SYSTEMIC SJOGREN'S

CATEGORY 1 - ESSDAI FEATURES

What is the ESSDAI? 

The EULAR  Sjogren's syndrome disease activity index (ESSDAI) is the classic tool used by Sjogren's researchers to measure systemic disease activity. It is impractical for clinicians to measure the ESSDAI in routine clinical settings. While it is is a useful research tool, using the ESSDAI alone to define systemic involvement fails to capture many systemic Sjogern's manfestations. 

How systemic Sjogren's features are defined

Terminology used to describe systemic Sjogren's manifestations is often confusing. Some researchers consider the systemic features measured by the 12 ESSDAI domains to be synonymous with “Extraglandular Manifestations” (EGM). Some researchers use the term "systemic" to mean ESSDAI features only. Others use the terms "systemic" and "EGM" more broadly. Therefore, it is important to define exactly what is meant when using the terms "EGM" or "systemic" when talking with rheumatologists about systemic manifestations. 

Most Sjogren’s patients have at least one systemic (EGM) feature as measured by the ESSDAI  (3861, 92) 


Multiple EGMs would be the norm if "out of ESSDAI" EGM especially small fiber neuropathy and autonomic disorders were counted (50, 53, 87, 96138).

The 12 "official" EGM categories:  A Brief Summary  (10, 37, 38)

  1. Constitutional - Fever, night sweats, and involuntary weight loss not thought to be due to other causes.
     

  2. Lymphadenopathy and lymphoma - Lymph node swelling not thought to be due to infection or other cause.  Lymphoma, usually low grade, impacts 5-10%.
     

  3. Glandular - This does not mean sicca. It refers to swelling of the salivary or tear glands, not dryness. The most common glandular feature is parotid gland enlargement. 
     

  4. Articular (joints) - Pain and stiffness of joints, especially of the hands, ankles, and feet, often accompanied by morning stiffness. Inflammatory arthritis may occur, although without the joint erosions seen in rheumatoid arthritis (45).
     

  5. Cutaneous (skin) - Skin lesions including erythema multiforme, subcutaneous lupus, cutaneous vasculitis, and others. It does not include dryness of the skin, a very common Sjogren's feature.
     

  6. Pulmonary (lungs) - Numerous manifestations, including small and large airway disease, interstitial lung disease, and more rarely, pulmonary hypertension (39, 5489,150)
     

  7. Renal (kidneys) - renal tubular acidosis (often mild), interstitial kidney disease, glomerulonephritis
     

  8. Muscular - Myositis may cause muscle weakness. Muscle pain without weakness is included with a “low score” if tests are normal.
     

  9. Peripheral nervous system (PNS) - Numerous manifestations: large and small fiber neuropathies (SFNs), cranial neuropathies, and ganglionopathies. SFN can be found in many Sjogren's patients, but is often overlooked. 
    Autonomic disorders, along with SFN, are the most common Sjogren's neurological manifestations. Autonomic disorders are not included in the ESSDAI, and SFN tends to be overlooked. Both require special tests that go beyond typical neurological examination.
     

  10. Central nervous system (CNS) - MS-like syndrome, cerebral vasculitis, transverse myelitis, and others. Less common than PNS disorders. CNS involvement would be highly prevalent if headache and cognitive problems (brain fog), and mood changes were counted like they are for systemic lupus erythematosus.  
     

  11. Hematological - Anemia (normocytic) not due to another cause, thrombocytopenia (low platelets), lymphopenia (low lymphocytes), neutropenia (low neutrophils), autoimmune hemolytic anemia.
     

  12. Biological - Low complement, hypergammaglobulinemia, high IgG or recent decrease of IgG level, cryoglobulinemia.

Updated 08-20-2022