Mixed Connective Tissue Disease
Warren Prokopiw
Resident 2011-2012
Outline
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Past History
Current Medication History
Clinical Course
All DRPs
Summary on Mixed Connective Tissue Disease
Goal of Treatment
Recommendations and rationale
Monitoring
Actions and Follow up
Patient Information
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SH is a 41 or female
Married, 2 children, on disability
Social smoker, quit 18 years ago
Non-drinker, used to smoke marijana
NKDA
Chief Complaint / HPI
• Abdominal pain, nausea, vomiting
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Partial L nephrectomy 13 Sep - Disch 18 Sep
Night sweats day after discharge
Increasing fatigue, and sweats
Presented to ER on 22 Sep
T36.8, BP 168/108, HR 105
– Urinalysis – protinuria, 10-40 RBC
• Bilateral pyelonehprtitis – Cefriaxone 1g IV Q24
Previous Medical/medication History
• Anxeity/ panic disorder
– Paroxetine 40 mg daily
• Systemic Lupus erythematosus – mostly affecting
joints
– Methotrexate 10 mg q Thurs – last dose 8 Sep
– Prednisone 20 mg x 5 days 1 year ago
• Nehrolithiasis – calyceal diverticulum with stones
– Partial nephrectomy
• L uteric stent 2001
• 20 Sep - Cephalexin 500 QID and Tylenol #3
Clinical Course
• Persistent fever and Tacycardia
– added Cipro 400 IV Q12H, then changed to
imipenem 500 IV Q6H
• Cultures – blood negative, urine candidia
– Changed to fluconazole 200 mg PO daily
• 2 Oct – pleuritic chest pain/SOB, ST Elevation
<1mm ? PE and antiphospholipid syndrome
• MCTD myopericarditis – Methylprednisolone 60 mg IV Q8H to Prednisone
Review of systems
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Vitals T 36.2, BP 111/83, HR 78, R 18, 98% RA
EENT – dipoplia
Resp – chest clear, CT Chest – No PE
CVS – S1S2 normal, no pericardial rub, JVP
normal
• Abdomen – soft, nontender, complaint of GI
upset
• Skin – improving Janeway lesions, remaining
splinter hemorrhages
Splinter Hemorrhages
Janeway lesions
Labs
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WBC 12.3, Hg 90, RDW 15.3, Neut 11.39
Scr 38, eGfr > 120
CRP 16.7 (down from 121.4)
AST 40, ALT 12, AlkP 149
Trop +
ANA +, RNP +, Cardiolipin IgG 31, IgM 17
Current Medications
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Zopiclone 3.75 – 7 mg QHS
Paroxetine 20 mg daily
Lorazepam 1 mg PO/SL TID prn
ASA 162 mg daliy
Heparin 5000 U Subcut Q12H
Nitroglycerin 0.3 mg pumpspray prn
Metoprolol 6.25 mg BID
Esomeprazole 40 mg daily
Cardiac bowel protocol
Ferrous Fumarate 300 mg BID – Folic Acid 1 mg daily
Prednisone 50 mg QAM
Tramacet 1-2 tabs TID prn
Acetaminophen 0.5 – 1 g QID prn
Drug Related Problems
• SS is experiencing a flare in her MCTD due to lack of
suppressive therapy
• SS is experiencing GI upset as an adverse effect of
prednisone treatment
• SS is at risk of worsening anxiety due to lowered
paroxetine dose
• SS is at risk of osteoporosis from recurrent steroid
use
• SS is at risk of stomatitis or hepatotoxicity as an
adverse effect of methotrexate treatment
Mixed Connective Tissue Disease
• Autoimmune disease against own connective
tissues
• considered an overlap of
– SLE, scleroderma, polymyositis
• 2.7 cases per 100,000
• Female 10:1 over males
• Typical onset age 15-25
MCTD Symptoms
• Common Symptoms
– Raynaud phenomenon, swollen hands,
– anti–U1-RNP antibodies (hallmark)
• SLE findings
– Polyarthritis, Lymphadenopathy, Facial erythema,
Pericarditis or pleuritis, Leukopenia or thrombocytopenia
• Scleroderma findings
– Sclerodactyly, Pulmonary fibrosis, esophageal hypomotility
• Polymyositis findings
– Muscle weakness, elevated serum muscle enzymes
Raynaud phenomenon
Goals of Treatment
• No RTCs to guide therapy
– Management based on therapies for SLE, scleroderma, or
polymyositis
• Control symptoms and maintain function
– Target medical therapy to specific organ involvement
– Minimize glucocorticoid burden
• hydroxychloroquine 400 mg daily
• methotrexate 7.5 -15 mg per week
– Monitor for development of pulmonary hypertension
Medications
Fatigue, arthralgias, myalgias
NSAIDs, antimalarials, low-dose prednisone (<10
mg/day).
Arthritis
NSAIDs, antimalarials, methotrexate
Raynaud phenomenon
Nifedipine, prazocin
Myositis
Acute onset/severe - prednisone (60 to 100 mg/day)
Myocarditis
Trial of steroids and cyclophosphamide
Heartburn/dyspepsia
H2 antagonists, H+ proton pump blockers,
metoclopramide trial
Pericarditis
NSAID or short course of prednisone
Osteoporosis
Calcium/vitamin D supplements, bisphosphonates
Back to the case
• SS showed marked improvement on
prednisone
• Added
– hydroxychlorloquine 200 mg daily
– Methotrexte 10 mg weekly
– Dimenhydrinate 50-100 mg q6h for N&V
• Discharged 8 Oct to community
– Plan for 2 weeks prednisone, then
Drug Related Problems
• SS is experiencing a flare in her MCTD due to lack of
suppressive therapy
• SS is experiencing GI upset as an adverse effect of
prednisone treatment
• SS is at risk of worsening anxiety due to lowered
paroxetine dose
• SS is at risk of osteoporosis from recurrent steroid
use
• SS is at risk of stomatitis or hepatotoxicity as an
adverse effect of methotrexate treatment
Recommendation
• SS required prophylaxis from steroid induced
osteoporisis
– Calcium Citrate 400 mg TID
– Vitamin D 1000 Units daily
• Monitor – attempt taper off steroids
– If plan continued to 3 months annual use
– Bisphosphonates - etidronate(Didrocal)
• Monitor adverse effects (esophageal, ONJ
Plan/Follow up
• Letter to GP
– Recommend calcium, vit D
– Etidronate if necessary
– Discrepancy on paroxetine dose
References
• Bennet, R., Axford, J., Romain, P., (2011). Prognosis and treatment of
mixed connective tissue disease. Up to Date, ecapp0505p.utd.com207.194.133.9-9974F6FC8B-64913.14 accessed 9 Oct 2011.
• Papaioannou, A., Morin, S., Cheung, A. M., Atkinson, S., Brown, J. P.,
Feldman, S., Hanley, D. a, et al. (2010). 2010 clinical practice guidelines for
the diagnosis and management of osteoporosis in Canada: summary.
CMAJ : Canadian Medical Association journal = journal de l’Association
medicale canadienne, 182(17), 1864-73. doi:10.1503/cmaj.100771