From the Guidelines
Mild systemic disease can be differentiated from severe systemic disease based on the ESSDAI score, with mild disease typically having a score of 1-4 and severe disease having a score of 14 or higher, or high activity in any of the ESSDAI domains. According to the EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies 1, the ESSDAI score is a helpful instrument to measure systemic involvement in primary Sjögren's syndrome. The score takes into account the extent of organ dysfunction, vital sign abnormalities, and impact on daily functioning.
- Mild systemic disease typically presents with minimal physiological disturbance, well-controlled symptoms, and limited impact on daily activities, with an ESSDAI score of 1-4.
- In contrast, severe systemic disease involves significant organ dysfunction, unstable vital signs, and substantial functional limitations, with an ESSDAI score of 14 or higher, or high activity in any of the ESSDAI domains, such as lymphadenopathy and lymphoma, articular, cutaneous, pulmonary, renal, muscular central and peripheral neurological and haematological domains. The distinction between mild and severe systemic disease is important for risk stratification, determining appropriate levels of care, and guiding treatment intensity across various medical specialties. Laboratory values, imaging findings, and functional capacity assessments can provide objective measures to help differentiate the severity 1. Additionally, the use of systemic therapies, such as glucocorticoids, antimalarials, immunosuppressive agents, intravenous immunoglobulins, and biologics, should be restricted to patients with active systemic disease, as defined by the ESSDAI score 1.
From the Research
Differentiation of Mild and Severe Systemic Disease
To differentiate mild systemic disease from severe systemic disease, several factors must be considered, including the severity of symptoms, the impact on daily life, and the presence of complications.
- Severity of Symptoms: Severe systemic disease is often characterized by more intense and widespread symptoms, such as high fever, significant weight loss, and severe fatigue, compared to mild systemic disease which may present with milder symptoms 2.
- Impact on Daily Life: Severe systemic disease can significantly impair an individual's ability to perform daily activities, whereas mild systemic disease may have a minimal impact on daily life 3.
- Presence of Complications: Severe systemic disease is more likely to be associated with complications, such as organ damage or failure, compared to mild systemic disease 4.
- Treatment Requirements: Severe systemic disease often requires more aggressive treatment, including immunosuppressive therapy, whereas mild systemic disease may be managed with less intensive treatments 5, 6.
- Response to Treatment: The response to treatment can also help differentiate between mild and severe systemic disease, with severe disease often requiring longer treatment durations and multiple therapeutic agents 2, 3.
Clinical Considerations
When evaluating the severity of systemic disease, clinicians must consider various clinical factors, including:
- Patient History: A thorough patient history is essential to understand the progression of the disease and the presence of any underlying conditions 5.
- Physical Examination: A comprehensive physical examination can help identify signs of severe disease, such as significant weight loss or organ dysfunction 4.
- Laboratory Tests: Laboratory tests, including blood work and imaging studies, can provide valuable information about the severity of the disease and the presence of complications 2, 3.
- Treatment Outcomes: Monitoring treatment outcomes is crucial to assess the effectiveness of the treatment plan and make adjustments as needed 6.