exuberant desmoplastic response to retroperitoneal exuberant desmoplastic response to retroperitoneal metastases-from ca of prostate, breast, colon or metastases-from ca of prostate, breast, colon or retroperitoneal primary tumour like Hodgkin’s or Non-retroperitoneal primary tumour like Hodgkin’s or Non-Hodgkin lymphoma, sarcoma or carcinoid (may Hodgkin lymphoma, sarcoma or carcinoid (may produce RPF by humeral factors without metastasing)produce RPF by humeral factors without metastasing)

• RPF secondary to infections is caused by local spread RPF secondary to infections is caused by local spread of a contiguous infectious process-eg spinal or of a contiguous infectious process-eg spinal or paraspinal abcesses in tuberculosisparaspinal abcesses in tuberculosis

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Idiopathic RPFIdiopathic RPF

• Macroscopic appearance-white hard retroperitoneal plaque of Macroscopic appearance-white hard retroperitoneal plaque of varying thickness surrounding retroperitoneal structuresvarying thickness surrounding retroperitoneal structures

• Rarely extends to mesenteric root or posteriorly to spinal cordRarely extends to mesenteric root or posteriorly to spinal cord• May appear as poorly circumscribed retroperitoneal massMay appear as poorly circumscribed retroperitoneal mass• Microscopic appearance-sclerotic tissue invasionMicroscopic appearance-sclerotic tissue invasion• Infiltrated with mononuclear cellsInfiltrated with mononuclear cells• Early stage edematous tissue and highly vascular, chronic Early stage edematous tissue and highly vascular, chronic

inflammationinflammation• Later pronounced sclerosis and scattered calcificationsLater pronounced sclerosis and scattered calcifications

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Idiopathic RPF Biopsy FindingsIdiopathic RPF Biopsy Findings

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Idiopathic RPF Biopsy FindingsIdiopathic RPF Biopsy Findings

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Idiopathic RPF Biopsy FindingsIdiopathic RPF Biopsy Findings

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Idiopathic RPF Biopsy FindingsIdiopathic RPF Biopsy Findings

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Idiopathic RPF Clinical Idiopathic RPF Clinical Manifestations: Manifestations: www.thewww.the Lancet.com Lancet.com

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Idiopathic RPF Lab FindingsIdiopathic RPF Lab Findings

• ESR and CRP increase 80-100%ESR and CRP increase 80-100%• AzotemiaAzotemia• AnemiaAnemia• Eosinophil elevation, microscopic hematuria less Eosinophil elevation, microscopic hematuria less

commoncommon• Positive ANA in 60 % with Idiopathic RPFPositive ANA in 60 % with Idiopathic RPF

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Idiopathic RPF Associations: Idiopathic RPF Associations: www.thewww.the lancet.com lancet.com

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Idiopathic RPF Management: Idiopathic RPF Management: www.thewww.the lancet.com lancet.com

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Idiopathic RPF Medical TreatmentIdiopathic RPF Medical Treatment

• Aim of treatment:Aim of treatment: Stop the progression of Stop the progression of fibroinflammatory reactionfibroinflammatory reaction

• Inhibit, relieve obstruction of ureters and other Inhibit, relieve obstruction of ureters and other retroperitoneal structuresretroperitoneal structures

• Switch off acute-phase reaction and its systemic Switch off acute-phase reaction and its systemic manifestationsmanifestations

• Prevent disease recurrence or relapsePrevent disease recurrence or relapse

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Idiopathic RPF: Drug TreatmentIdiopathic RPF: Drug Treatment

• Corticosteroids-suppress synthesis of most of the Corticosteroids-suppress synthesis of most of the cytokines involved in acute-phase reactioncytokines involved in acute-phase reaction

• Reduce inflammatory componentReduce inflammatory component• Inhibit collagen synthesis and maturationInhibit collagen synthesis and maturation• Improved symptomsImproved symptoms• Reduction size of massReduction size of mass• Resolution of obstructive complicationsResolution of obstructive complications• Optimum dose and duration not yet establishedOptimum dose and duration not yet established• Initial dose 40 – 60 mg up to two yearsInitial dose 40 – 60 mg up to two years

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Idiopathic RPF Drug: TreatmentIdiopathic RPF Drug: Treatment

• Other immunosuppressants-cyclophosphamideOther immunosuppressants-cyclophosphamide– AzothiaprineAzothiaprine– MethotrexateMethotrexate– CyclosporinCyclosporin– Cellcept (mycophenolate mofetil)Cellcept (mycophenolate mofetil)

• TamoxifenTamoxifen

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Followup of TSH CaseFollowup of TSH Case

• October, 2006, Creatinine 146, 168October, 2006, Creatinine 146, 168• Prednisone 40 mg tapered to 5 mgPrednisone 40 mg tapered to 5 mg• Tamoxifen 20 mg increased to 40 mgTamoxifen 20 mg increased to 40 mg• Norvasc and flomaxNorvasc and flomax• Diovan discontinuedDiovan discontinued

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Idiopathic RPF Followup of TSH Idiopathic RPF Followup of TSH CaseCase

• CT neck and abdomen,Sept 2006CT neck and abdomen,Sept 2006• Prominent parotid glandsProminent parotid glands• Submandibular glands unremarkableSubmandibular glands unremarkable• Bilateral hydronephrosisBilateral hydronephrosis• Infiltrative process in both collecting systems Infiltrative process in both collecting systems

bilaterally extending into proximal ureters bilaterally extending into proximal ureters bilaterallybilaterally

• Stents in both uretersStents in both ureters

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Idiopathic RPF Followup of TSH Idiopathic RPF Followup of TSH casecase

• USUS• Right kidney 11.6 cm, Left 11.2 cmRight kidney 11.6 cm, Left 11.2 cm• Very mild dilatation on right and mild hydro on Very mild dilatation on right and mild hydro on

leftleft• Solid thickening of proximal third of ureter Solid thickening of proximal third of ureter

around the stentaround the stent