Presentation on theme: "Morning Report Alan Chan, MD Med-peds PGY4"— Presentation transcript:
1 Morning Report Alan Chan, MD Med-peds PGY4 “Everything's got a moral, if only you can find it.”- Lewis Carroll, British authorAlan Chan, MDMed-peds PGY4207512
2 Chief Complaint: altered mental status and worsening edema HPI: 82yo AA woman with the usual PMHx problems of uncontrolled HTN, hyperlipidemia presents when family notes confusion for 2 weeks. Increasing. Refuses to leave apartment for past 6 weeks, keep rearranging furniture and using umbrella indoors.Increasing swelling in LE bilaterally, family has noted more in the past 2 weeks and does not think was there earlier. No leg painDecreased appetite, only walks around apartment now used to home shopping a little with family. No urinary changes she notes.Slightly hard of hearingNo chest pain, SOA. Vision fine except known cataracts. Denies weakness, F/C, fatigue, night sweats. No diarrhea, vomiting.
4 PMH: HTN – uncontrolled, hyperlipidemia, cataracts PSurgHx: L 5th toe amputation for melanomaROS: No chills, fatigue, night sweats. No wt changes.No vision changesNo rhinorrhea, sneezing; No dyspnea on exertion, no shortness of breath, wheezeNo GI issues like diarrhea or vomiting.No urinary changes.No recent illnesses.Unsure of palpitations, orthopnea at home, but patient is lying 30 deg in bed.
5 SH: No tob, EtOH, or anything else. FH: Alzheimer in both parents. Have been deceased for a long time. Family is unaware of anything else.
6 VS in ER: Temp 97.1 oral, Resp 18, BP 138/71, Pulse 94. 97% on RA General: frail appearing woman appears stated age. Confused about location, thinks it’s the Sabetes eye clinicHEENT: EOMI, PERRL, pale conjunctiva. OP clear, edentatousNeck: soft, supple, no elevated JADChest: CTA bilat, no wheezingCVS: reg regular rhythm S1, S2, no murmur
7 Abd: BS +, non TTP. No guarding. No CVA tenderness Ext: at least 2+ pitting edema to knee bilat, 2+ pulsesNeuro: CN 2-12 intact, no focal deficits. 4/5 strength but poor effort throughout.Skin: old incision site on left foot without signs of infection; no purpuric rash
8 Differential Diagnosis CC: confusion, LE swellingHPI:80yo with 2 wk of symptoms. Currently confined to home.PMH:HTN, hyperlipidemiaExam FindingsedemaDifferential Diagnosis
9 Laboratory Data CBC BMP Urinalysis Cardiac Enzymes Liver Function TestsCoagulationEndocrinologySerologyOther SerologyCytologyPathologyMicrobiologyCXREKGUltrasoundCT ScanEchoOther StudiesTruman panelClinical CourseDifferential DiagnosisDiscussion
25 EKG on admit Normal sinus rhythm Chest Lead Error Moderate voltage criteria for LVH, may be normal variantBorderline ECG
26 U/S renal with dopplers Impression:1. Right complex renal cyst with an internal nodule vs septation. Recommend triple phase CT renal imaging.2. Left simple renal cyst.3. Aortic aneurysm measuring 3.4 cm in greatest caliber below the level of the renal arteries.4. Aortic atherosclerosis.Doppler -This study shows no evidence for renal artery stenosis bilaterally. There is evidence for small 3-cm abdominal aortic aneurysm. Further clinical correlation is recommended.
27 CT studiesCT head-CHRONIC CHANGES OF PERIPHERAL CEREBROVASCULAR DISEASE ONLY. NO FINDINGS OF AN ACUTE CEREBROVASCULAR EVENT.CT chest without contrast1. Mild aneurysmal dilatation of the aortic arch.2. Atheromatous changes of the aorta.3. Granulomatous calcifications.
28 2-D Echocardiogram CONCLUSIONS: 1. Severely dilated left atrium by LA volume index calculation.2. Mild aortic valve sclerosis.3. Overall left ventricular ejection fraction is estimated at 65%.4. Mild tricuspid regurgitation.5. Grade 1 LV diastolic dysfunction.6. Mildly elevated pulmonary artery systolic pressure.
29 MAG scan Impression: 1. Normal bilateral renal perfusion. 2. Diminished renal washout bilaterally suggesting a mild degree of obstruction and associated diminished renal function (given the intermediate T1/2 max values of less than 20).
31 Clinical CourseARF, FeNa 1.8, wnl U/S except cysts, MAG scan with decreased renal fxnuremiaElevated inflammatory markers – vasculitis?Renal biopsy not done due to family wishes and question of patient to tolerate procedure.Serology shows a microscopic polyangiitis picture!
33 Vasculitis - definition presence of inflammatory leukocytes in vessel walls leading to damageMechanics still unknownPrimary or secondary to another disease processTreatment in acute phase important because early deaths are due to active disease, but later deaths may be due to treatment complications.
34 Large – Takayasu (aorta and branches) and giant cell (medium as well) ClassificationClassically by affect vessel size… but a lot of overlapLarge – Takayasu (aorta and branches) and giant cell (medium as well)Medium – polyarteritis nodosa (med and small), Kawasaski (all sizes with mucocutaneous involvement), primary CNS vasculitis (med and small without extracranial vessels)
35 Classification Small – Churg-Strauss (lung and skin muscular arteries),Wegener granulomatosis (sm and med of lung and necrotizing pauci immune glomerulonephritis in kidneys)microscopic polyarteritis/polyangiitis (like WG spectrum)Henoch-Schonlein purpura (tissue deposition of IgA immune complex)Hypersensitivity vasculitis – like above, but no IgAEssential cryoglobulinemic vasculitis
36 More!Vasculitis due to connective tissue d/o and some others due to viral infectionLots of overlap, this criteria last updated by Am College of Rheumatology in 1990, based on prospective data from the 1980s and only help distinguish from other vasculitides.Since the new ANCA testing in 1982, new classification may consider ANCA positive vs negative
37 Diagnosis Hard to diagnosis Suspect in systemic symptoms, or if can be associated in patient’s other diseases“mononeuritis multiplex” or asymmetric polyneuropathy… also seen in diabetesPalpable purpura – HSP, MPAPulmonary-renal involvement
38 Ask… Patient History and past medical Other blood work ANA Complement ANCAEMGBiopsy
39 False positivesSometimes reported in rheumatic inflammatory disease, but these were before MPO and PR3 testingANCA, typically p type, in UC, but NOT Crohn’sAlso in primary sclerosis cholangitisCystic fibrosis patients with lung disease, but non MPO
40 ANCA?? Include WG, MPA, renal limited vasculitis, Churg Strauss. The ACR criteria for WG/MPA – did not differentiate between these 2Nasal or oral inflammationAbnormal CXR with nodules, cavities, or fixed infiltratesAbnormal urine sediment (microscopic hematuria +/- rbc casts)Granulomatous inflammation on biopsy of an arteryTwo or more 88% sens, and 92% spec
41 Other classificationChapel Hill Consensus - Defines disease, but no classification and NOT diagnostic, ANCA is not includedEuropean Medicines Agency algorithm – must exclude Churg Strauss and WGClassify by??? --- MP0 ANCA, PR3 ANCA, and seroneg ANCAOne study suggests specificity of antibodies to PR3/MPO as high as 98.4%!
43 LabsANCA are directed against the granular and lysosomal parts of neutrophils and monocytesIIF (indirect immunofluor) stain or enzyme linked immunosorbent assay in a cytoplasmic or perinuclear patternTarget of cANCA is proteinase-3 and pANCA is myeloperoxidase
44 ANCA Associated Vasculitis Genetic factors on HLA genes of chr6Silica – bad stuff. From 22-46% of AAV have had exposure. Brings immune response and inflammatory reactionsBacterial infection – S. aureusDrugs – also bad – PTU (most common), hydralazine, D-penicillamine, and minocycline.
45 Microscopic Polyangiitis Originally described in a case series as a type of polyarteritis nodosa with RPGN (rapidly necrotizing glomerulonephritis) and sometimes lung hemorrhage – but has capillary, venule, or arterioles involvementMale slightly > femaleAvg age of onset > 50 yoGeneral symptoms may occur months to years before acute phase.
46 Treatment If don’t treat, prognosis is bad Corticosteroids with cyclophosphamide – up to 90% go into remissionBut… Cytoxan long term use side effects – secondary infections, bone marrow suppresion, hemorrhagic cystitis, and bladder cancer.Recommend prophylactic TMP-SMX for PJPOther trials looking at MTX, rituximab, plasma exchange, etanercept, mycophenolate mofetil, azathioprine
48 Question of the Day53 yo man with 6 wk hx of fatigue, fever, numbness and tingling in the hands and feet, mild abd pain, and a nodular rash on the hands, arms, and legs. Weight lost 9.0 lb.Exam – T °F, 150/82, HR 96 RR 14. CV exam normal. Hands reveals numerous subcutaneous nodules. There is shotty cervical, axillary, and inguinal LAP. Abd - hepatomegaly and mild diffuse tenderness NO rebound. testes are tender. No synovitisHgb 8.6, wbc15.2, plt 523, esr 113, AST 85, ALT 73HBsAg POS, HBeAg POSANCA, ANA, HIV, anti-Hbs, anti-HCV all negSkin bx – necrotizing vasculitisA cytoxanB predC pred with cytoxanD pred with lamivudine
49 D – pred with lamivudine Polyarteritis nodosa in acute setting with hep B needs short term high dose corticosteroid with long term anti-viral
50 ReferencesMKSAP 14/15Uptodate.com “Classification of and approach to the vasculitides in adults”, “Clinical manifestations and diagnosis of Wegener's granulomatosis and microscopic polyangiitis “. Accessed 6/10/2011.Chen, M, Kallenberg CG. ANCA-associated vasculitides—advances in pathogenesis and treatment. Nat Rev Rheumatol. 2010; 6( ).Kallenbery CG. The Last Classification of Vasculitis. Clinic Rev Allerg Immunol ; 35(5-10)Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic Polyangiitis: Clinical and Laboratory findings in 85 patients. Arthritis and Rheumatism ; 42:3 ( ).