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My Interesting Case

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

  2. Submandibular Gland Swelling and Acute Renal Failure • 47 Year old Male • Well until late October 2005 • Travelled to Cancun, Mexico, 2nd week of October • Developed bilateral, nontender swelling in both submandibular areas, late in October, 2005 • Saw ENT specialist-Dx “inflammatory process in the submandibular gland” • 2 courses of antibiotics gave no improvement

  3. Submandibular Gland Swelling & ARF • Continued to feel well but submandibular swelling continued until March 2006 • Developed symptoms of “sinus congestion” plus epistaxis and feeling of “fever” • Tx by family physician with decongestant • Denied deafness or ear pain but he felt his ears weren’t “popping” back as quickly as usual when flying • Chest X-ray done & reported as normal

  4. SMG Swelling and ARF • Referred by ENT to Rheumatology for assessment for possible Sjogren’s syndrome • BP noted to be elevated (February 2006) • Patient started on calcium channel blocker and a beta blocker • Serum creatinine elevated at 180, 200

  5. SMG Swelling & ARF • Referred to Nephrology • Seen in consultation on May 3rd, 2006 • Further Hx: Patient has been followed by a cardiologist downtown for a “bulge” in his aorta • Presumably an aortic aneurysm, stable for ten years (measuring 44 mm) • Has been told it might be a congenital abnormality

  6. SMG & ARF HX. Continued • Headaches since starting BP meds • “Tearing” of eyes but no redness or pain • No change visual acuity • No joint pain or ankle swelling • Recent increased shortness of breath(attributes it to his past hx of asthma as a child) • SOB was significant to cause his family MD to order the chest X-ray in March

  7. SMG & ARF Hx continued • Increased urine frequency since March 2006 • now voiding q2hours day and night • Urine colour is pale, never red or brown • No dysuria, no flank or abdominal pain • Family hx-father died age 59 of liver cancer • 2/3 brothers had premature CAD with CABG

  8. SMG & ARF Physical Exam • BP 136/88 • Bilateral hard submandibular swellings • Palpable liver 4 cm below costal margin, firm, smooth • Dx Impression: Systemic vasculitis, probably Wegener’s granulomatosis • Plan-Vasculitis w/u • Ultrasound and renal biopsy

  9. SMG & ARF Lab Results • Nuclear Renal Scan-May 5th, 2006 • Decreased blood supply; 25% function in left kidney • Good function of right kidney (75%) but holdup of activity in upper pole calyx • Abdominal Ultrasound May 9th, 2006 • Abdominal Aorta unremarkable • Fatty infiltration of liver-no focal lesions • Right kidney large-13.4 cm long • Mild to moderate right hydronephrosis

  10. SMG & ARF Abdominal US Continued • Right renal pelvis mildly distended along with proximal portion of the ureter, debris or solid material located in the proximal portion of the right ureter • Left kidney enlarged, 13.6 cm • Mild hydronephrosis and distention of proximal portion of the left ureter up to 2.2 cm and it also contains either debris or solid material • No cortical thinning • Prostate mildly enlarged • Recommend exclusion of solid lesion such as transitional cell carcinoma

  11. SMG & ARF Lab Results Continued • Vasculitis workup including c and p ANCA, anti DNA antibody, complements, angiotensin converting enzyme all negative • But ESR is 121 • Urine protein and microalbumin/creatinine ratio normal • Urinalysis negative for blood

  12. SMS & ARF • Urgent referral to urologist delayed because patient had to travel out of town • Seen by urologist, May 24th and cystoscopy, PSA, repeat imaging planned • Creatinine 174, PSA normal • Flexible cystoscopy June 13th, 2006 showed mild prostatic enlargement with inflammation consistent with prostatitis • CT (noncontrast) ordered for further workup of other causes of hydronephrosis including retroperitoneal fibrosis

  13. SMG & ARF: CT Scan June 30, 2006 • CT Result • Kidneys inhomogenous and large • Mild to moderate bilateral hydronephrosis • Marked enlargement and thickening of the proximal ureters. • Ureters are high in density in the proximal third

  14. SMG & ARF CT Scan • Cause for findings uncertain but could include bilateral transitional cell carcinomas or blood clots. Infectious debris cannot be excluded. • Inflammatory processes in the ureters could include ureteritis cystica or leukaplakia. • No discrete renal masses noted. • No evidence of retroperitoneal, pelvic or inguinal lymphadenopathy. • Vascular structures are within normal limits

  15. SMG & ARF • Returns to my office on July 10th, 2006 • Now metallic taste in his mouth, severe fatigue, pruritis • Symptoms suggestive of uremia • Tenderness in left flank • Blood work July 11, 2006-creatinine 411, hemoglobin 110, potassium 5.1 and bicarb 16 • Sinus X-ray-Mucosal swelling of left maxillary sinus with suggestion of retention cyst • Chest Xray-normal

  16. SMG & ARF • Admitted to SGH, July 12th, 2006 • Retrograde pyelography-bilateral hydronephrosis • Ureters both show diffuse irregularity • Bilateral stents inserted

  17. SMG & ARF • Biopsy submandibular mass, July 12, 2006 • Chronic inflammation • Numerous plasma cells • Lymphocytes • Scattered lymphoid follicle • Germinal cell formation • Marked fibrosis and parenchymal atrophy • Dx- chronic sialadenitis with marked fibrosis

  18. MRI Abdomen and Pelvis • July 13, 2006 • Extensive infiltration of the renal hila bilaterally • Extending into the renal pelvis bilaterally and along the ureters • Infiltrative process extends into the perirenal fat on the right side • Infiltration or thickening of the left portal vein • Subtle infiltration of the pelvic fat surrounding the internal iliac arteries and veins and extending to the bladder base • No adenopathy • Pericardial effusion-maximum thickness of 1.5 cm • Provisional diagnosis retroperitoneal fibrosis but seen by Infectious Disease-Consider TB, histoplasmosis, and Brucellosis

  19. Patient Radiology

  20. Patient Radiography

  21. Patient Radiography

  22. Patient Radiology

  23. Patient Radiology

  24. Patient Radiology

  25. Patient Radiology

  26. Diagnosis: • Dr. Tabo Sikenata • Did a literature Review and made the diagnosis • Multifocal Idiopathic Fibrosclerosis

  27. The Journal of Rheumatology, 2006; 33: 358-361 (February) Idiopathic RPF

  28. Multifocal Idiopathic Fibrosclerosis Case 1: • 69 year old male • 30 year history of chronic intermittent abdominal pain, pseudoobstruction, cholangiolar fibrosis, right renal masses with hydronephorisis • Biopsies showed marked fibrosis with chronic inflammation typical of retroperitoneal fibrosis • Bilateral edema of eyes and diplopia, Left submandibular gland swelling • Dx multifocal idiopathic fibrosclerosis • Tx with prednisone and cylcosporine • Relapsed when cyclosporine withdrawn and remitted when cyclosporine reintroduced

  29. Multifocal Idiopathic Fibrosis Case 2 • 74 year old male • 5 year history of left upper quadrant pain • Gangrenous gallbladder-abdominal CT shows enlarged pancreas with localized mass. • Pre-aortic mass 3 years later • Biopsy dense fibrosis with mild patchy chronic inflammation typical of idiopathic retroperitoneal fibrosis. Mass encases ureter at up junction • Develops bilateral enlargement of submandibular glands • Bx shows chronic sclerosing sialadenitis • Responds to prednisone and cyclosporine

  30. Multifocal Idiopathic Fibrosclerosis Literature Review • www.the lancet.com Vol. 367 January 21, 2006 Retroperitoneal Fibrosis, Vaglio, Salvarani and Buzio • Range of diseases characterised by the presence of a fibro-inflammatory tissue • Usually surrounding abdominal aorta and the iliac arteries and extending into the retroperitoneum to envelop neighbouring structures-eg the ureters

  31. Multifocal Idiopathic Fibrosclerosis • First described by French urologist in 1905 • Next described in English by Ormond who described two cases in 1948 • Incidence 1:200,000-1:500,000 • Prevalence 1-2 per 100,000 • Usual presentation-vague, poorly localized pain over flank, low back and abdomen or nonspecific systemic complaints-malaise, anorexia, weight loss, moderate pyrexia, nausea and vomiting • Acute phase reactants are often elevated • Obstructive uropathy and renal insufficiency may be present secondary to fibrous encasement of the ureter

  32. Multifocal Idiopathic Fibrosclerosisis: Major Causes of 2ry RPF (UpToDate, 2007)

  33. Multifocal Idiopathic Fibrosclerosis • Idiopathic RPF • Umbrella term-chronic periaortitis • Idiopathic retroperitoneal fibrosis • Inflammatory abdominal aortic aneurysm • Perianeurysmal retroperitoneal fibrosis

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

  35. Multifocal Idiopathic Fibrosclerosis • IRPF aorta not dilated, surrounding fibro-inflammatory tissue might or might not encase adjacent structures • Inflammatory abdominal aortic aneurysm-tissue develops around a dilated aorta but does not cause obstructions • Perianeurysmal rpf involves an inflammatory aneurysm-surrounding tissue of which entraps adjacent organs • Distinguish non-aneurysmal from aneurysmal forms • In the last 20 years introduction of medical therapy mainly based on corticosteroids, and imaging techniques such as CT and MRI has greatly improved patient outcome

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

  37. Idiopathic RPS: Diagnosis • No standard diagnostic criteria • CT or MRI scans are modalities of choice for diagnosis and followup • Soft tissue mass surrounding the abdominal aorta and iliac arteries with possible encasement of neighbouring structures such as ureters and inferior vena cava suggests diagnosis of RPF • Histological exam of RP tissue usually needed when mass shows atypical locations-pelvic, peripancreatic or if malignancy or infectious disease is suspected

  38. Idiopathic RPF • Pathogenous theory by Mitchinson and Parums-chronic periaortitis is a range of diseases including idiopathic retroperitoneal fibrosis • Characterized by advanced aortic atherosclerosis, medial thinning, pronounced adventitial and perioaortic inflammation and fibrosis • Local inflammatory reaction to oxidized low-density lipoproteins (LDL) and ceroid (a lipooproteic polymer resulting from LDL oxidation within plaque macrophages) • Aortic media breached or thinned-oxidized lipids presented to T and B lymphocytes

  39. Idiopathic RPF • Self perpetuating inflammatory response occurs • Occurrence of constitutional symptoms, raised acute phase reactants, positive antibodies and associated autoimmune disease involving other organs suggest iRPF may be a manifestation of systemic autoimmune disease and not an exaggerated local reaction to atherosclerosis • May be an association with HLA-DRB1*03, an allele linked to various autoimmune diseases suggested, as type 1 diabetes, myasthenia gravis and systemic lupus erythematosis

  40. Idiopathic RPF • Malignant disease associated RPF results from exuberant desmoplastic response to retroperitoneal metastases-from ca of prostate, breast, colon or retroperitoneal primary tumour like Hodgkin’s or Non-Hodgkin lymphoma, sarcoma or carcinoid (may produce RPF by humeral factors without metastasing) • RPF secondary to infections is caused by local spread of a contiguous infectious process-eg spinal or paraspinal abcesses in tuberculosis

  41. Idiopathic RPF • Macroscopic appearance-white hard retroperitoneal plaque of varying thickness surrounding retroperitoneal structures • Rarely extends to mesenteric root or posteriorly to spinal cord • May appear as poorly circumscribed retroperitoneal mass • Microscopic appearance-sclerotic tissue invasion • Infiltrated with mononuclear cells • Early stage edematous tissue and highly vascular, chronic inflammation • Later pronounced sclerosis and scattered calcifications