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My Interesting Case

My Interesting Case. Janet M. Roscoe, MDCM, FRCPC, FACP University of Toronto The Scarborough Hospital. Submandibular Gland Swelling and Acute Renal Failure. 47 Year old Male Well until late October 2005 Travelled to Cancun, Mexico, 2 nd week of October

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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

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