SCLERODERMA. Scleroderma update. No disclosures or conflicts. Leslie Kahl, M.D. April 10, 2015

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1 Scleroderma update Leslie Kahl, M.D. April 10, 2015 No disclosures or conflicts KT is a 45 year old woman who developed puffiness in her fingers 1/2013 and carpal tunnel syndrome and arthralgias 3/2013. ROS was otherwise negative. Rheum and derm consults led to no diagnosis; CBC, CMP, ESR, TSH, CK, ANA, ENA, RF, CCP were all normal or negative. By 6/2013 she had discernable thickening of skin on hands, feet and ankles. A second rheumatologist diagnosed probable scleroderma. RNA polymerase III +. She was told to buy BP cuff and measure daily. Third opinion with me 2 weeks later confirmed diagnosis. Tendon friction rubs were palpated. Diagnosis of diffuse scleroderma confirmed. PPI and hand OT were started. ECHO was normal. PFTs showed mild restrictive disease (FVC 69%). In 8/2013 BP was 160 s/90 s. UA had trace protein and a few hyaline casts. She was hospitalized and captopril was begun. CT chest showed mild ground glass at bases consistent with NSIP. MMF was begun. By 12/2013 skin thickening above elbows and up to knees. In 4/2014 she was dizzy, had guaiac+ stool and was found to have GAVE; treated with argon plasma coagulation. By 6/2014 skin was improving (scores 31, 21,17 over 6 mos) In 10/2014 FVC 85%. SCLERODERMA Systemic Localized diffuse scleroderma linear scleroderma limited scleroderma morphea (CREST syndrome)

2 Morphea Linear scleroderma Epidemiology of Scleroderma Incidence: 5 10 new cases/million/year Sex ratio: F:M = 3 or 4:1 Age: Scleroderma hands: puffy phase Scleroderma hands: tight phase Digital telangiectasias and acroosteolysis Facial features of scleroderma

3 Excess dermal collagen in scleroderma Digital calcinosis Raynaud s phenomenon Conditions Associated with Raynaud s phenomenon Digital vessel response to cold Digital pitting scars

Dilated nailfold vessels Management of Raynaud s phenomenon Avoid the cold; keep

amphetamines, ergot-containing migraine meds, beta blocker Add vasodilators: Ca

up to 90% dysphagia, GERD, Barrett s and cancer Small intestine 20-60% hypomotility

incontinence Hepatobiliary rare; primary biliary cirrhosis Gastric antral vascular

4 Dilated nailfold vessels Management of Raynaud s phenomenon Avoid the cold; keep all body parts warm Avoid tobacco Avoid vasoconstrictors: cold and diet pills, amphetamines, ergot-containing migraine meds, beta blocker Add vasodilators: Ca channel blocker, alpha blocker, phosphodiesterase inhibitor (sildenafil, tadalafil); ASA? 20 Esophageal dysmotility in scleroderma GI involvement in scleroderma Esophageal up to 90% dysphagia, GERD, Barrett s and cancer Small intestine 20-60% hypomotility with bacterial overgrowth, leads to malabsorption Colon 10-40% constipation, fecal incontinence Hepatobiliary rare; primary biliary cirrhosis Gastric antral vascular ectasia (GAVE) Interstitial lung disease in scleroderma Up to 50% in diffuse, 25% in limited disease Cough, dyspnea NSIP > UIP Scl-70 is a risk factor No data on management yet

Pulmonary hypertension in scleroderma PH - involving small muscular arterioles 15-20% (PAH) - due to hypoxia/severe ILD 5-10% - due to myocardial dysfunction - rare Limited> diffuse disease, usually

Nt-proBNP are recommended for screening (beware FVC%/DLCO% > 1.

5 Pulmonary hypertension in scleroderma PH - involving small muscular arterioles 15-20% (PAH) - due to hypoxia/severe ILD 5-10% - due to myocardial dysfunction - rare Limited> diffuse disease, usually longstanding Often silent; dyspnea, chest pain may occur Endothelin receptor antagonists, sildenafil and IV epoprostenol improve exercise capacity, functional class Annual ECHO, PFT including DlCO, Nt-proBNP are recommended for screening (beware FVC%/DLCO% > 1.6) Scleroderma renal crisis Acute or subacute development of hypertension, relative or absolute Often with proteinuria, microangiopathy May lead to stroke, myocardial infarction, death Risk factors: diffuse disease first 3 5 years of disease rapidly progressive skin disease RNA polymerase III use of prednisone, cyclosporine Scleroderma renal crisis Early detection is key home BP monitoring, patient education Treatment is blockade of renin-angiotensin system, regardless of serum Cr Consider this a medical emergency! Best outcome if Cr<3.0 at diagnosis, but 30-40% needing dialysis can discontinue it Prophylaxis with ACE/ARB not recommended Autoantibodies in scleroderma Antibody % Associations ANA Centromere limited; PAH, ulcers Scl diffuse; ILD RNA polymerase III 4-20 diffuse; renal crisis; GAVE U1-RNP 5-10 MCTD Classification criteria for scleroderma Item Score Skin thickening proximal to MCPs 9 Puffy fingers 2 OR sclerodactyly up to MCPs 4 Digital tip scars 2 OR ulcers 3 Telangiectasia 2 Abnormal nailfold capillaries 2 Pulmonary arterial hypertension 2 OR ILD 2 Raynaud s phenomenon 3 Scleroderma-related antibody 3 (centromere, SCL-70 or RNA polymerase III) Differentiation of limited from diffuse scleroderma Limited Diffuse Skin distal distal and proximal Telangectasias Calcinosis Renal crisis 0 ++ Pulmonary ++ 0 hypertension Anticentromere year survival 90% 40% > 9 points is definite systemic sclerosis (Ann Rheum Dis 2013; 72: 1747)

Scleroderma mimics Diabetic chieroarthropathy longstanding, poorly controlled DM; fingers only Nephrogenic systemic fibrosis gadolinium given to CKD patients; skin, lungs, heart, neuropathy

6 Scleroderma mimics Diabetic chieroarthropathy longstanding, poorly controlled DM; fingers only Nephrogenic systemic fibrosis gadolinium given to CKD patients; skin, lungs, heart, neuropathy Scleredema: mucin deposition in skin of back or chest; DM, infection, myeloma Scleromyxedema: papular mucinosis esp on face; lung, GI, heart, joints; IgG MGUS Eosinophilic fasciitis: fibrosis of deep tissue on arms, legs and trunk, with eosinophilia Pathogenesis of Scleroderma Microvascular occlusion Immune cell activation Excess connective tissue; fibrosis (NEJM 2015; 372; 1056) Disease process and clinical manifestations Inflammatory Fibrotic alveolitis skin thickening myositis interstitial lung disease arthritis Vascular Atrophic renal crisis GI motility pulmonary hypertension skin ulcers Raynaud s Problem-oriented management of scleroderma Skin: methotrexate? Arthritis/serositis: NSAIDs Loss of mobility: PT and OT Raynaud s: dress warmly, avoid cold and tobacco; CCBs, PDE5 inhibitors, topical nitrates Digital ulcers: IV prostanoids, endothelin receptor antagonists, botulinum toxin Renal crisis: home BP checks; ACE inhibitors Problem-oriented management of scleroderma Pulmonary hypertension: endothelin receptor antagonist, prostacyclin, PDE5 inhibitor ILD: cyclophosphamide? MMF? GERD: PPI; may consider metaclopramide, erythromycin as pro-motility agents Malabsorption: rotating antibiotics (Annals of Rheumatic Diseases 2009; 68: Seminars in Arthritis and Rheumatism; 2013; 42:42-55)

7 Take home points on scleroderma PPI for everyone Screen for PAH, ILD annually Home BP monitoring, especially in diffuse disease Use ACE-I in renal crisis, regardless of serum Cr PT and OT can have a big impact

Scleroderma. Nomenclature Synonyms. Scleroderma. Progressive Systemic Sclerosis. Systemic Sclerosis. Edward Dwyer, M.D. Division of Rheumatology

Scleroderma. Nomenclature Synonyms. Scleroderma. Progressive Systemic Sclerosis. Systemic Sclerosis. Edward Dwyer, M.D. Division of Rheumatology Scleroderma Edward Dwyer, M.D. Division of Rheumatology Nomenclature Synonyms Scleroderma Progressive Systemic Sclerosis Systemic Sclerosis Scleroderma 1 Scleroderma Chronic systemic autoimmune disease