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

(1)

A patient with systemic sclerosis

and joint pain

Christian Beyer

(2)

What is important to you?

lungs

heart

Raynaud‘s

GI tract

muscles

skin

fatigue

(3)

What is important to patients?

fatigue

_pain

(4)

(5)

Joint involvement and quality of life

• Several cross-sectional studies highlight a major impact on

quality of life.

(Baron M et al., ARD 1982; Mau W et al., J Rheumatol 2005; Brower LM, Arthritis Rheum 2004; Poole JL et al., Arthritis Res Care 2000; Poole JL, Arthritis Res Care 1991)

• The symptoms patients most frequently associated with SSc

are stiff joints (79%), pain (75%), and fatigue (75%).

(Richards HL, Arthritis Rheum 2003)

• Joint involvement impaires ADLs and hand mobility.

(Sandqvist G et al., Scand J Rheumatol 2004)

–

Dexterity reduced by 68-80%

–

Grip force reduced by 46-65%

• Joint involvement impaires hand function as assessed by the

Duröz Hand Index.

(Brower LM, Arthritis Rheum 2004)

–

Mean score 21.1 +/- 19.25

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Case

• male, 36 years, electrician, smoking 20 PYs, occasionally marihuana

• 04/10: morning stiffness, arthralgias of wrists, MCPs, PIPs

(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.

(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-onset Raynaud‘s, skin thickening of both hands, lower arms, feet,

lower leg and face

(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 of the hands: tenosynovitis of flexor and extensor tendons of both hands close to the wrists

(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 of the hands: tenosynovitis of flexor and extensor tendons of both hands close to the wrists

– X-ray of hands: normal

– Normal chest X-ray, echocardiography, lung function testing

– EGD: hypomotile esophagus

– diagnosis: overlap syndrome of RA and SSc; therapy recommendation: GCS + methotrexate 25/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

(12)

A rare case of SSc joint disease

• Overlap between RA and SSc is rare

–

30% RF pos., anti-CCP rare

(Blocka et al, Arthritis Rheum 1981; Avouac J et al. ARD 2006; Avouac J et al., J Rheumatol 2010)

• Pathophysiology

–

Rare: classical synovitis with pannus tissue (overlap with RA?)

–

Often: mild inflammation, synovial fibrosis, tethering and contracture

of the surounding tissues

–

Synovial fluid: less than 2000 cells/mm³; predominantly monocyptes

(Schumacher Jr HR, Am J Clin Pathol 1973)

Iagnocco A et al, Medical Ultrasonography 2012 Avouac J et al.,

(13)

Pattern of joint involvement

• Arthralgias: among the most frequent presenting symptoms

of SSc

(Baron M et al., ARD 1982; Tuiffanelli DL, Arch Dermatol 1961)

–

Presenting symptom 12-65%

–

Eventual manifestation 46-97%

• EUSTAR database: 16% synovitis, 31% joint contracture

(Avouac J et al, J Rheumatol 2010)

• Mild joint effusions in around 50% of patients with SSc by US

(Cuomo G et al, Rheumatology 2009)

• Virtually all joints can be affected, most commonly MCPs,

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)

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Radiographic changes

• Radiographic erosive disease up to 5 to 40% in SSc

patients

(Baron M et al., Arthritis Rheum 1982; Avouac J et al, ARD 2006; La Montagna

G et al, Semin Arthritis Rheum 2002; La Montagna G et al., Skeletal Radiol 2005)

• Radiographic lesions:

–

Juxta-articular osteoporosis, joint space narrow., erosions

(Baron M et al., Arthritis Rheum 1982; Avouac J et al, ARD 2006; La Montagna G et al, Semin Arthritis Rheum 2002; La Montagna G et al., Skeletal Radiol 2005)

–

Selective involvement of the first

carpometacrapal phalangeal joint

(Resnick D

et al, AJR 1978)

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Joints predict disease course

• Patients with early SSc and synovitis are more

likely to develop the diffuse cutaneous and a

more fulminant disease course

(Avouac et 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 does not have benefical effects: SLS

(Au K et al, Arthritis Res Care 2010)

–

TNFalpha: good for the joints, bad for the lungs?

(Lam GK, J Rheumatol 2007; Allanore Y, ARD 2006; Ostor AJ, BMJ 2004)

–

RTX

(Smith V, ARD 2010; Lafayatis R, Arthritis Rheum 2009; Daoussis D, Rheumatology 2009; Jordan S, ARD 2014)