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 hypopigmentedmacules and patches eventually becoming generalized • Tightening of the skin also involved both arms and legs One year PTA
HPI 2 weeks PTA increased stiffness of joints causing inability to walk without assistance • (+) 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 pruritichyperpigmentedmacules to patches over dorsal surface of upper extremities, over legs, lower back measuring 1x1 cm to 2 x 3 cm • Hyperpigmentedhyperkeratotic plaque over Dorsal aspect of L foot topped with some crusts
Physical Exam • Supple neck, hypopigmented patches over the neck area • Pale palpebral conjunctivae, anictericsclerae • (+)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 • Adynamicprecordium, 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. • Inserheplock. • 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 (Adalatgitz) 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.
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 Raynaudphenomenon. • 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 corpulmonale 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’sphenomenon. 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 nflammatorymyositis 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.