Systemic Sjogren's

Category 1- ESSDAI features

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The EULAR  Sjogren's syndrome disease activity index (ESSDAI) is the classic tool used by Sjogren's researchers to measure systemic disease activity. While this is a useful research tool, using the ESSDAI alone to define systemic involvement is problematic on many fronts. (LINK)  

 Some researchers consider the systemic features measured by the  12 ESSDAI domains as synonymous with “Extraglandular Manifestations” (EGM).  Some 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."

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. Lymphadeneopathy 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. The most common glancular 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 (RA) (45).
     

  5. Cutaneous (skin)- Skin lesions including erythema multiforme, subcutaneous lupus, and cutaneous vasculitis.
     

  6. Pulmonary (lungs)- Numerous manifestations, including small and large airway disease, interstitial lung disease, and more rarely, pulmonary hypertension (39, 54,89,150)
     

  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 in EGM with a “low score” if tests are normal.
     

  9. Peripheral nervous system (PNS)- Numerous manifestations: large and small fiber neuropathies are fairly common. The most common PNS feature, dysautonomia, is not included in the ESSDAI scoring system. See blog posts 1-15-19 and 4-11-19, and  https://www.hopkinssjogrens.org/disease-information/sjogrens-syndrome/neurologic-complications/ 
     

  10. Central nervous system (CNS)-  Less common than PNS. MS-like syndrome, cerebral vasculitis, transverse myelitis and others.  See link to immediately above. 
     

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

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

Most Sjogren’s patients have at least one of these EGMs as measured by the ESSDAI  (38, 61, 92


 Multiple EGMs would be the norm if "out of ESSDAI" EGM and the vastly underdiagnosed peripheral neuropathies were counted. ( 50, 53   87, 96, 138)

Updated 7-31-20