What is the treatment for flexion contractures in scleroderma?

Treatment of Flexion Contractures in Scleroderma

Flexion contractures in scleroderma should be managed with a comprehensive physical therapy program including daily stretching, thermal modalities, tissue mobilization, and structured upper extremity exercises, with surgical soft tissue release reserved for severe, refractory cases. 1, 2

Non-Pharmacological Management: First-Line Approach

Physical and Occupational Therapy

• Daily stretching exercises are essential and should be initiated at disease onset to prevent contracture development and progression. 1, 3
• Structured occupational therapy programs consisting of 8 weekly sessions with thermal modalities, tissue mobilization, and upper extremity mobility exercises produce statistically significant improvements in function (QuickDASH scores, p=0.0012) in patients with early diffuse SSc. 2
• Approximately 50% of patients achieve clinically meaningful improvements exceeding minimally important differences with this approach. 2
• Home exercise programs between therapy sessions are critical for maintaining gains. 2

Positioning and Orthotic Interventions

• Splinting should be used to counteract deforming forces, particularly ankle-foot orthoses for plantar flexion contractures, knee splints for knee flexion contractures, and resting wrist/hand/finger splints for flexor contractures. 1
• Correction of positioning throughout the day is necessary, as the number of hours a muscle remains in shortened position directly determines contracture risk. 1
• Serial casting is effective for contractures at the wrist, knee, and ankle joints. 4

Manual Lymphatic Drainage

• For patients with puffy hands and early contractures, manual lymphatic drainage (MLD) over 5 weekly sessions improves hand function, though benefits may not be fully sustained at 9-week follow-up. 1

Surgical Management: For Refractory Cases

Soft Tissue Release

• Soft tissue release is effective for severe contractures at the wrist, hip, and ankle when conservative measures fail. 4, 5
• At the MCP joint level, capsular excision with collateral ligament and volar plate release addresses the primary pathology of joint capsule contracture. 5
• At the PIP joint level, volar plate release combined with tight palmar skin release is necessary, as tight volar skin is the main contributor to flexion contracture at this level. 5
• Intensive hand therapy post-operatively is mandatory to maximize functional outcomes. 5

Important Surgical Considerations

• Surgery should not be avoided solely due to wound healing concerns, as successful outcomes are achievable with appropriate patient selection. 5
• Arthrodesis and arthroplasty can be avoided in many cases with proper soft tissue release techniques. 5

Critical Timing Considerations

Physical therapy interventions must be instituted at disease inception to minimize or prevent contracture development. 3 This is particularly crucial because:

• Contractures cause major morbidity and disability in scleroderma. 6
• Once established, contractures become progressively more difficult to reverse. 3
• Early intervention in patients with disease duration of approximately 3 years shows the best functional improvements. 2

Common Pitfalls to Avoid

• Do not delay physical therapy referral until contractures are severe—early intervention is far more effective than late-stage treatment. 3
• Avoid relying solely on pharmacological approaches, as drugs are usually of little benefit for established contractures. 3
• Do not underestimate the contribution of tight volar skin versus joint capsule pathology when planning surgical intervention—these require different release techniques. 5
• Ensure adequate post-operative or post-casting therapy, as gains are lost without continued mobilization. 1, 5

Monitoring and Adjustment

• Regular assessment of joint range of motion and functional status should guide therapy intensity. 2
• Treatment duration should extend for at least 8 weeks with weekly sessions to achieve meaningful functional improvements. 2
• Patients require ongoing home exercise programs to maintain gains achieved during supervised therapy. 2