ICU Case of the Block
LONG BEACH MEMORIAL MICU
DR. ROFOOGARAN
DR. BELTRAN
LIANNE LIN
BABAK ESHAGHIAN
SAAHIR KHAN
MELANIE KUSONRUKSA
MIRIAM NOJAN
SAMANTHA COSTANTINI
MARK FARAG
Prior Admission
 33 year old male admitted 6mo ago for shortness of
breath, productive cough x 3 days, night sweats, and
subjective fever, also notable for 40 lb weight loss in
past 4 months, attributed to depression due to losing
custody of child.
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CXR: Suspicious for multifocal pneumonia
CT Chest: Suggestive of atypical/multifocal pneumonia,
possible interstitial pattern
Treatment: Ceftriaxone and Azithromycin -> Levaquin,
Remeron added by psychiatry for severe depression
Negative workup for TB, HIV, Hepatitis
Discharged to home in stable condition
HPI
 32yo M, PMH of major depressive disorder and 80-lb weight
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loss over 8 months attributed to depression
Presents with chest pain, dyspnea on exertion
Admitted to ICU for hypotension, dyspnea and elevated
troponin.
Intermittent chest pain since 3-4 days ago, lower left chest,
relieved by rest.
Shortness of breath since 3-4 days ago with activity, relieved
by rest, associated with bilateral lower leg swelling and 2pillow orthopnea.
Denies any trauma, denies recent substance use other than
marijuana, denies meth and cocaine other than one episode
10yrs ago, states last alcohol use 4 months ago, denies
personal or family history of cardiac disease, denies
fever/chills, headache, abdominal pain.
Physical Exam
 GEN: Anxious on BIPAP, labored breathing without BIPAP, no
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acute distress
HEENT: normocephalic, atraumatic, perrl, eomi, oropharynx clear,
moist mucous membranes
NECK: supple, no jvd
CV: tachycardic with regular rhythm, + S4, 1/6 systolic murmur in
LRSB, no rubs or gallops
LUNGS: bibasilar crackles L>R, otherwise clear
ABD: normoactive bs, soft, nt/nd, no HSM, no masses, no CVAT
EXT: warm, well-perfused, peripheral pulses 1+ difficult to palpate
due to thick skin, no cyanosis or edema
SKIN: No rashes, ulcers, or lesions noted
NEURO: alert and oriented x3, CNII-XII intact, 5/5 motor strength
and sensation to light touch grossly intact throughout, nonfocal
Labwork
 Pertinent labs:
 CBC: WBC 13.1, Hgb 12.7, Plat 334
 CMP: BUN 23, Cr 0.67, ALT 166, AST 152, otherwise wnl
 BNP: 952
 Trop: 3.00, CK 119
 ABG: 7.55, 28, 178, 25
 Lactate: 1.9
 Utox: +Cannabinoids
Imaging: CXR
Imaging: CT Chest
ECHO
 SUMMARY:
1. Left ventricular ejection fraction \R\ <20 %.
2. Severely decreased left ventricular systolic function.
3. Moderate to severe mitral valve regurgitation.
4. Grade II (pseudonormal) left ventricular diastolic
dysfunction.
5. Hypokinetic right ventricular function.
6. Dilated right ventricle.
7. Dilated left atrium.
8. Dilated right atrium.
9. Moderately reduced right ventricular systolic
function.
10. Small pericardial effusion.
11. Estimated pulmonary artery pressure 35 mmHg
EKG
Hospital Course
 8/21: Hospital Day #1
 Pt continues to have increased WOB, but does not tolerate
BIPAP well and refuses intubation
 Central line placed for blood pressure support, pt declines
arterial line
 Consults:
Cardiology: On physical exam, noticed upper chest wall
thickening, skin thickening on bilateral hands, unable to form
fists; Recs: unlikely ACS, await echo, hold heparin and follow
rheum consult
 Rheumatology: Findings as seen above +nail pitting, pt reported
difficulty swallowing liquids and solids, always feels like he is
choking; Recs: IVIG for systemic sclerosis picture, Cytoxan once
PNA clears, full rheumatologic workup
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Hospital Course
 8/22: Hospital Day #2
 Trop trending down, 1.92
 ESR 24, CRP 2.7
 Pt subjectively feeling better after first dose of IVIG
 Increasing levophed requirement to maintain BP
 Consults:
Cardiology: Top of cardiac differential is either Scleroderma
effects and/or acute myocarditis, IVIG would help with both
 Rheumatology: Continue IVIG, f/u rheum labs, recommend
barium swallow, bronchoscopy and BAL to rule out infection
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Hospital Course
 8/23: Hospital Day #3
 Acutely short of breath on BIPAP, RR 30-40s
 Emergent intubation and A-line insertion, patient markedly
hypotensive
 Asystole on cardiac monitoring @ 10:30, code blue called
 Coded for 30 minutes in PEA arrest, ROSC, pulses not
palpable but seen on doppler
 Bicarb drip + bicarb amps + pressors
 Family informed, at bedside, patient full code
Hospital Course
 8/23: Continued
 Code Blue Labs:
Cr: .63 -> 1.16
 AG: 9 -> 26
 ALT: 135 -> 701
 AST: 113 -> 1051
 Lactic Acid: 1.9 -> 18.7
 CPK: 1025
 Myoglobin: 2871
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Hospital Course
 8/23-8/24: Hospital Day #4
 Continued to require increasing doses of pressors, family at
bedside.
 Family decided to make patient DNR/DNI, pt went into
asystole and declared dead.
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Post-mortem labs:
ANA +,
 Anti-Scl70, dsDNA, anti-Smith, anti-CCP, RF, C3, C4, Ro, La (-)
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Diagnosis??
Scleroderma Disorders
Peak onset between 30-50, women
Skin involvement nearly universal from excess
collagen
Severity and extent is variable
Classifications
 Limited cutaneous systemic sclerosis (lcSSc)
 restricted to hands, distal forearm > face and neck
 prominent vascular manifestations (CREST)
 Diffuse cutaneous systemic sclerosis (dcSSc)
 trunk, upper arms and shoulders involvement
 significant fibrotic internal organ damage
 Systemic sclerosis sine scleroderma
 Environmentally induced scleroderma
 Overlap syndromes
 Pre-scleroderma
Clinical Manifestations
SCLERODACTYLY plus multiorgan involvement:
Rheumatology Image bank
"On-line Archives of Rheumatology" © ,
1996-2006
http://www.rheumatologynetwork.com/articles/limited-joint-mobility-diabetesmellitus-clinical-implications#sthash.A8P1MsuN.dpuf
Clinical Manifestations
SCLERODACTYLY plus multi-organ involvement:
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RAYNAUD phenomenon
HEARTBURN, dysphagia and esophageal dysmotility
Dyspnea on exertion (ILD from NSIP)
Pulmonary hypertension
Myocardial fibrosis, pericarditis and effusion, arrhythmias, CAD, dilated
> restrictive heart failure
Acute hypertension and renal insufficiency (renal crisis)
Malabsorptive diarrhea
Characteristic mucocutaneous telangiectasia on the face
Digital infarctions and digital tip pitting scars
Arthralgias, arthritis, myalgias, tendon friction rub
Erectile dysfunction
Diagnosis
 Suggested by skin thickening and sclerosis
 Supported by extracutaneous features and autoantibodies (ANA)
 Serologic tests confirm but do not exclude diagnosis of
systemic sclerosis
>99.5% specific, but 20-50% sensitive
>95% of SSc patients have at least one:
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Anti-topoisomerase I (anti-Scl-70)
Anti-RNA polymerase III antibodies
Anti-centromere (ACA)
Anti-U3 RNP (fibrillarin)
Anti-PM-Scl myositis overlap
Anti-beta2 glycoprotein I
Additional Studies
 BAL fluid: exclude infection
 typically increased neutrophils and eosinophils, sometimes
lymphocytes and mast cells
 Lung biopsy: limited utility
 symptomatic and physiologic severity of ILD are better
predictors of outcome
 Skin biopsy: differentiate from other etiologies
Treatment
 Cyclophosphamide with low dose glucocorticoids
 Azathioprine plus glucocorticoids
 Pneumocystis jirovecii prophylaxis
 Methotrexate (but risk for pulmonary fibrosis)
 ? Rituximab
 ? IVIG
 Lung Transplantation in select SSc patients
Prognosis
 SSc has the highest case-specific mortality of any of
the auto-immune rheumatic diseases
 Predictors of poor prognosis
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Diffuse skin involvement
Symptomatic cardiac involvement: two- and five-year
mortality rates of 60 and 75%
Interstitial lung disease and rapid DLCO decline: nine-year
survival rate of 30%
References
 Denton, Christopher P. Immunomodulatory and antifibrotic approaches
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to the treatment of systemic sclerosis (scleroderma). Uptodate.
Denton, Christopher P. Overview of the treatment and prognosis of
systemic sclerosis (scleroderma) in adults. Uptodate.
Vargas, John. Clinical manifestations of systemic sclerosis (scleroderma)
lung disease. Uptodate.
Vargas, John. Diagnosis and differential diagnosis of systemic sclerosis
(scleroderma) in adults. Uptodate.
Vargas, John. Overview of the clinical manifestations of systemic
sclerosis (scleroderma) in adults. Uptodate.
Vargas, John. Prognosis and treatment of interstitial lung disease in
systemic sclerosis (scleroderma). Uptodate.