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Z A CD Alan Chan, MD Med-peds PGY4 “Everything's got a moral, if only you can find it.” - Lewis Carroll, British author 207512.

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Presentation on theme: "Z A CD Alan Chan, MD Med-peds PGY4 “Everything's got a moral, if only you can find it.” - Lewis Carroll, British author 207512."— Presentation transcript:

1 Z A CD Alan Chan, MD Med-peds PGY4 “Everything's got a moral, if only you can find it.” - Lewis Carroll, British author 207512

2 Z A CD 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 pain Decreased appetite, only walks around apartment now used to home shopping a little with family. No urinary changes she notes. Slightly hard of hearing No chest pain, SOA. Vision fine except known cataracts. Denies weakness, F/C, fatigue, night sweats. No diarrhea, vomiting. Chief Complaint: altered mental status and worsening edema

3 Z A CD Medications Metoprolol 25 mg bid Detrol LA 2 mg Clonidine 0.2 mg bid Verapamil 240 mg bid Lisinopril 40 mg Lipitor 10 mg Claritin 10 mg KCl 20 mEq Allergies NKDA

4 Z A CD PMH: HTN – uncontrolled, hyperlipidemia, cataracts PSurgHx: L 5 th toe amputation for melanoma ROS: No chills, fatigue, night sweats. No wt changes. No vision changes No rhinorrhea, sneezing; No dyspnea on exertion, no shortness of breath, wheeze No 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 Z A CD 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 Z A CD 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 clinic HEENT: EOMI, PERRL, pale conjunctiva. OP clear, edentatous Neck: soft, supple, no elevated JAD Chest: CTA bilat, no wheezing CVS: reg regular rhythm S1, S2, no murmur

7 Z A CD Abd: BS +, non TTP. No guarding. No CVA tenderness Ext: at least 2+ pitting edema to knee bilat, 2+ pulses Neuro: 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 Z A CD Differential Diagnosis CC: confusion, LE swelling HPI: 80yo with 2 wk of symptoms. Currently confined to home. PMH: HTN, hyperlipidemia Exam Findings edema

9 Z A CD Laboratory Data CBC BMP Urinalysis Cardiac Enzymes Liver Function Tests Coagulation Endocrinology Serology Other Serology Cytology Pathology Microbiology CXR EKG Ultrasound CT Scan Echo Other Studies Truman panel Clinical Course Differential Diagnosis Discussion

10 Z A CD Please Press to Return

11 Z A CD CBC 12.5 8 24.6 285 Gran 84, L 11 M 5 % MCV 77 (80-99) RDW 19 (<14.5)

12 Z A CD BMP 139 4 100 23 92 5.5 123 AG 13 (3-15) Ca 8.7 (8.8-10.5) Mg 2.1 (1.8-2.5) PO 4 2.9 (2.4-4.7)

13 Z A CD Urine Analysis - cath Microscopic 5-10 wbc 10-25 rbc 2-5 Sq epi tr mucus Upro/Cr- 1 coloryellow sp gr1.020 pH5 bloodmod ketoneX gluX prot30 LEX nitriteX urobil0.2 bilix

14 Z A CD Cardiac Enzymes 1 st – TnI 0.06

15 Z A CD Liver Function Tests AST19 (15-41) ALT29 (7-35) Alk Phos32 (32-91) Albumin2.2 (3.5-4.8) T Bilirubin0.6 (0.3-1.2) D Bilirubinxx (0.1-0.5) Protein 6.1 (6.1-7.9)

16 Z A CD Coagulation and stuff PTTx (21-33) PTx (10.3-13.0) INRx Retic 2.2

17 Z A CD Endocrinology TSH 2.4 (0.34-5.6) Free T4X (0.6-1.6) Folate and B12 normal Epo 8.3 (4.2-27.8) A1C 4.9 Ferritin 773 !!

18 Z A CD Serology MPO >100 PR3 <3.5 cANCA <1:20 pANCA > 1:320 Anti GBM IgG neg

19 Z A CD Pathology Normochromic normocytic anemia.

20 Z A CD Other Serology ESR – 118 CRP 11 Ammonia - <16 C3, C4 wnl LDH – 247 (98-192) Chol panel - wnl

21 Z A CD Microbiology UCx Mixed Gram positive flora isolated. BCx neg

22 Z A CD

23 Chest X-ray 1. No focal airspace disease. 2. Remote granulomatous disease. 3. Borderline cardiomegaly. 4. Prominence of the ascending aorta which may be seen in aortic valvular disease.

24 Z A CD

25 EKG on admit Normal sinus rhythm Chest Lead Error Moderate voltage criteria for LVH, may be normal variant Borderline ECG

26 Z A CD 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 Z A CD CT head- CHRONIC CHANGES OF PERIPHERAL CEREBROVASCULAR DISEASE ONLY. NO FINDINGS OF AN ACUTE CEREBROVASCULAR EVENT. CT chest without contrast 1. Mild aneurysmal dilatation of the aortic arch. 2. Atheromatous changes of the aorta. 3. Granulomatous calcifications. CT studies

28 Z A CD 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 Z A CD 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).

30 Z A CD Truman panel Cbc, bmp, lft, Cardiac enzymes, CXR, CT head…. All already drawn! BNP - 425

31 Z A CD Clinical Course ARF, FeNa 1.8, wnl U/S except cysts, MAG scan with decreased renal fxn uremia Elevated inflammatory markers – vasculitis? Renal biopsy not done due to family wishes and question of patient to tolerate procedure. Serology shows a microscopic polyangiitis picture!

32 Z A CD Discussion - Goals Overview of vasculitis Classification MPA Diagnosis Treatment MKSAP Q?

33 Z A CD Vasculitis - definition presence of inflammatory leukocytes in vessel walls leading to damage Mechanics still unknown Primary or secondary to another disease process Treatment in acute phase important because early deaths are due to active disease, but later deaths may be due to treatment complications.

34 Z A CD Classification Classically by affect vessel size… but a lot of overlap Large – 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 Z A CD 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 IgA –Essential cryoglobulinemic vasculitis

36 Z A CD More! Vasculitis due to connective tissue d/o and some others due to viral infection Lots 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 Z A CD 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 diabetes Palpable purpura – HSP, MPA Pulmonary-renal involvement

38 Z A CD Ask… Patient History and past medical Other blood work ANA Complement ANCA EMG Biopsy

39 Z A CD False positives Sometimes reported in rheumatic inflammatory disease, but these were before MPO and PR3 testing ANCA, typically p type, in UC, but NOT Crohn’s Also in primary sclerosis cholangitis Cystic fibrosis patients with lung disease, but non MPO

40 Z A CD ANCA?? Include WG, MPA, renal limited vasculitis, Churg Strauss. The ACR criteria for WG/MPA – did not differentiate between these 2 –Nasal or oral inflammation –Abnormal CXR with nodules, cavities, or fixed infiltrates –Abnormal urine sediment (microscopic hematuria +/- rbc casts) –Granulomatous inflammation on biopsy of an artery Two or more 88% sens, and 92% spec

41 Z A CD Other classification Chapel Hill Consensus - Defines disease, but no classification and NOT diagnostic, ANCA is not included European Medicines Agency algorithm – must exclude Churg Strauss and WG Classify by??? --- MP0 ANCA, PR3 ANCA, and seroneg ANCA One study suggests specificity of antibodies to PR3/MPO as high as 98.4%!

42 Z A CD Not bad, but could be better

43 Z A CD Labs ANCA are directed against the granular and lysosomal parts of neutrophils and monocytes IIF (indirect immunofluor) stain or enzyme linked immunosorbent assay in a cytoplasmic or perinuclear pattern Target of cANCA is proteinase-3 and pANCA is myeloperoxidase

44 Z A CD ANCA Associated Vasculitis Genetic factors on HLA genes of chr6 Silica – bad stuff. From 22-46% of AAV have had exposure. Brings immune response and inflammatory reactions Bacterial infection – S. aureus Drugs – also bad – PTU (most common), hydralazine, D-penicillamine, and minocycline.

45 Z A CD 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 involvement Male slightly > female Avg age of onset > 50 yo General symptoms may occur months to years before acute phase.

46 Z A CD Treatment If don’t treat, prognosis is bad Corticosteroids with cyclophosphamide – up to 90% go into remission But… Cytoxan long term use side effects – secondary infections, bone marrow suppresion, hemorrhagic cystitis, and bladder cancer. Recommend prophylactic TMP-SMX for PJP Other trials looking at MTX, rituximab, plasma exchange, etanercept, mycophenolate mofetil, azathioprine

47 Z A CD Treatment – may base on severity

48 Z A CD Question of the Day 53 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 100.3 °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 synovitis Hgb 8.6, wbc15.2, plt 523, esr 113, AST 85, ALT 73 HBsAg POS, HBeAg POS ANCA, ANA, HIV, anti-Hbs, anti-HCV all neg Skin bx – necrotizing vasculitis A cytoxan B pred C pred with cytoxan D pred with lamivudine

49 Z A CD D – pred with lamivudine Polyarteritis nodosa in acute setting with hep B needs short term high dose corticosteroid with long term anti-viral

50 Z A CD References MKSAP 14/15 Uptodate.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(653-664). Kallenbery CG. The Last Classification of Vasculitis. Clinic Rev Allerg Immunol. 2008; 35(5-10) Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic Polyangiitis: Clinical and Laboratory findings in 85 patients. Arthritis and Rheumatism. 1999; 42:3 (421-430).

51 Z A CD 11 and done!


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