Mixed Connective Tissue Disease Warren Prokopiw Resident 2011-2012.

Mixed Connective Tissue Disease

Warren ProkopiwResident 2011-2012

Outline• 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

• 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

• 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

• 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 HemorrhagesJaneway lesions

Labs

• 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• 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

MedicationsFatigue, 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.com-207.194.133.9-9974F6FC8B-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