Transcript case conferance (1)

Case Conference
History
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56 year old African American female
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Was transferred from outlying hospital for:
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B/L LE infected ulcers (due to heating pad) and possible
amputation.
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Increased output from tracheostomy.
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Outpatient HD set up.
She was recently started on HD at outlying facility.
Previous Hospitalization
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She went at outlying facility in early Aug with shortness of breath and
generalized edema.
Diagnosed with Acute on chronic systolic heart failure.
She also developed Acute Kidney Injury and was started in HD for
fluid removal.
From reviewing the records, We found a CT scan Abdomen from July
which was done due to gross hematuria.
Kidneys, ureters and bladder was normal in that.
Creatinine trend
HD
DC
Baseline Creatinine: 0.9
Past Medical History:
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Complicated CABG surgery in 2011 with prolonged intubated and subglottic
stenosis s/p tracheostomy.
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DM
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Ischemic Cardiomyopathy
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COPD
Social History:
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Non smoker, non drinker.
Family History:
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Positive for DM and HTN. Sister had HIV related renal disease (was on HD).
Medications
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Amiodarone 200mg QD
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Metoprolol 50 mg QD
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Metformin
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Omeprazole
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Iron sulfate
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Insulin
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Furosemide 40 mg BID
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Digoxin 125 mcg QD
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Imdur
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Ibuprofen (3-4 times a month 800 mg)
Exam
BP 89/56 | Pulse 89 | Temp 98.2 °F | Resp 15 | Wt 120.9 kg | SpO2 94%
Body mass index is 43.02 kg/(m^2).
Constitutional: well developed, well nourished, in no apparent distress
Neck: tracheostomy present
Lungs: decreased breath sounds at bases
Cardiac: regular rate and rhythm, S1, S2 normal, no murmur, click, rub or gallop
Abdomen/GI: soft, non-tender; bowel sounds normal; no masses, no organomegaly
Extremities: b/l LE bandaged due to wounds. Wounds were deep and foul smelling.
Skin: no rash
Labs
138
97
23
3.8
27
2.12
Calcium: 8.8, Phos 3.0
T. Protein: 6.4, Albumin: 2.4
98
11.5
7.0
225
37.2
UA showed pH: 6.5, Sp Gr: 1.015, Protein: 30, WBCs: 392, RBCs: 11, No casts
Urine Protein/Crt: 0.4 gm/gm
Differential Diagnosis
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Prerenal: Decreased kidney perfusion sec to severe systolic heart
failure (EF <10%), sepsis related hypotension
Renal:
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GN: FSGS, IgA nephropathy, Post infectious, Vasculitis,
amyloidosis due to chronic infection, HIV, Hep C
membranoprofilerative, DM, Anti-GBM disease
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AIN sec to recent antibiotics.
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ATN from long standing hypotension.
Postrenal
Follow up Labs
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Anti ds DNA, C-ANCA, P-ANCA, HIV, Hep Panel: negative
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ANA: 1:80 Nucleolar
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Haptoglobin: 179. LDH: 258
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Kappa/Lambda: 1.42 normal
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C3, C4: Normal
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SPEP/UPEP: negative
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Urine Eosinophils: None
US Kidneys: No evidence of obstructive lesion or other concerning
abnormality of the kidneys.
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Anti-GBM Antibody (9/25/13):
Positive: 49 (By ELISA)
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<19 AU/ML..................... Negative
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20-25 AU/mL ................. Equivocal
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26 AU/mL or Greater ...... Positive
Repeat one was done on 10/1/13: Positive: 32
Progress
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During her Hospitalization, We started her on diuretics.
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She didnt receive any Dialysis since admission.
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She was also diagnosed with LV thrombus and is on anticoagulation.
Questions
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Does our patient has Anti-GBM disease or the positive anti-GBM Ab
is a false positive test?
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Should we proceed for biopsy?
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If biopsy is positive, Should we consider treatment?
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Do we treat patients with positive Anti-GBM but normal renal
function?
Anti-GBM Antibody Disease
Anti-GBM antibody disease is a disorder in which circulating
antibodies are directed against an antigen intrinsic to the
glomerular basement membrane (GBM), thereby resulting in
acute or rapidly progressive glomerulonephritis that is typically
associated with crescent formation.
Type IV Collagen
N Engl J Med 2003; 348:2543-2556, June 19, 2003
N Engl J Med 2003; 348:2543-2556June 19, 2003
Clinical Presentation
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Young men in late 20s and in men and women over 60 years of age.
In the younger age group, the disease is usually eruptive, with
hemoptysis, anemia, cough, fever, dyspnea, hematuria, nonnephrotic proteinuria, and red-cell casts. CXR shows diffuse alveolar
infiltrates.
Goodpasture's syndrome is generally detected earlier in patients
who present with lung hemorrhage, and such patients may do better
than those who present with silent renal injury alone.
Presentation with oliguria is a particularly bad sign
Diagnosis
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Blood Tests:
Antiglomerular basement membrane antibodies
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Based on diagnostic case-control study.
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103 serum samples were analyzed.
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34 serum samples from 19 patients with anti-GBM antibody disease.
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41 serum samples from disease controls (22 with Wegener granulomatosis, 15 with
microscopic polyangiitis, 1 with Churg-Strauss syndrome, 2 with SLE, 1 with
idiopathic pulmonary fibrosis)
28 samples from healthy controls
Nephrol Dial Transplant 2006 Feb;21(2):397 full-text
Results
Nephrol Dial Transplant 2006 Feb;21(2):397 full-text
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Western Blot
Western blot screening for antibody against the human Goodpasture antigen (alpha3(IV) NC1)
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Urine tests:
Active urine sediment with red blood cells, white blood cells, and red blood cell casts
occurs with glomerulonephritis
Hematuria
Non-nephrotic range proteinuria
Treatment
Positive Anti-GBM antibody
JASN November 1, 1999 vol. 10 no. 11 2446-2453
Case Reports
Diabetic nephropathy with interstitial nephritis presenting with a false-positive anti-GBM
antibody.
Clin Nephrol. 2002 May;57(5):381-5
An unusual case of pulmonary-renal syndrome associated with defects in type IV
collagen composition and anti-glomerular basement membrane autoantibodies.
Am J Kidney Dis. 2005;45(4):743.
A Friday afternoon case of apparent anti-glomerular basement nephritis. (The blocking
reagent of the ELISA kit from Imtec contained bovine serum albumin, suggesting the
reason for the false-positive result)
Nephrology Dialysis Transplantation Volume 21, Issue 8Pp. 2328-2330
Do we treat patients with positive Anti-GBM but
normal renal function?
Anti-glomerular basement membrane (GBM)-antibodymediated
disease with normal renal function
Nephrol Dial Transplant. 1998 Apr;13(4):935-9.
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All got same treatment: patients with no renal impairment survived
with normal creatinine and no need for dialysis. Patients with renal
impairment are on either dialysis or got renal transplant.
Br Med J (Clin Res Ed). 1986 February 1; 292(6516): 301–304.
Clinical analysis of anti glomerular basement
membrane disease with normal renal function
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Total no. of patients: 35 (6 with normal renal function)
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5/6 had microhematuria and proteinuria
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4/6 patients had linear deposition in immunofloresence.
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5/6 got treatment with immunosuppresives and plasmapheresis
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These 6 patients with normal renal function were followed up for 12133 months, among whom 4 patients achieved complete remission
and 2 had mild proteinuria and microhematuria.
2011 Aug;33(4):432-5. doi: 10.3881/j.issn.1000-503X.2011.04.016.
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How should we manage our patient?