A patientwithsystemicsclerosis andjointpain. Christian Beyer University Erlangen-Nuremberg(GER)

Transcription

1 A patientwithsystemicsclerosis andjointpain Christian Beyer University Erlangen-Nuremberg(GER)

2 Whatisimportanttoyou? lungs GI tract fatigue heart skin muscles Raynaud s joints

3 Whatisimportanttopatients? fatigue pain joints

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5 Joint involvementandqualityoflife Severalcross-sectionalstudieshighlighta majorimpacton qualityoflife. (Baron M et al., ARD 1982; Mau W et al., J Rheumatol2005; BrowerLM, Arthritis Rheum2004; Poole JL et al., Arthritis Res Care 2000; Poole JL, Arthritis Res Care 1991) The symptoms patients most frequently associated with SSc arestiffjoints(79%), pain(75%), andfatigue(75%). (Richards HL, Arthritis Rheum 2003) Joint involvementimpairesadls andhandmobility. (SandqvistG et al., Scand J Rheumatol 2004) Dexterity reduced by 68-80% Grip force reduced by 46-65% Joint involvement impaireshand function as assessed by the Duröz Hand Index. (Brower LM, Arthritis Rheum 2004) Mean score / In RA around 17, in OA around 19

6 Case male, 36 years, electrician, smoking 20 PYs, occasionally marihuana 04/10: morning stiffness, arthralgias of wrists, MCPs, PIPs 09/10: seen by rheumatologist in private practice

7 Case male, 36 years, electrician, smoking 20 PYs, occasionally marihuana 04/10: morning stiffness, arthralgias of wrists, MCPs, PIPs 09/10: seen by rheumatologist in private practice symmetrical, tender and swollen wrists, MCPs and PIPs RF pos., anti-ccp-ab pos., anti-mcv-ab pos. diagnosis: Rheumatoid arthritis; therapy: GCS + methotrexate 15 mg/w

8 Case male, 36 years, electrician, smoking 20 PYs, occasionally marihuana 04/10: morning stiffness, arthralgias of wrists, MCPs, PIPs 09/10: seen by rheumatologist in private practice symmetrical, tender and swollen wrists, MCPs and PIPs RF pos., anti-ccp-ab pos., anti-mcv-ab pos. diagnosis: Rheumatoid arthritis; therapy: GCS + methotrexate 15 mg/w 11/10: treatment stop since no improvement 12/10: new-onsetraynaud s, skinthickeningofbothhands, lowerarms, feet, lowerleg andface

9 Case 02/11: inpatient at a larger rheumatology center Scleroderma of arms and legs, trunk, face No tender joints, MCPs, PIPs, DIPs with swollen character ANA 1:160, anti-topo neg., anti-centromer neg. Capillaroscopy: capillary rarefications, megacapillaries US ofthehands: tenosynovitisofflexorandextensortendonsofbothhandsclosetothewrists X-ray of hands: normal

10 Case 02/11: inpatient at a larger rheumatology center Scleroderma of arms and legs, trunk, face No tender joints, MCPs, PIPs, DIPs with swollen character ANA 1:160, anti-topo neg., anti-centromer neg. Capillaroscopy: capillary rarefications, megacapillaries US ofthehands: tenosynovitisofflexorandextensortendonsofbothhandsclosetothewrists X-ray of hands: normal Normal chest X-ray, echocardiography, lung function testing EGD: hypomotile esophagus diagnosis: overlapsyndromeofra andssc; therapyrecommendation: GCS + methotrexate25/w (+ atnf)

11 Case 04/11: rheumatologist in private practice No improvement Referral to us 05/11: seen in our center Scleroderma of arms and legs, trunk, face: mrss 27 Flexion and extension contractions of MCPs, PIPs and DIPs of both hands No swollen and tender joints ANA 1:160, anti-topo neg., anti-centromer neg., anti-rna-polyermase 3 pos., anti-ro pos. diagnosis: overlap syndrome of RA and SSc treatment: how to proceed?

12 A rare caseofsscjointdisease OverlapbetweenRA andsscisrare 30% RF pos., anti-ccp rare (Blockaet al, Arthritis Rheum1981; AvouacJ et al. ARD 2006; AvouacJ et al., J Rheumatol 2010) Pathophysiology Rare: classicalsynovitiswithpannustissue(overlapwithra?) Often: mild inflammation, synovial fibrosis, tethering and contracture of the surounding tissues Synovial fluid: less than 2000 cells/mm³; predominantly monocyptes (Schumacher JrHR, Am J ClinPathol1973) AvouacJ et al., Rheumatology 2012 Iagnocco A et al, Medical Ultrasonography 2012

13 Pattern ofjointinvolvement Arthralgias: among the most frequent presenting symptoms ofssc (Baron M et al., ARD 1982; TuiffanelliDL, ArchDermatol1961) Presenting symptom 12-65% Eventual manifestation 46-97% EUSTAR database: 16% synovitis, 31% jointcontracture (AvouacJ et al, J Rheumatol 2010) Mild jointeffusionsin around50% ofpatientswithsscbyus (Cuomo G et al, Rheumatology 2009) Virtuallyall jointscanbeaffected, mostcommonlymcps, PIPs, wrists and ankles Symmetrical polyarticular(60%), oligoarticular(20%) and monoarticular(20%) (La Montagna G et al, Semin Arthritis Rheum 2002; La Montagna G et al., Skeletal Radiol 2005)

14 Radiographicchanges Radiographicerosivediseaseupto5 to40% in SSc patients (Baron M et al., Arthritis Rheum1982; AvouacJ et al, ARD 2006; La Montagna G et al, SeminArthritis Rheum2002; La MontagnaG et al., SkeletalRadiol2005) Radiographic lesions: Juxta-articular osteoporosis, joint space narrow., erosions (Baron M et al., Arthritis Rheum1982; AvouacJ et al, ARD 2006; La MontagnaG et al, SeminArthritis Rheum 2002; La Montagna G et al., Skeletal Radiol 2005) Selective involvement of the first carpometacrapalphalangealjoint (ResnickD et al, AJR 1978) Limited data for ultrasound and MRI

15 Avouac J et al., Rheumatology 2012

16 Joints predictdiseasecourse PatientswithearlySScandsynovitisaremore likelytodevelopthediffuse cutaneousanda morefulminant diseasecourse (Avouacet al., ARD 2014; Maurer et al. ARD 2014)) Joint disease: increase risk for vascular and muscle involvement

17 Joint involvement Treatment: no trials Physical and occupational therapy NSAIDs and low-dose GCS MTX CYC doesnot havebeneficaleffects: SLS (Au K et al, Arthritis Res Care 2010) TNFalpha: goodforthejoints, badforthelungs? (Lam GK, J Rheumatol2007; AllanoreY, ARD 2006; OstorAJ, BMJ 2004) RTX (Smith V, ARD 2010; Lafayatis R, Arthritis Rheum 2009; Daoussis D, Rheumatology 2009; Jordan S, ARD 2014) TOC

18 Tendoninvolvement leatherycrepitus : tendonfrictionrubs (AvouacJ et al, J. Rheumatol2010; Steen VD, Arthritis Rheum 1997) Common 11 to 28% (i.p. early diffuse disease) In the leg: tiabilis anterior, perenous muscles In the forearm: tendons of the flexor or extensor muscles immediately proximal to the wrist Thickened reticula of tendons (Cuomo et al., Arthritis Rheum 2012)

19 Tendoninvolvement Risk prediction: Associated withdus, muscleweakness, pulmonary fibrosis, proteinuria Treatment: Favourable course of the manifestation Tenosynovitis: NSAIDs, low doses GCS Tendon friction rubs: symptomatic

20 Summary Joint/tendon involvement Reduces quality of life Predicts severe disease Is still poorly understood and often overlooked

21 Other facts modifiedritchieindex, DAS28 (Baron M et al., ARD 1982)