1. Long Beach memorial MICU
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

2. 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.
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

3. HPI
32yo M, PMH of major depressive disorder and 80-lb weight 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 2-pillow 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.

4. Physical Exam
GEN: Anxious on BIPAP, labored breathing without BIPAP, no 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

5. 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

6. Imaging: CXR
Cardiac silhouette remains enlarged, more so than prior exam. There is
localized left lower lobe consolidation with underlying diffuse increased
interstitial markings. No pneumothorax. No significant pleural effusion.

7. Imaging: CT Chest
1. LEFT LOWER LOBE PNEUMONIA, RIGHT LOWER LOBE PNEUMONIA. SUPERIMPOSED UPON
CHRONIC INTERSTITIAL LUNG DISEASE.
2. MILD PERICARDIAL EFFUSION.
3. TRACE ASCITES.
4. MODERATE LEFT PLEURAL EFFUSION INCLUDING SUBPULMONIC COMPONENT. SMALL RIGHT
PLEURAL EFFUSION.
5. MEDIASTINAL LYMPHADENOPATHY. CONSIDER REACTIVE AND NEOPLASTIC CAUSES.

8. 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

9. EKG Sinus tachycardia Right bundle branch block
Left anterior fascicular block
Non-specific ST-T wave abnormalities

10. 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

11. 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

12. 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 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

13. 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

14. 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.
Post-mortem labs:
ANA +,
Anti-Scl70, dsDNA, anti-Smith, anti-CCP, RF, C3, C4, Ro, La (-)

15. Diagnosis??

16. Scleroderma Disorders
Peak onset between 30-50, women
Skin involvement nearly universal from excess collagen
Severity and extent is variable
Prevalence: cases per million

17. 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

18. Clinical Manifestations
SCLERODACTYLY plus multi-organ involvement:
Rheumatology Image bank
Myocardial fibrosis is thought to result from recurrent vasospasm of small vessels and is often associated with contraction band necrosis, a histological lesion indicative of myocardial ischemia followed by reperfusion. Myocardial involvement can lead to systolic or more often diastolic ventricular dysfunction. This was illustrated in a Doppler echocardiography study of 570 patients with SSc in which left ventricular systolic and diastolic dysfunction were present in 1 and 18 percent, respectively [23]. Higher rates of myocardial dysfunction have been reported with tissue Doppler studies in which the abnormalities were independent of pulmonary artery hypertension or interstitial lung disease.
The earliest HRCT change is usually a narrow, often ill-defined, subpleural crescent of increased density in the posterior segments of both lower lobes. Among patients with SSc-associated ILD, the most common pathologic pattern, nonspecific interstitial pneumonia (NSIP).
"On-line Archives of Rheumatology" © ,

19. Clinical Manifestations
SCLERODACTYLY plus multi-organ involvement:
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
Myocardial fibrosis is thought to result from recurrent vasospasm of small vessels and is often associated with contraction band necrosis, a histological lesion indicative of myocardial ischemia followed by reperfusion. Myocardial involvement can lead to systolic or more often diastolic ventricular dysfunction. This was illustrated in a Doppler echocardiography study of 570 patients with SSc in which left ventricular systolic and diastolic dysfunction were present in 1 and 18 percent, respectively [23]. Higher rates of myocardial dysfunction have been reported with tissue Doppler studies in which the abnormalities were independent of pulmonary artery hypertension or interstitial lung disease.
The earliest HRCT change is usually a narrow, often ill-defined, subpleural crescent of increased density in the posterior segments of both lower lobes. Among patients with SSc-associated ILD, the most common pathologic pattern, nonspecific interstitial pneumonia (NSIP).

20. 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:
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
Antitopoisomerase I (anti-Scl-70) dcSSC and severe interstitial lung disease
Antcentromere (ACA)- lcSSc
Anti-RNA polymerase III antibodies- dcSSC, rapidly progressive skin involvement, renal crisis, risk of concomitant cancer
Anti-U3 RNP (fibrillarin)- pulmonary hypertension and skeletal muscle involvement
Anti-PM-Scl0 myositis overlap
Anti-beta2 glycoprotein I- recurrent thromboembolic events, ischemic digital loss, active digital ischemia, and echocardiographic evidence for PAH
Note high titers of rheumatoid factor, anti-citrullinated peptides, anti U1 RNP, anti-dsDNA and Anti=smith are uncommon and have more prominent arthritis

21. 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

22. 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
Carefully selected SSc patients undergoing lung transplantation have the same morbidity and mortality of lung transplantation as patients undergoing lung transplantation for idiopathic pulmonary fibrosis. None of the patients had cutaneous ulcers, recurrent episodes of aspiration, renal failure, or left ventricular dysfunction. Four year survival was approximately 70 percent.

23. Prognosis
SSc has the highest case-specific mortality of any of the auto-immune rheumatic diseases
Predictors of poor prognosis
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%
Symptomatic heart failure and arrhythmias
Pulmonary fibrosis and pulmonary hypertension

24. References
Denton, Christopher P. Immunomodulatory and antifibrotic approaches 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.