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