Promoting optimal care for Sjogren's, a common, serious, systemic disease.
GENERAL SYSTEMIC
MANIFESTATIONS
What we see are patients who come in with arthritis or rashes, but what they’re concerned with is losing their jobs because of their [term: cognitive] changes. These need to be triaged in an immediate fashion and not simply blown off because they change family dynamics and the loss of income,” he said."
~ Dr. Robert Fox, IAS presentation April, 2021
Introduction
Patients and doctors tend to think about diseases based on visible and measurable manifestations. This partly explains why dryness (sicca) gets so much attention in Sjogren’s. When there is an over-emphasis on sicca features, other common manifestations such as debilitating flu-like fatigue, lung disease, and neurologic complications tend to be overlooked (2, 3, 22, 24, 32).
Multiple studies have shown that fatigue and pain, not dryness, are the primary reasons for reduced quality of life (QOL) in Sjogren's patients (169,193) These are the features that keep many patients from living a normal life.
Severe fatigue, widespread pain, flu-like malaise and brain fog are sometimes called “benign” manifestations in the academic literature. This unfortunate term further perpetuates an already dismissive attitude toward Sjogren’s. A better name for these features is "general systemic manifestations". They are general because they impact the entire body. They are systemic because they are not sicca manifestations and should never be labeled a part of "sicca syndrome". Sjogren's is always systemic and should never be referred to as "sicca syndrome". The vast majority of patients experience some or all of these general systemic features (2,10, 26).
Sjogren's patients, on average, score worse on QOL measures than patients with related diseases that tend to be taken more seriously, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and systemic sclerosis (94). The burden of these general systemic manifestations can be devastating. The functional disability in Sjogren’s has been shown to be as great as it is in SLE and in RA patients with active disease who fail TNF therapy (27, 43, 44).
More Than Dry
Health-related quality of life (HRQOL) is more dependent on fatigue, pain, and depression than on the severity of dryness symptoms (27). Yet little research has been done about these core features. They are often ignored by clinicians or dismissed as stress or anxiety. It can be hard for clinicians (and family members) to “get it” that a patient who looks well and has relatively normal labs can feel so sick.
"In pSS, QoL has been found to be associated with fatigue, pain/articular involvement, ocular and oral involvement, pruritus, sexual dysfunction, impaired sleep, pulmonary manifestations, psychological dysfunction and impaired physical function. On the other hand, delayed diagnosis, protracted course of the disease, and the lack of social support are also causes of the decline in the quality of life in these patients" (169)
For most Sjogren's patients, these invisible systemic features are the most devastating part of the disease. Kathy Hammitt of the Sjogren's Foundation wrote an excellent article about the impact of these features on function and quality of life. I highly recommend that patients read and share her article (161) with their rheumatologists and other clinicians. Click on the box that says "free to view" to print Kathy Hammitt's article.
It is also important that rheumatologists and PCPs be familiar with the Sjogren's Foundaton Clinical Practice Guidelines for systemic management, including pain and fatigue. Please share them with your clinicians.
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Key Links & Self-Advocacy Tools
See Myths # 6 and #7 on the MYTHS ABOUT SJOGREN'S page to for self-advocacy tools that can help you counter misconceptions about fatigue, pain, and quality of life.
Updated 01-30-2024