Musculoskeletal manifestations of scleroderma are common although variable and are a major contributor to morbidity ⁷.

For a general discussion of scleroderma, please refer to the parent article: scleroderma.

Epidemiology

Symptomatic joints are present in ~40% (range 12-65%) of patients when diagnosed and overall are present in ~60% (range 24-97%) of patients at sometime during their illness course ^6,8.

Clinical presentation

Musculoskeletal presentations of scleroderma stiffness, pain, reduced dexterity, reduced power, which is often multifactorial in etiology ^6-8:

• joints:

  • arthralgia/arthritis in a non-specific oligo- or poly-articular pattern

  • fingers (particularly proximal interphalangeal and metacarpophalangeal joints), wrist and ankles are most commonly involved although any joint can be affected ⁸

• tendons: tenosynovitis, tendon friction rub

• bursae: trochanteric and/or olecranon bursitis ⁸

• skin: cutaneous thickening resulting in contractures, soft tissue calcification

• muscles: fibromyalgia, myositis (also see overlap myositis)

• nerves:

  • carpal tunnel syndrome: thought to be from flexor tendon abnormalities in the cubital tunnel ⁸

  • cubital tunnel syndrome ⁶

Radiographic features

Plain radiograph

Articular findings

• acro-osteolysis (resorption of the distal phalanges) ^2-4

• periarticular osteopenia ^2-4,6

• joint space narrowing ^2-4,6

• rheumatoid arthritis-like joint erosions ^2-4,6

• severe resorption of the first carpometacarpal joint with radial subluxation is common ^2-4,7

• pencil-in-cup deformity ⁵

Extra-articular findings

• subcutaneous and periarticular calcification ^2-4

• soft tissue atrophy, especially at tips of fingers ^2-4

• flexion contractures: small joint contractures present in ~30% ^6,8

Less common musculoskeletal findings

• rib resorption (bilateral superior rib notching, predominantly along posterior surface), mandibular angle resorption (+/- loss of lamina dura), radius and ulna resorption

• terminal phalangeal sclerosis

Ultrasound

Articular findings

• joint effusion (common) ⁶

• synovial thickening/synovitis (common) ⁶

• joint erosion ⁶

Extra-articular findings

• tenosynovitis (common) ⁶

  • tendon sheath thickening and fibrinous deposits may be present ⁸

  • may be a stenosing tenosynovitis ⁶

  • may be an inflammatory tenosynovitis predisposing to tendon rupture ⁸

• A1 flexor pulley thickening (associated with reduced hand mobility) ⁷

Differential diagnosis

• rheumatoid arthritis: similar appearance with joint erosions and anti-CCP positivity ¹

See also

• pulmonary manifestations of scleroderma

• cardiac manifestations of scleroderma

• gastrointestinal manifestations of scleroderma

• hepatobiliary manifestations of scleroderma

• renal manifestations of scleroderma

• differential diagnosis of erosive arthritis

• overlap myositis