Scleroderma, or systemic sclerosis, is a chronic autoimmune condition characterized by the abnormal buildup of connective tissue throughout the body. Musculoskeletal pain is a highly common feature of the disease. This process affects the skin, causing it to harden, and also impacts internal organs and joint structures, frequently leading to discomfort and reduced function. Joint pain is often among the earliest symptoms and is a major focus of management.
What Scleroderma Is and Its Link to Joints
Scleroderma is classified as an autoimmune rheumatic disease where the immune system mistakenly attacks its own tissues, resulting in the overproduction of collagen. This excessive collagen deposition, called fibrosis, causes the skin, blood vessels, and internal organs to thicken and harden. When the disease affects areas beyond the skin, it is termed systemic sclerosis, which is the form most associated with significant joint involvement.
Musculoskeletal pain is a frequent complaint, affecting between 40% and 80% of those with systemic sclerosis. Patients often report joint stiffness and pain, which can be an early sign of the condition, sometimes preceding a formal diagnosis. The disease is broadly categorized into limited and diffuse forms; joint symptoms, including tendon friction rubs and contractures, are often more problematic in the diffuse type. Joint problems arise because the same pathological process that stiffens the skin also impacts the connective tissues supporting movement.
How Scleroderma Affects Joint Tissue
Joint discomfort in scleroderma is rooted in the widespread fibrosis and inflammation characteristic of the disease. The joint lining, known as the synovium, can become inflamed, leading to synovitis. This inflammation within the joint space causes pain and swelling similar to other forms of inflammatory arthritis.
Beyond the joint itself, surrounding structures are also impacted by the buildup of scar-like tissue. Tendon sheaths, the coverings that protect tendons, can develop inflammatory fibrinous deposits. This causes a painful sensation of scraping or rubbing when the joint moves, known as tendon friction rubs. These rubs are particularly common in the wrists and ankles of patients with diffuse systemic sclerosis. The combination of synovial inflammation and fibrosis contributes significantly to persistent pain and stiffness.
Distinguishing Joint Pain from Joint Contractures
Scleroderma patients experience several distinct types of musculoskeletal problems, requiring differentiation of the source of discomfort. One common symptom is arthralgia, which is joint pain without clear signs of inflammation, swelling, or damage on imaging. This pain is often symmetrical and affects multiple joints, particularly in the hands. True inflammatory arthritis, or synovitis, involves detectable inflammation of the joint lining, presenting with swelling, warmth, and tenderness.
A separate, often more debilitating, issue is the development of joint contractures, which are fixed deformities that severely limit the range of motion. These contractures are typically caused by the tightening and thickening of the skin and underlying connective tissue, not damage inside the joint. As the skin around a joint, such as the fingers or elbows, becomes hard and inelastic, it physically pulls the joint into a bent position. This makes it difficult or impossible to fully straighten the limb. Understanding whether the problem is inflammatory pain, non-inflammatory pain, or mechanical restriction is important for directing appropriate treatment.
Managing Scleroderma-Related Joint Discomfort
Managing joint discomfort requires a comprehensive approach targeting both inflammation and mechanical restrictions. For mild pain or occasional inflammatory flares, nonsteroidal anti-inflammatory drugs (NSAIDs) may be used. Their use must be monitored due to potential side effects on the kidneys and digestive system. In cases of true inflammatory arthritis, low-dose corticosteroids or disease-modifying anti-rheumatic drugs (DMARDs) may be prescribed to suppress immune activity.
Non-pharmacological strategies are equally important, especially for combating stiffness and contractures. Physical and occupational therapy are essential to maintain strength and mobility through regular range-of-motion exercises. Consistent stretching helps keep the skin and joints flexible and can slow the progression of fixed deformities. Applying heat and cold therapy can also provide localized relief; heat helps ease stiffness, and cold reduces inflammation in a painful joint.