10/26/2015

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There’s Always a First TimeA Clinical Problem Solving Case

Gurpreet Dhaliwal, MD

Professor of Medicine University of California, San Francisco

10/26/2015

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Ground Rules for CPS Exercise

Goop has never heard these cases Not a trivial undertaking

Goal is to make the thought process of a master clinician transparent It’s not magic

You don’t have to “know everything”

“Getting it right” is cool, but relatively unimportant in the grand scheme

Enjoy – this is the fun part of medicine

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Ockham’s Razor vs. Hickam’s Dictum

“Entities must not be multiplied beyond necessity.”

-- William of Ockham

“Patients can have as many diseases as they damn well please.” -- John Hickam

History A 73-year-old man with a history of COPD and a

mechanical MVR/porcine AVR (on coumadin) was admitted to an outside hospital for several acute episodes of dyspnea over the prior month.

He denied cough, CP, palpitations, orthopnea, or fever. He did endorse mild abdominal distension.

He had no prior history of PE, pneumonia, or heart failure. He had never been hospitalized for COPD. His valve surgery was 5 years earlier. He claimed to be taking his coumadin. No travel history documented.

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ED Assessment and Exam The patient was noted to be wheezing and in mild

respiratory distress

Afebrile, RR 20, O2 97% RA, BP 85/57, which responded to fluids

Initial ABG: 7.46/42/63 (RA)

WBC 9.7, diff normal

CXR unremarkable

A CT scan was neg for PE and volume overload; it showed only mild bibasilar atelectasis

ED Management

The patient was treated for a COPD exacerbation

He received a steroid burst, duonebs, and azithromycin

He improved over the first 6-12 hours but was admitted for further treatment and observation

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What do you think is going on?

1. Sounds like a routine case of COPD exacerbation. Is Bob trying to fool Goop by giving him a bread and butter VA case?

2. Must have something to do with the valves

3. I remember one of my profs from med school saying something like, “All that wheezes isn’t asthma,” but I can’t remember what it is

4. Did he say “no travel history documented”?

5. Did he also say “the patient claimed to be taking his coumadin”?

Goop’s Initial Thoughts

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Hospital Course The patient’s abdominal distension (a mild complaint on

admission, not confirmed on exam) worsened over the first 2-3 days of hospitalization

A KUB on hospital day 4 showed dilated bowel loops consistent with ileus

An abdominal CT was obtained: no evidence of ileus or bowel abnormalities (his symptoms had improved)

On hospital day 6, his breathing took a marked turn for the worse – with severe dyspnea and tachypnea

A diagnosis of respiratory failure was made

The patient was taken to the ICU and intubated

Now I’m worried about…

1. Bowel ischemia

2. Churg-Strauss vasculitis

3. Inflammatory bowel disease

4. Lupus

5. Sepsis and ARDS

6. A hypercoagulable state and in-situ thromboses

7. That Donald Trump could really be our next president

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ICU Course Repeat CXR unchanged from admission

TTE showed no evidence of heart failure, valvulardysfunction, or vegetations

Antibiotics were broadened to vanco and tigecycline

Blood cultures from the time of the deterioration grew enterococcus faecalis

Vanco was changed to linezolid

UA was negative

PICC line felt likeliest source of bacteremia and d/ced

Aggressive COPD rx led to improvement, extubated on hospital day 14

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Post-Intubation Course

The patient complained, for the first time, of back pain and lower extremity weakness

On further questioning, he noted that he had had progressive leg weakness for several weeks

Spinal imaging showed a T5-6 burst fracture with retropulsion and mild central canal narrowing, along with soft tissue fullness around the spine, c/w necrotic mass or abscess

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Wow, that’s not good. Now I’m worried about…

1. Syphilis

2. Tuberculosis

3. Lymphoma

4. Cocci

5. Endocarditis

6. MRSA

7. Sorry, I’m still worried about Donald Trump

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Post-Intubation Course

Cocci serum titers sent and returned weakly positive Started on fluconazole

Soon, cocci immunodiffusion and comp fix returned negative, so fluconazole d/c’ed

Patient transferred from community hospital to UCSF neurosurgery service

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Past Medical History (obtained at UCSF admission)

COPD (no prior PFTs, hospitalizations) HTN Bioprosthetic AVR & Mechanical MVR (both placed 2

yrs earlier) Knee osteoarthritis, treated with NSAIDs, injections Hypothyroidism

SH: Originally from Guatemala, with frequent trips back. Single, lives with son. 20 pack year tobacco hx, quit in 1992. 2 cans of beer/wk. No elicits. Used to work in a warehouse; now retired.

FH: Son with pulmonary TB rxed for at least 6 months (more than 20 years ago). No other history of cardiac, pulmonary, infectious, rheum, heme, bone disorders.

NKDA

Home Meds:CoumadinCarvedilolLisinoprilFurosemide Simvastatin Levothyroxine OmeprazoleVitamin D

Meds on transfer: Fluconazole Budesonide nebs Furosemide Aspart insulin SSLevothyroxineFamotidineDocusateSennaPolyethylene glycolFerrous sulfate

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Physical Exam After TransferVITALS: 36.9 °C, 98, 159/43, 20, 95 % RA GENERAL: Deeply sedated.

HEENT: NC/AT. Neck supple. No JVD.

CVR: RRR. Mechanical second heart sound. No m/r/g.

PULM: Clear to ascultation bilaterally.

ABD: Soft, non-tender. Distended and tympanitic.

MSK: No edema. Warm distally.

NEURO:

After lightening sedation, the patient was A+O x 2.

PERRL, EOMI.

5/5 strength in face and BUE with no pronator drift. No movement in LE’s.Absent rectal tone.

Nl sensation to light touch and pain in bilat UEs. Sensory level at T3~T4.

0+ reflexes in patella/ankles bilaterally; UE reflexes normal.

Labs

WBC: 16.6Hgb: 13.1Plt: 411

Na: 129K: 4.4Cl: 95CO2: 25BUN: 7Cr: 0.5Glucose 126Ca: 9.2PTT 37.4, INR 1.9

CRP 112

ABG: 7.44/41/382 (60% FiO2) Lactate 0.8

Blood, urine cultures sent

EKG: LVH with repolarization abnormality

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CXR at time of transfer

Low lung volumes. RLL patchy consolidation. Diffuse indistinct pulmonary vascularity.

Studies

KUB: Nonspecific bowel gas pattern.

TTE:1. Normal ventricular size and EF. 2. Severe concentric LVH. Paradoxical septal motion. 3. Mod LAE. Nl right atrium.4. Mechanical mitral prosthesis normal. Bioprosthetic

aortic valve normal.5. Mitral prosthesis precludes the accurate

evaluation of diastolic function.6. PASP estimated 12-16 mmHg.7. No pericardial effusion.

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MRI Spine – T2

MRI Spine – T2

Vertebral collapse at T5, 50% height loss at T6. Retropulsionat T5 leading to canal stenosis. Abnormal cord signal T7 on up, with moderate cord compression at T5-6. Pre-syrinx (fluid filled cavity within cord) formation.

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Neurosurgery Management While the neurosurgeons felt there was little hope for LE

recovery, the pre-syrinx formation risked moving upwards, potentially compromising UE function Recommended decompressive laminectomy

A few days after transfer, pt had posterior spinal fusion Finding: epidural phlegmon,T5 fracture with cord infarct,

spinal stenosis—fused. Fluid from phlegmon, tissue from ligament sent for culture

and path

Path: hypercellular, esp. plasma cells, but not clonal C/w chronic inflammation

Micro: gram stain, culture, AFB, special stains all negative

Post-op LabsDay 30 (2 days post-neurosurgery) labs:

WBC 16.9, with 6.51K eos (39%)

Looking back:

Admission to outside hospital: WBC 9.7, 194 eos (2%)

Day 16: 270 eos (3%)

Day 26: (day prior to transfer, 2 days pre-op) 3.6K eos (40%)

(This bump in eos was not previously recognized)

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Huh. Eos. Wow. Now…

1. Could this be a really nasty case of asthma?

2. Could this be whatever they call Wegener’s now?

3. Can TB do this?

4. Can cocci do this?

5. Could this all be a worm?

6. Could this be another sign of thromboembolism?

7. Pulmonary infiltrates and eos… I think that’s a syndrome

8. Gotta be from one of his drugs

Goop’s Riff on the Eos

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Hospital Course Because eos developed in-house, suspicion for drug

reaction Antibiotics changed to aztreonam, dapto

Stool O&P and strongyloides antibody sent, along with IgE, ANCA, SPEP, UPEP

Cosyntropin test sent to r/o adrenal insufficiency

Eos continued to rise, peaking at 9.8K

Patient continued to have episodes of respiratory distress and wheezing

A chest CT was performed to further assess lungs and eosinophilia

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Low lung volumes; diffuse ground glass opacities, some ill defined nodules.

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Bronchoscopy

Differential: 88% monos, 5% lymphs and 7% eos

Gram stain & culture: Mod mixed gram positive flora

CMV culture: positive

Pneumocystis: negative

KOH stain and fungal culture: negative

No strongyloides on parasite wet mount

AFB smears: negative

Respiratory virus panel PCR and Ag testing: negative

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Recurrent respiratory distress

On hospital day 40, the patient woke from a nap with severe respiratory distress Exam, Diffuse expiratory wheezing, RR 20 30

92% on 2L 87% on 2L

ABG 7.30/58/107

CXR unchanged

Continuous nebs, tx to ICU for bipap, trial of diuresis

VBG 7.32/53

A diagnostic test returned

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Goop, Time to Take a Shot

Gurpreet Dhaliwal, is that your… final

answer?

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A diagnostic test returned… Strongyloides antibody: 3.76, 4.94 on repeat

Stool O&P: Strongyloides stercoralis rhabditiformlarvae

ANCA neg

SPEP, UPEP unremarkable

HIV neg

Cort stim 6 15

Treatment The diagnosis of strongyloides hyperinfection was

made, involving lungs, GI tract, and possibly vertebrae

Started treatment with ivermectin, 15 mg/d

Steroids weaned and then held

Patient placed on bipap along with COPD meds

Over next few days, rapid improvement in respiratory condition

Discharged back to outside hospital for PT for paraplegia, with markedly improved pulmonary status

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Final Diagnosis

Strongyloides stercoralis hyperinfection with

Pulmonary infiltrates and recurrent wheezing

Eosinophilia

Gram-negative bacteremia

Spinal osteomyelitis with cord compression

Strongyloides vs. enterococcus faecalis

Special thanks to Kara Bischoff for preparing the case

Ddx of Profound Eosinophilia

ID: Parasitic infections, certain fungi (cocci, ABPA), infestations (scabies)

Allergic or atopic diseases

Heme-Malignant: hypereosinophic syndromes, some leukemias & lymphomas, other tumors (particularly lung, bladder), systemic mastocytosis

Immunologic: HyperIgE syndrome, GVH disease

Endocrine: hypoadrenalism

Other: radiation, atheroembolic, sarcoid

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Doesn’t anybody take histories

anymore?

DDx of Eosinophilic Lung Diseases

Primary

Simple pulmonary eosinophilia

Chronic eosinophilicpneumonia

Acute eosinophilic pneumonia

Churg-Strauss vasculitis

Idiopathic hypereosinophilicsyndrome

ABPA

Bronchocentricgranulomatosis

Secondary Drug-induced

Parasite-induced

Fungal-induced

Diseases Assoc w/ Eos

Asthma

Ideopathic pulmonary fibrosis

Sarcoidosis

Hypersensitivity pneumonitis

Malignancy

Langerhans cell granulomatosis

Cryptogenic organizing pneumonia

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Strongyloides HyperinfectionSyndrome

Parasite endemic in tropical, subtropical regions Including SE United States

Normal life cycle: skin->lungs->GI tract

Autoinfection cycle: may lay dormant for decades, or cause indolent disease w/ GI symptoms and eosinophilia

With immunosuppression, massive growth in disease burden, disseminated disease Lungs, GI tract (enteric bacteremia), skin, CNS No cases of strongyloides osteomyelitis reported, but

there is one case of entercoccus faecalis involving CNS in setting of hyperinfection

Rhabditiform larvae of strongyloidesfound in stool specimen

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There’s Always a First TimeA Clinical Problem Solving Case

Gurpreet Dhaliwal, MD

Professor of Medicine University of California, San Francisco

10/26/2015

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