Audit

VENICE CHAN

General information

• 26/F• Unemployed• Roman Catholic• Informant: Patient and Sister• Reliability 85%

Chief Complaint

• Dyspnea

HPI• tightening of skin of both hands and a

hypopigmented patch at the upper back • (+) raynaud’s phenomenon• was subsequently referred to a

rheumatologist where she was diagnosed as a case of scleroderma

• was prescribed colchicine 500mcg/tab 3 tabs TID

• took colchicines for 1 week but discontinued • Laboratory exams were also requested but

the patient did not comply. • consulted an “albularyo” and took unrecalled

herbal medications.

18 mos PTA

HPI• tested for serum SCl-70 with

an elevated result• (-) consult• continued taking the herbal

medications at this time• noted increase in the number

of hypopigmented macules and patches eventually becoming generalized

• Tightening of the skin also involved both arms and legs

One year PTA

HPI increased stiffness of joints

causing inability to walk without assistance

2 weeks PTA

•(+) palpitations, cough and intermittent difficulty of breathing

•consulted a pulmonologist and was prescribed Sinecod forte and azithromycin 500mg/tab 1 tab OD.

•CXR revealed cardiomegaly. “water bottle heart” was prescribed furosemide and aldactone, uncompliant (BP: 80/60)

1 week PTA

• (+) increased difficulty of breathing, (+) insomnia, (+) two pillow orthopnea and bipedal edema.

• consulted a cardiologist, who upon seeing the CXR plates along with patient’s dyspnea, advised admission

3 days PTA

Few hours PTA

ADMISSION

Past Medical History

• s/p incision and drainage of abscess (2nd digit of the R hand) July 2009

• Transfused 4 ‘u’ of PRBC (July 2009), 3 ‘u’ of PRBC (Aug, 2009)

• No other rheumatologic disorders in the family• No HPN• No asthma• No allergies

Menstrual History

• M-13 y/o• I- 29 to 30 days, stopped menstruating (July,

2009)• D- 3 to 4 days• A-1 to 2 pads per day• S- None

Family History

• (-) connective tissue disease• (+) HPN – mother• (+) asthma – father• (-) DM• (-) thyroid disorders• (-) allergies

Personal Social History

• Nonsmoker• Non alcoholic beverage drinker• No illicit drug use• No food preference.• Prefers soft food, easy to chew

Review of Systems• General:

– (+) weakness, (+) easy fatigability– No weight changes, no fever, no night sweats, no anorexia, no insomnia

• SKIN– See HPI

• EYE– (+) blurring of vision, (-) eye pain, (-) itchiness

• EAR– (-) deafness, (-) tinnitus, (-) discharge

• NOSE– (-) epistaxis, discharge, obstruction,, postnasal drip, sinusitis

Review of Systems

• MOUTH– (+) bleeding gums, (-) oral ulcers

• THROAT– (-) throat pain

• NECK – (+) hypopigmented patches over the neck

• BREAST– (-) skin changes

ROS• Pulmonary

– See HPI• Cardiac

– (-) chest pain, PND, syncope, easy fatigability, orthopnea, • Vascular

– See HPI• Gastrointestinal

– (-) nausea, vomiting, retching, hematemesis, belching• Genito-urinary

– (-) frequency, dysuria, hematuria, flank pain, hesitancy, nocturia

ROS

• Musculoskeletal• (+) bilateral elbow stiffness, bilateral knee stiffness

• Endocrine• (-) heat/cold intolerance, no heat intolerance, no polydipsia,

polyphagia, polyuria

• Neurologic– No altered sensorium, no dizziness, no vomiting, no hx of

head trauma• Psychiatric

– (-) depression, delusions, paranoia, hallucinations, illusions

Physical Exam

• Conscious, coherent, wheelchair borne, not in CP distress

• VS: BP: 90/60 PR 76, RR 20 T 36.3C• Warm dry skin with pruritic hyperpigmented

macules to patches over dorsal surface of upper extremities, over legs, lower back measuring 1x1 cm to 2 x 3 cm

• Hyperpigmented hyperkeratotic plaque over Dorsal aspect of L foot topped with some crusts

Physical Exam

• Supple neck, hypopigmented patches over the neck area

• Pale palpebral conjunctivae, anicteric sclerae• (+)oral ulcers, forward protrusion of teeth with

difficulty closing, nonhyperemic posterior pharnyngeal wall, “mask-like” facies

• Asymmetric chest expansion, lagging on L, Fremiti L>R, whispered pectoriloquy on L, breath sounds L > R fine crackles from T7 down bilateral

Physical Exam

• Adynamic precordium, distant heart sounds, no murmurs, thrills, (+) PA lift, (-) heaves

• Flat abdomen, no scars, soft, nontender, Liver span 6 cm, Traube’s space not obliterated

• Pulses full and equal, no cyanosis, no edema, (+) skin tightening of all extremities, excised scar at end of first digit of the R hand

Assessment

• Scleroderma

POS

• Please admit to Bed 209C under the service of Dr. Yamamoto

• Soft diet: 35 kcal/day 60% CHO, 15% CHON, 25% Fats divided into 3 meals and 2 snacks

• Monitor VS Q1 and record.• Monitor I and O Qshift and record.• Inser heplock.• IVF PNSS 1L to run at 20 gtts/min.

Diagnostics

• CBC w/ plt• Urinalysis• CXR (PA-Lat)• 12L ECG• 2D Echo• BUN, Crea• PT, aPTT• ABG

Therapeutics

• Nifedipine (Adalat gitz) 30 mg/tab 1 tab OD. Hold for BP<100/60

2D Echo

• Large pericardial effusion posterior to LV 2.9 superior to RA 2.6 and to RV < 1cm with no signs of tamponade

• Severe PHPV by TR regression 82 mmHg• Concentric LVH with good wall motion and

muscular contractility, septal flattening in systole 73% EF

• Refer to TCVS for pericardiostomy.

• Refer to Rheumatology for co-management.• Request for pericardial fluid Gram stain and Culture

and sensitivity, WBC and differential count, AFB stain, cytology. Save specimen for possible MTB culture.

• Watch out for hypotension or dyspnea.• Inform Drs. Dysangco/Vicera of this admission.• Accomplish Database, Record for hospital admission

c/o CIC.• Accomplish admitting history c/o IIC

• Start omeperazole 40 mg/tab 1 tab OD.• Hook to O2 supplementation 2-3lpm via nasal

cannula.• Start prednisone 30 mg/tab 1 tab once daily after

breakfast.• Add MTB culture and fungal culture if

pericardiostomy will be done.• Add aerobic and anaerobic culture at once using

fresh specimen.

Course In The Ward

• Patient was referred to TCVS, however, advised pericardiostomy with possible pericardial biopsy were not done due to financial constraint.

• Patient was subsequentky transfused with 2 units of pRBC properly typed and cross-matched.

9/20Urea nitrogen 7.9Creatinine 0.69Sodium 137Potassium 3.4Ionized Calcium 1.15

CBC 9/20 9/24Hgb 79 110Hct 0.24 0.33MCV 76.30 80.70MCH 25.40 26.90MCHC 33.30 33.30RDW 22 21.00Platelet 223 248WBC 4.9 3.00 Neutrophils 0.76 0.78 Lymphocytes 0.21 0/22 Eosinophils 0.03 0

09/20/09 9/24PT 15.2 12.9aPTT 48.5 39.6INR 1.2 1.1

09/20Color YellowTransparency Slightly turbidpH 7.0Sp. Gr. 1.010Albumin NegativeSugar NegativeRBC 0-1/hpfPus cell 15-25/hpfSquamous cell FewRenal cell Few

09/20pH 7.473pCO2 24.9pO2 72.1HCO3 18.2O2 sat 95.5%dFiO2 23.07%P/F 343.33

Pericardial fluid

• NO AFB seen• Yellow, slightly turbid with big coagulum, 25

ml yellow, clear with red sediment, 561/cu mm total rbc, no wbc’s found

Scleroderma

• Skin: sclerotic changes• Esophagus: dysphagia• Small vessels with manifestations such as

Raynaud phenomenon. • Cardiac involvement: manifested by intramural

coronary involvement and immune-mediated endothelial injury, which is often associated with the Raynaud phenomenon clinically.

• Cardiac involvement is the third most common cause of mortality in patients with scleroderma.

• Conduction defects occur in up to• 20% of patients, and a pericardial effusion is found in a third

of• patients, but it is often asymptomatic. • Indirect cardiac involvement due to pulmonary hypertension

and cor pulmonale is frequent.• • Coronary vasculitis is associated with clinical Raynaud

phenomenon.• • Conduction defects may occur in up to 20% of patients.

Scleroderma

• May cause sudden oliguric renal failure and severe hypertension due to smallvessel

• occlusion in previously stable pts.Aggressive control of bp with ACE

• inhibitors and dialysis, if necessary, improve survival and may restore renal

• function.

SYSTEMIC SCLEROSIS (SCLERODERMA, SSC)

• DEFINITION AND PATHOGENESIS • Multisystem disorder characterized by

inflammatory, vascular, and fibrotic changes of skin and various internal organ systems (chiefly GI tract, lungs, heart, and kidney).

• Pathogenesis unclear; involves immunologic mechanisms leading to vascular endothelial damage and activation of fibroblasts.

CLINICAL MANIFESTATIONS• • Cutaneous—edema followed by fibrosis of the skin

(chiefly extremities, face, trunk); telangiectasis; calcinosis; Raynaud’s phenomenon

• • Arthralgias and/or arthritis• • GI—esophageal hypomotility; intestinal hypofunction• • Pulmonary—fibrosis, pulmonary hypertension,

alveolitis• • Cardiac—pericarditis, cardiomyopathy, conduction

abnormalities• • Renal—hypertension; renal crisis/failure (leading

cause of death)

CLINICAL MANIFESTATIONS

• Two main subsets can be identified:• 1. Diffuse cutaneous scleroderma—rapid development

of symmetric skin thickening of proximal and distal extremity, face, and trunk. At high risk for development of visceral disease early in course.

• 2. Limited cutaneous scleroderma—skin involvement limited to face and extremity distal to elbows; associated with better prognosis; frequently has features of CREST syndrome (calcinosis, Raynaud’s, esophageal dysmotility, sclerodactyly, telangiectasias).

VALUATION

• E• • Hx and physical exam with particular attention to

blood pressure (heralding feature of renal disease).• • Laboratories: ESR, ANA (anticentromere pattern

associated with CREST), specific antibodies may include antitopoisomerase I (Scl-70), UA

• • Radiographs: CXR, barium swallow if indicated, hand x-rays may show distal tuft resorption and calcinosis.

• • Additional studies: ECG, consider skin biopsy

TREATMENT• • Education regarding warm clothing, smoking cessation,

antireflux measures• • Calcium channel blockers (e.g., nifedipine) useful for

Raynaud’s phenomenon. Other agents with potential benefit include sildenafil, losartan, ketanserin, fluoxetine.

• • ACE inhibitors—particularly important for controlling hypertension and limiting progression of renal disease.

• • Antacids, H2 antagonists, omeprazole, and metoclopramide may be useful for esophageal reflux.

• • D-Penicillamine—controversial benefit to reduce skin thickening and prevent organ involvement; no advantages to using doses 125 mg every other day

TREATMENT

• Glucocorticoids—no efficacy in slowing progression of SSc; indicated for nflammatory myositis or pericarditis; high doses early in disease may be associated with development of renal crisis.

• • Cyclophosphamide—improves lung function outcomes and survival in pts with alveolitis.

• • Epoprostenol (prostacyclin) and bosentan (endothelin-1 receptor antagonist)— may improve cardiopulmonary hemodynamics in pts with pulmonary hypertension.

Discussion