SJOGREN'S IS SYSTEMIC
It affects your entire body
Sjogren’s manifestations include sicca (dryness) and systemic (non-sicca) manifestations. This page describes systemic manifestations, using these three categories:
Classic (ESSDAI) Systemic Manifestations
Other (non-ESSDAI) Systemic Manifestations
General Systemic Manifestations
Manifestations are not the same thing as symptoms. Manifestations are disease states that are associated with signs (measurable/observed) and symptoms (what the patient feels).
Systemic manifestations are caused directly by Sjogren’s disease; comorbidities are not.
Clinicians sometimes confuse the two.
Learn about manifestations vs comorbidities.
Although studies have clearly demonstrated that Sjogren’ is a multisystem disease, many doctors still think of it as a sicca (dryness) disease. Sjogren’s is never a sicca-limited disease. Sjogren’s can impact every organ and system in the body. (38, 243).
One or more systemic manifestations can be found in nearly every Sjogren’s patient who is thoroughly evaluated (38, 92). Despite this, “the evaluation of the systemic manifestations of SS are not properly incorporated into clinical practice” (241).
Knowing how your clinician thinks about Sjogren’s will help you advocate for Sjogren’s care that goes beyond sicca. The Sjogren’s Is More Than Just Sicca Handout and User Guide offers tools and strategies for discussing the serious, systemic nature of the disease with clinicians.
“Systemic” has a special meaning in Sjogren’s.
Sicca manifestations are not referred to as systemic, even when sicca occurs in multiple areas of the body.
A systemic manifestation occurs when Sjogren's causes damage or dysfunction to any part of the body that is not a fluid-secreting (exocrine) gland.
Manifestations vs. Symptoms
Patients usually think about diseases based on symptoms. This is because symptoms describe the patient’s experience of the disease.
Clinicians usually think about diseases based on manifestations.
Manifestations are disease states that are associated with signs (measurable/observed) and symptoms (what the patient feels).
Some manifestations have no obvious symptoms. Other manifestations have no obvious signs.
Learn more about signs, symptoms, and manifestations.
How Clinicians Think About Systemic Manifestations
Sicca manifestations often get the most attention in clinical practice and research. Systemic manifestations are often overlooked, downplayed, or misattributed to other conditions. Because there is no consistent medical education about Sjogren's, diagnosis and management varies widely.
Learn more about Sjogren’s rheumatology care
Systemic disease terms can be confusing, even to clinicians.
Multiple terms are used to describe systemic manifestations. These terms do not always mean the same thing from one clinician to another. It is important to clarify what your doctor means any time they use the following terms:
Systemic manifestations- non-sicca manifestations. These can be divided into the three categories described below: Classic (ESSDAI) systemic manifestations, Other (non-ESSDAI) Systemic Manifestations, and General Systemic manifestations. Some clinicians ignore or minimize the manifestations include in the “Other” and “General” categories.
Glandular manifestations-sicca manifestations. Sometimes used more broadly to include both sicca and parotid gland enlargement which is a Classic (ESSDAI) Manifestation (systemic).
Extraglandular manifestations (EGM)- systemic manifestations. Clinicians frequently have unique and creative definitions of this term.
Organ system involvement- various definitions. This may refer to systemic manifestations that impact the solid organs, e.g., lung, kidney, etc. Some include nerve and skin involvement. As with EGM, clinicians vary widely in their use of this term.
TIP: Make sure you are on the same page as your clinician.
Any time you discuss “systemic manifestations”, “extraglandular manifestations”, or “organ system involvement” with your clinician, ask them to explain what they mean.
Even though the ESSDAI is rarely used in routine practice, this list of systemic manifestations has a big influence on Sjogren’s care. Some rheumatologists think that the ESSDAI lists all of the important systemic manifestations. This is not the case. The ESSDAI measures an important subset of systemic manifestations but misses many others.
Learn more about the limitations of the ESSDAI here.
ESSDAI- EULAR Sjogren’s syndrome disease activity index.
The ESSDAI is scoring system that uses 12 specific “domains” of systemic manifestations as a measure of disease activity.
EULAR- European League Against Rheumatism, a professional rheumatology organization.
The 12 ESSDAI domains
Learn more here.
Constitutional: fever, night sweats unexplained weight loss
Lymphadenopathy and lymphoma: Enlarged lymph nodes or lymphoma
Glandular: swelling of the saliva glands or tear glands (this is not sicca!)
Articular: joint pain or inflammation, morning stiffness
Cutaneous (skin): vasculitis, purpura, erythema multiforma
Pulmonary (lung): multiple types of lung disease
Peripheral nervous system (PNS): neurologic complications outside the CNS
Central nervous system(CNS): neurologic complications of the brain and spinal cord
Hematological: “cytopenias” not due to another cause. Low red blood cells, low neutrophils, low lymphocytes, low platelets
Biological: low complement, high or low IgG, cryoglobulinemia
Other Systemic Manifestations- a partial list
Small fiber neuropathy is theoretically part of the ESSDAI, (PNS). However, clinicians and researchers may not be familiar with the diagnosis and testing.
Carpal tunnel syndrome
Vasculitis of organs in addition to the skin and CNS.
Gastrointestinal (GI) manifestations are nearly ubiquitous
Extraglandular eye manifestations such as scleritis, iritis, and uveitis.
Severe headaches, including migraines.
Serositis, including pericarditis, pleuritis, and abdominal serositis (perotinitis).
Interstitial cystitis, overactive bladder and frequent UTI.
Sensorineural hearing loss.
Restless legs syndrome and other sleep disorders.
General Systemic Manifestations
The most common general systemic manifestations include fatigue (pathological, not normal tiredness), cognitive dysfunction (“brain fog”), and widespread muscle pain.
There is no strict definition of general systemic manifestations. Symptoms that are characteristic of Sjogren’s but poorly understood are often assigned to this category or disregarded altogether. Some rheumatologists consider general systemic manifestations to be part of a sicca-limited version of Sjogren’s. This is illogical because these features are obviously not caused by sicca or a part of sicca. The idea of a “glandular” or sicca-limited version of Sjogren’s needs to be put to rest.
A total of 3622 Sjogren's patients participated in the Sjogren's Foundation 2021 Living with Sjogren's survey. d 88% of respondents reported fatigue, 80% had brain fog, and 72% experienced muscle pain (240). These core characteristics of Sjogren’s disease should not be attributed to fibromyalgia, psychiatric issues, or functional disorders.
Not "all in your head".
Increasing evidence supports a biologic basis for fatigue, brain fog, and widespread muscle pain.
These symptoms appear to be driven in part by neurological and immune system dysfunction (88, 204).
Comorbidities may contribute to these complex manifestations but should not be taken to be their primary cause.
General systemic manifestations are a top concern for most patients.
Multiple studies have shown that fatigue and pain, not dryness, are the primary reasons for reduced quality of life in Sjogren's patients (169, 193). These symptoms occur are common in related diseases such as rheumatoid arthritis and systemic lupus erythematosus, but are more debilitating, on average, in Sjogren’s (27, 99).
Research is urgently needed to better understand the underlying causes of these disabling Sjogren's features. This, in turn, could lead to more effective treatments.
The most important thing for clinicians to know about Sjogren’s is that it is a serious, multisystem disease.
This fundamental fact serves as the basis for comprehensive Sjogren’s care.