From the Guidelines
Sclerotic degeneration is a pathological process characterized by the hardening or stiffening of tissues due to the abnormal accumulation of fibrous connective tissue, particularly collagen, as seen in systemic sclerosis 1. This condition typically occurs as part of aging or in response to chronic inflammation, injury, or disease. In sclerotic degeneration, normal tissue is gradually replaced by dense, fibrous scar tissue, leading to decreased elasticity and function of the affected organ or structure. The process can affect various parts of the body, including:
- Blood vessels (arteriosclerosis)
- The liver (cirrhosis)
- Kidneys (glomerulosclerosis)
- Heart valves
- Joints The underlying mechanism involves an imbalance in tissue repair processes, where excessive collagen production and reduced breakdown lead to fibrosis, as discussed in the context of systemic sclerosis treatment 1. As sclerotic changes progress, the affected tissues become increasingly rigid and less functional, which can ultimately impair organ function. Treatment approaches depend on the specific organ affected and typically focus on managing the underlying cause, slowing progression, and addressing symptoms, as the fibrotic changes themselves are often irreversible, with treatments such as mycophenolate mofetil and nintedanib being used in the treatment of systemic sclerosis-related interstitial lung disease 1. The management of sclerotic degeneration should prioritize the reduction of morbidity and mortality, and improvement of quality of life, through the use of evidence-based treatments, such as those outlined in the state-of-the-art evidence in the treatment of systemic sclerosis 1.
From the Research
Definition and Overview
- Sclerotic degeneration refers to the process of sclerosis or hardening of tissues, which can occur in various diseases, including systemic sclerosis (SSc) or scleroderma 2, 3, 4, 5.
- Systemic sclerosis is a rare and chronic connective tissue disease characterized by fibrosis of the skin and internal organs, as well as vasculopathy 6, 3, 4, 5.
Pathogenesis and Clinical Features
- The etiopathogenesis of SSc involves vasculopathy and immune system dysregulation, occurring on a permissive genetic and epigenetic background, ultimately leading to fibrosis 3, 5.
- The disease is characterized by progressive skin fibrosis and other clinically heterogeneous features, including autoantibody profiles that are predictive of skin and internal organ involvement and disease course 3.
- Sclerosing disorders of the skin consist of a heterogeneous spectrum of entities that share in common cutaneous sclerosis with excessive local accumulation of collagen and/or other extracellular matrix components in the dermis, subcutaneous tissue, and/or underlying soft tissues 2.
Diagnosis and Management
- A correct diagnosis of sclerotic degeneration is extremely significant, as these disorders are very different in terms of pathogenesis, course, treatment, and outcome 2.
- Clinicopathologic correlation, immunohistochemical staining, and other laboratory data may be required to arrive at a proper diagnosis in doubtful cases 2.
- Early diagnosis of SSc, with commencement of disease-modifying treatment, has the potential to improve patient outcomes 3.