Scleroderma is an autoimmune, connective tissue disease characterized by the overproduction and excessive deposition of collagen, a protein that causes the hardening and tightening of tissue. The name scleroderma literally translates to “hard skin,” reflecting the most visible symptom of the condition. Scleroderma potentially affects blood vessels and internal organs like the lungs, heart, kidneys, and gastrointestinal tract. As a chronic condition, achieving a state of remission is an important question for those affected. The outcome depends heavily on the specific type of scleroderma a person has, ranging from frequent, natural resolution to a more complex state of disease control.
The Difference Between Localized and Systemic Scleroderma
Scleroderma is classified into two major categories: localized scleroderma and systemic sclerosis. Localized scleroderma, which includes the subtypes morphea and linear scleroderma, generally affects only the skin and the tissues immediately beneath it. This form typically does not progress to involve internal organs, and natural resolution is a common outcome.
Systemic sclerosis (SSc) is a more serious form that involves the skin alongside internal organs and the microvasculature. SSc is further divided into limited cutaneous SSc and diffuse cutaneous SSc, based on the extent of skin involvement. The diffuse form is often more aggressive, with rapid skin thickening and a higher risk of early internal organ involvement, while the limited form progresses more slowly. The systemic nature of SSc makes true, drug-free remission significantly rarer than in the localized form.
Defining Disease Stability and True Remission
For people with systemic sclerosis, “remission” is typically viewed as achieving “disease stability” or “quiescence” rather than a complete, drug-free cure. True remission, defined as a sustained absence of disease activity without ongoing treatment, is an infrequent occurrence for the systemic form. The goal of modern treatment is to stop the immune and fibrotic processes to prevent new, irreversible organ damage.
Disease stability is a more realistic therapeutic target, signifying that the disease activity has been effectively halted. This state is characterized by no further skin thickening, stabilization of organ function, and management of inflammatory markers. Although skin thickening in diffuse SSc may naturally soften after a period of activity, this softening alone does not confirm stability, as the underlying systemic process may still be active. Achieving and maintaining stability often requires continuous, long-term therapy to suppress autoimmune activity.
Treatment Approaches for Halting Disease Progression
Treatment protocols for systemic sclerosis focus on controlling immune system dysfunction and preventing fibrosis. Immunosuppressive drugs are used to achieve disease stability by calming the overactive immune response. Medications such as mycophenolate mofetil (MMF) and cyclophosphamide are commonly used, particularly to manage interstitial lung disease (ILD), a frequent complication of SSc.
Targeted therapies, including biologic agents, are increasingly used to interrupt specific disease pathways. Rituximab, a B-cell depleting therapy, has demonstrated effectiveness in reducing skin disease and improving lung function in some patients. Newer antifibrotic agents like nintedanib slow the decline in lung function for patients with SSc-associated ILD, directly addressing the fibrotic component. Successful intervention is the primary pathway to achieving the state of stability that mimics remission for systemic disease.
Methods for Monitoring Disease Activity
Physicians rely on clinical assessments and tests to determine if a patient has reached or maintained disease stability. The Modified Rodnan Skin Score (mRSS) is a standardized tool used to measure the extent and severity of skin thickening across 17 body areas. A reduction in this score is a positive indicator of improvement.
Internal organ involvement is monitored through specific functional tests and blood work. Pulmonary function tests (PFTs), including measurements of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO), track lung function and detect progression of ILD. Inflammatory markers, such as C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR), are monitored in blood tests, as their normalization signals reduced systemic inflammation and maintenance of stability.