10 pearls in scleroderma for the family practitioner · Family Medicine Rounds, St Joseph’s...
Transcript of 10 pearls in scleroderma for the family practitioner · Family Medicine Rounds, St Joseph’s...
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10 pearls in scleroderma for the family practitioner
Dr Maggie Larche, MRCP(UK), PhD Associate Professor,
Division of Rheumatology, McMaster University St Joseph’s Hospital
Family Medicine Rounds, St Joseph’s Hospital, January 2015
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Objectives
• To describe clinically pertinent points for family practitioners
• To outline evidence around each of the “pearls”
• To increase knowledge of systemic sclerosis
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Scleroderma (Systemic Sclerosis) is …
“sclero” (Greek meaning hard) + “derma” (Latin meaning skin)
= scleroderma (hard skin)
• Hippocrates
• 1945 association with systemic disease
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Terminology
Scleroderma
Localised
Morphea Linear (en coup de Sabre)
Systemic
Limited (distal to elbows or
knees) Diffuse
Images from ACR Image bank
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Pearls
1. Clinical
2. Red flags in Raynauds
3. Early diagnosis
4. Outcome
5. Risk factors
• Treatment
6. Raynauds
7. GI
8. Viagra
9. Renal crisis
10. pregnancy
1 2 3 4 5 6 7 8 9 10
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Many aspects of scleroderma • Vascular system
• Raynauds • Healed pitting ulcers in fingertips • Cutaneous and mucosal telangiectasia
• Gastrointestinal system • GERD, GAVE • Dysmotility • Constipation /diarrhea
• Respiratory system • ILD • Pulmonary hypertension
• Musculoskeletal system
• Arthritis/myositis • Flexion contractures • Carpal tunnel syndrome • Muscle weakness
• Constitutional: Fatigue/weight loss
• Skin
• Sclerodactyly
• Edema
• Digital ulcers
• Calcinosis
• Hyper or hypo-pigmentation
• Cardiovascular system • Pulmonary hypertension • Arrhythmias
• Genitourinary system • Erectile dysfunction • Dyspareunia
• Ears, nose, and throat • Sicca syndrome • Poor dentition • Hoarseness due to acid reflux with vocal
cord inflammation or fibrosis
• Endocrine system • - Hypothyroidism
• Renal system • Hypertension • Renal crisis • Chronic renal insufficiency
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• Vascular system • Raynauds • Healed pitting ulcers in fingertips • Cutaneous and mucosal telangiectasia
• Gastrointestinal system • GERD, GAVE • Dysmotility • Constipation /diarrhea
• Respiratory system • ILD • Pulmonary hypertension
• Musculoskeletal system
• Arthritis/myositis • Flexion contractures • Carpal tunnel syndrome • Muscle weakness
• Constitutional: Fatigue/weight loss
• Skin
• Sclerodactyly
• Edema
• Digital ulcers
• Calcinosis
• Hyper or hypo-pigmentation
• Cardiovascular system • Pulmonary hypertension • Arrhythmias
• Genitourinary system • Erectile dysfunction • Dyspareunia
• Ears, nose, and throat • Sicca syndrome • Poor dentition • Hoarseness due to acid reflux with vocal
cord inflammation or fibrosis
• Endocrine system • - Hypothyroidism
• Renal system • Hypertension • Renal crisis • Chronic renal insufficiency
• Usually presents with Raynauds, and puffy hands
• Particularly affects lungs, heart, kidneys and skin
• Can affect almost any organ
• May overlap with other autoimmune diseases
Pearl 1: clinical aspects
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Red flag symptoms or signs in Raynauds
• Sudden and late onset
• Constitutional features
• Thickened skin
• Digital ulcers
• Rashes
• Calcium deposits
• Arthritis
• Dry eyes/mouth
• Apthous ulcers
• Hypertension
• Dyspnea
• Muscle weakness
• Swallowing difficulties
• GI disturbances
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Pearl 2: Raynauds Red flags
Bottom Lines:
• Older age
• Male
• Digital ulcers
• Other features of autoimmune diseases
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New criteria aid early diagnosis
• Puffy fingers
• Raynauds phenomenon
• Dilated nail fold capillaries
• (ANA)
ACR Image bank Koenig et al A&R 2008
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2013 ACR/EULAR SSc guidelines
Item Sub-item Weight
Skin thickening of fingers of both hands extending proximal to MCP joints (sufficient criterion)
9
Skin thickening of fingers (only count higher score)
Puffy fingers Sclerodactyly of fingers (distal to MCP joints but proximal to PIP joints
2 3
Fingertip lesions (only count higher score)
Digital tip ulcers Fingertip pitting scars
2 3
Telangiectasia 2 Van den Hoogen 2013
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2013 ACR/EULAR SSc guidelines
Item Sub-item Weight
Abnormal nailfold capillaries 2
Pulmonary arterial hypertension and/or interstitial lung disease (maximum score is 2)
Pulmonary arterial hypertension Interstitial Lung Disease
2
2
Raynaud’s phenomenon 3
SSc-related autoantibodies (anticentromere, anti-RNA polymerase III, anti-topomerase I [anti-SCl-70] (Maximum score is 3)
Anti-centromere Anti-topomerase I Anti-RNA polymerase
3
Total score = Sum of maximum score in each category Total Score ≥ 9 Classified as Definite SSc; Maximum Score is 19
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Early diagnosis allows improved monitoring and treatment of
complications
• Diffuse disease – more likely to get ILD, PAH, renal disease
• Intensive monitoring necessary – ECHO, PFTs, renal function, BP
• Improved treatments available
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Pearl 3: early diagnosis
• New criteria make it easier to diagnose SSc early
• Consider in patients with Raynaud’s, puffy hands
and dilated nailfold capillaries
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Outcome: Diffuse versus Limited
Raynauds, digital ischemia
Esophageal dysmotility
ILD Renal crisis
Adapted from www.Clevelandclinicmeded.com
10 year survival rates: Limited: 90% Diffuse: 60% Up to x8 increased risk of death
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Pearl 4: Outcome – 75% 10 yr survival • Causes of death
– Pulmonary hypertension (major prognostic factor)
– Pulmonary fibrosis
– Scleroderma renal crisis
• 10 year survival rates:
– Limited: 90%
– Diffuse: 60%
(age and gender adjusted mortality rates 5-8X general population)
• Huge impact on quality of life
Mayes MD. Scleroderma epidemiology. Rheum Dis Clin North Am. 2003, 29: 239-254
Bottom Lines:
• Important causes of premature death
• pulmonary fibrosis, • pulmonary hypertension, • renal failure
• Significant morbidity associated
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Mortality: reduced frequency of Scleroderma Renal Crisis - more
pulmonary fibrosis
Steen and Medsger ARD 2007 66:940-4
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Pearl 5: risk factors for poor outcome
Gurman et al ARD 2010
•Diffuse skin involvement •Proteinuria •PAH •Pulmonary fibrosis •NYHA class 2 •Late onset Raynauds
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Part 1: Take Home Messages • Consider SSc in:
– new onset Raynauds
– Raynauds in older patient
– Puffy fingers
– Digital ulcers
• Monitor for: – pulm fibrosis with annual PFTs (6 monthly if less than 5 yrs)
– PAH with annual ECHO refer for RHCath if pressures ≥40 and dyspnoeic
– BP and creatinine carefully esp in diffuse disease
• Beware of steroid dose in SSc – ≥15mg may precipitate Scleroderma Renal Crisis (SRC)
• Multisystem disease: Talk to colleagues…
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Management of specific aspects of SSc
• Raynauds
• Gastro-intestinal
• Pulmonary fibrosis
• Pulmonary hypertension
• Renal crisis
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Raynauds: Management First line medications
• Calcium Channel blockers
– Amlodipine 10-20mg daily
• Nitrate patches
– 0.2mcg daily
• Angiotensin Receptor blockers
– Losartan 50-100mg od
2nd line treatments
• Prostacyclins (IVI – CCU monitoring)
– Alprostadil
– Iloprost
• PDE5 inhibitors:
– Sildenafil 100mg od
– Tadalafil 40mg od
• SSRIs - Prozac
• Sympathectomy – digital vs axillary
Conservative measures
• Smoking cessation and avoiding the cold
• Emollients
• Avoid drugs such as
• Beta blockers
• Ergotamine
• Clonidine
• Cyclosporin
• Cocaine
• Heated gloves, socks
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Pearl 6: Specific treatments for Raynauds prevent damage
•Amlodipine 10mg od •Losartan 100mg od •Prozac +/-Pentoxyfilline •Nitrodur patches 0.2-0.4mcg/day •Sildenafil/Tadalafil •IV prostacyclins
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Treatment of GI manifestations Feature Treatment
GERD Conservative measures PPI – high doses
GAVE (Watermelon stomach) Laser photocoagulation Transfusions
Gastroparesis, Dysmotility Pseudoobstruction
Domperidone 20mg tid Prucalopride 2mg od Octreotide25-50mcg bid 1G liquid Erythromycin q3 days Pyrido- or Neostigmine 1g slow ivi
Bacterial overgrowth Cyclical antibiotics Rifamixin 200mg tid for 3 days
Constipation Prucalopride 2mg od Laxatives
Diarrhoea Codeine or loperamide
Incontinence Low dose loperamide Sacral nerve stimulator
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Pearl 7: GI treatments
•High dose PPI for dyspepsia •Domperidone 10mg tid for dysphagia •Prucalopride 2mg od
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PDE5 inhibitors
• Useful in:
• Prevention and treatment of Digital ulcers in 15 patients
– Reduced number of ulcers from 44 to 13
• Pulmonary arterial hypertension
• Erectile dysfunction Kumar U et al 2013 Rheumatol Int Foocharoen C et al 2012 Arthritis Res Ther Della Rossa et al 2011 Scand J Rheum
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Pearl 8: 3 reasons to use Viagra in SSc
• Pulmonary hypertension
• Digital ulceration (prevention and treatment)
• Sexual dysfunction
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Renal Crisis • Increasing BP • Microangiopathic hemolytic
anemia • Rising Creatinine
• ~25%mortality in 1 year
• 20 to 50% risk of developing end-stage renal disease – 2 year mortality 50% (vs 36%
in non-scleroderma dialysis)
Steen et al 2005 J Rheum
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Renal Crisis
• 2012 study:
• amongst SRC (91patients)
• 78% Hypertension; 58% encephalopathy; 56% microangiopathy (anemia, reticulocytes, schistocytes, bilirubin, haptoglobolin
• 70% had received steroids before SRC
• 54% required dialysis, 41% died
Guillevin et al 2012 Rheumatology
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Renal crisis risk factors
• Diffuse SSc
• Male gender
• Steroids >15mg daily – (used for arthritis, myositis, pulmonary fibrosis). 1.5% increased risk for each mg of prednisone/day
• (Calcium channel blockers protective)
DeMarco PJ et al 2002 Arthritis Rheum Steen VD et al 1998 Arthritis Rheum Montanelli et al 2013 Clin Exp Rheum
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• ~25% mortality in 1 year
• 20 to 50% risk of developing end-stage renal disease – 2 year mortality 50% (vs
36% in non-scleroderma dialysis)
Pearl 9: Scleroderma Renal Crisis – high index of suspicion; treat
aggressively
Steen et al 2005 J Rheum
• Monitor BP and creatinine
• Careful with dose of oral steroids <15mg daily
• ACE-I – Captopril 12.5mg tid increasing aggressively
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Pregnancy in SSc
• High risk pregnancy clinic • Increased risk of preterm birth and small for gestational age
(placental insuffificnecy)
• Good maternal and neonatal survival
• OK to use Hydroxychloroquine and low dose steroids and IVIG
• Renal Crisis most signficiant complication – prompt ACEI • Try to avoid pregnancies in dcSSc within 5 yrs
Taraborelli et al 2012 Arth Rheum Lidar and Langevitz Autoimmunity Review 2012
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Biologics/ Stem cell therapy for SSC
• AntiCD20 • Improved skin score (Jordan et al ARD 2014)
• RECOVER – Rituximab study in polyarthritis in SSc (http://www.clinicaltrials.gov/ct2/show/NCT01748084)
• Belimumab + MMF in early Diffuse SSc (http://www.clinicaltrials.gov/ct2/show/NCT01670565)
• Trials of antiIFNR in Ssc • Tocilizumab trial underway
(http://www.clinicaltrials.gov/ct2/show/NCT01532869)
• Stem cell treatments for scleroderma:
Tuesday, 22 April 2014
08:00 hrs, New Auditorium, 4th Floor
Juravinski Hospital
Stem cell therapy of autoimmune disease with focus on scleroderma
Dr Alan Tyndall
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Pearl 10: Pregnancy
• Monitor closely
• Aggressive management of hypertension (with ACE-I)
• Try to avoid in early diffuse disease
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Pearls
1. Clinical
2. Red flags in Raynauds
3. Early diagnosis
4. Outcome
5. Risk factors
• Treatment
6. Raynauds
7. GI
8. Viagra
9. Renal crisis
10. Pregnancy
1 2 3 4 5 6 7 8 9 10
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Conclusions • Focus on early diagnosis and intensive
monitoring
• Supportive management
• Specific treatments
• New hope…
Pearl 11: ``Team game``
Hamilton Scleroderma Group
http://www.hamiltonscleroderma.org/
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Thanks
• St Joseph`s Family Practitioners
• Multidisciplinary colleagues at Hamilton Scleroderma Group – Ellen McDonald – the patient’s quarterback!
• Rheumatology Colleagues
• Scleroderma Society of Ontario
• Questions: [email protected]
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Treatment of Pulmonary fibrosis
• Cyclophosphamide oral (2mg kg day) or IV (600mgm2) – only drug shown to be effective in RCT • Modest improvements in FEV1 and TLC and
dyspnea scores • Improvements in skin scores
• MMF not inferior to Cyclophos in ILD (Hinchcliff 2013)
• Rituximab – improves GG appearance (Daoussis D et al
Clin Exp Rheum 2013)
• ASTIS trial ARD 2012 Suppl Abs LB0002
Tashkin et al 2007 AJRCCM 176:1026 Hoyles et al 2006 A&R 54:3962
Bottom Lines:
• Several drugs helpful in SSc-ILD
• Cyclophosphamide
• Mycophenolate
• Rituximab
• Stem cell transplantation showing promise
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Treatment of Pulmonary fibrosis
• Cyclophosphamide oral (2mg kg day) or IV (600mgm2) – only drug shown to be effective in RCT • Modest improvements in FEV1 and TLC and
dyspnea scores • Improvements in skin scores
• MMF not inferior to Cyclophos in ILD (Hinchcliff 2013)
• Rituximab – improves GG appearance (Daoussis D et al
Clin Exp Rheum 2013)
• ASTIS trial ARD 2012 Suppl Abs LB0002
Tashkin et al 2007 AJRCCM 176:1026 Hoyles et al 2006 A&R 54:3962
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Pulmonary Hypertension Algorithm
TT ECHO
RVSP ≤ 35mmHg
Unlikely PAH
RVSP 35-40mmHg
No dyspnoea Dyspnoea
RVSP ≥ 40mmHg
Suspect PAH
Right Heart Catheterisation
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Pulmonary Hypertension: treatment • Improved survival in PAH
• Supportive therapies: – Oxygen (if resting pO2 under 60mmHg)
– Loop diuretics
– Digoxin
– (anticoagulation)
• Calcium channel blockers (after dynamic testing)
• IV epoprostenol
• Endothelin receptor antagonists – Bosentan, Ambrisentan- improved exercise capacity and functional status
• PDE5 inhibition- Sildenafil, tadalafil – improved exercise capacity