What are the characteristics of Connective Tissue Disease (CTD)?

From the Guidelines

Connective tissue diseases are a group of disorders characterized by inflammation and dysfunction of the body's connective tissues, which include skin, joints, and blood vessels, and are associated with significant morbidity and mortality, particularly due to interstitial lung disease (ILD) (1). The characteristics of connective tissue disease (CTD) include a wide range of clinical manifestations and outcomes, with ILD being a major complication (1).

Common Characteristics of CTD

• Inflammation and dysfunction of connective tissues
• Autoimmune conditions where the immune system mistakenly attacks healthy tissues
• Common examples include systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma, dermatomyositis, and Sjögren's syndrome
• Significant morbidity and mortality, particularly due to ILD

Interstitial Lung Disease (ILD) in CTD

• ILD is a major complication of CTD, contributing to significant morbidity and mortality (1)
• The epidemiology of ILD in CTD is difficult to determine due to undefined classification criteria, overlap syndromes, and undifferentiated CTD (1)
• Early detection and accurate diagnosis of ILD are essential for informed treatment decisions and prognosis (1)

Treatment and Management of CTD

• Treatment typically involves a combination of medications to manage inflammation and immune system activity
• For mild symptoms, nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or naproxen may provide relief
• Corticosteroids such as prednisone are often used to quickly reduce inflammation during flares
• Disease-modifying antirheumatic drugs (DMARDs) like methotrexate, hydroxychloroquine, or mycophenolate mofetil are prescribed for long-term management
• Biologic agents targeting specific immune pathways, such as rituximab, belimumab, or TNF inhibitors, may be necessary for refractory cases
• Management requires regular monitoring of disease activity, medication side effects, and organ function through blood tests and clinical evaluations
• Lifestyle modifications including regular exercise, stress management, and avoiding sun exposure (particularly for lupus patients) are important complementary approaches

Importance of Early Diagnosis and Treatment

• Early diagnosis and treatment are crucial to prevent organ damage and improve quality of life (1)
• Delayed diagnosis and treatment can lead to irreversible lung function loss and increased mortality (1)

From the Research

Characteristics of Connective Tissue Disease (CTD)

• Connective tissue diseases are a varied group of disorders with major musculoskeletal manifestations such as joint pain and loss of function 2
• CTDs can cause protean organ-related consequences, making patients with chronic CTD a highly challenging population in the perioperative context 2
• Interstitial lung disease is one of the most critical manifestations of connective tissue diseases that may cause morbidity and mortality 3
• The frequencies of CTDs can vary, with Sjogren's syndrome, rheumatoid arthritis, and systemic sclerosis being among the most common 3
• Overlap Syndromes (OSs) can occur, where a patient satisfies classification criteria of at least two connective tissue diseases (CTDs) occurring at the same or at different times 4
• Clinical manifestations observed in OS may be different from those observed in the single CTD 4
• Immunosuppressive treatment is often used to manage CTDs, and may be essential in patients with worsening symptoms, impaired pulmonary function tests, and radiological findings 3, 5
• Treatment approaches should be assessed in a patient-based way, and patients under immunosuppressive treatment should be cautiously followed for infections 3

Clinical Characteristics

• The clinical and demographic characteristics of patients with CTD-related interstitial lung disease can vary, with a mean age of 63.4 ± 11.9 years in one study 3
• Nonspecific interstitial pneumonia is the most common interstitial lung disease pattern in patients with Sjogren's syndrome and systemic sclerosis 3
• Usual interstitial pneumonia is most frequent among patients with rheumatoid arthritis 3
• Mortality rates can be higher in patients with certain CTD subtypes, such as systemic sclerosis 6

Treatment

• Immunosuppressive treatment is the mainstay of therapy for CTDs, but evidence for its effectiveness in slowing the progression of ILD is limited 5
• Targeted therapies for pulmonary hypertension can mitigate clinical deterioration in CTD patients with isolated PH, but the effect of these therapies in CTD patients with PH and ILD is poorly characterized 6
• Nintedanib has been approved to slow decline in lung function in patients with systemic sclerosis-associated ILD and chronic fibrosing ILDs with a progressive phenotype 5