What is good Sjogren’s care?
Does your rheumatologist
Listen to your lungs during routine clinic visits?
Order tests for blood cell counts, liver function, and kidney function?
Assess you for lymphoma risk?
Screen for celiac disease and autoimmune thyroid disease?
Recommend pneumonia and flu vaccines?
Ask you about fatigue, function, and coping?
In addition to managing oral and eye features, good Sjogren’s care includes monitoring for a large number of systemic complications and comorbidities. Your rheumatologist may already be doing this. Or perhaps not. Many patients do not know what their blood and urine tests mean. If you are not sure, ask your rheumatologist.
Practical details about ongoing care will be discussed later. This is a complicated topic. Monitoring will vary based on each patient’s unique situation and the rheumatologist’s style of practice. I chose the six examples above because they are important to address with every Sjogren’s patient, both seropositive (SS-A positive) and seronegative.
Systemic features include:
In Sjogren's, "systemic" means disease manifestations that are not directly caused by sicca. This is an overview, not a complete list. See the Always Systemic page for more detail. Future sections will discuss these features in more depth.
Organ involvement. Sjogren's can impact the lungs, kidneys, liver, skin, gut, and so on. EVERY organ in the body can be impacted. Sometimes this is subclinical, meaning there are no obvious symptoms but damage can be found if you look closely.
Nervous system involvement, including the central nervous system (CNS) and peripheral nervous system (PNS). PNS manifestations are extremely common and underdiagnosed. CNS manifestations are probably far more common than the current 5-15 % estimates.
Hematological abnormalities. The "cytopenias" such as low platelets, low white blood cells, etc.
Biological abnormalities such as cryoglobulins, low complement, and high or low IgG levels.
Lymph system changes. Sjogren's may cause enlarged lymph tissue in organs and lymph nodes. An overactive immune system leads to non-Hodgkins B cell lymphoma in 5-15 %.
General systemic features such as fatigue, brain fog and widespread pain are the most difficult part of the disease for most patients. Many rheumatologists do not categorize these as systemic, despite the fact that they impact the whole body and are obviously not caused by sicca. Because they are poorly understood, they often remain unaddressed.
A note about comorbidities:
Comorbidities are conditions that are associated with Sjogren's, but not thought to be direct manifestations. Autoimmune thyroid disease and celiac disease are common comorbidities. So are osteoporosis and cardiovascular disease. The list is long!
Why it is so important to monitor for systemic features and comorbidities
Patients who develop lung disease, chronic kidney disease, and other serious complications may go for years thinking that they are fine because no one ever bothered to check for these things. Some systemic manifestations can have mild symptoms or no symptoms at all until the problem is far advanced. All the while, ongoing damage is occurring. There are numerous Sjogren’s complications and comorbidities that should be identified, monitored and treated (as appropriate) as early as possible.
The disconnect: Many rheumatologists fail to address important systemic features.
Some patients are fortunate to find a rheumatologist who is engaged and up to date with Sjogren’s. The level of knowledge and practice varies widely. While I have a lot of sympathy for overworked rheumatologists, it is their job to take the lead in caring for Sjogren’s patients. This situation would be unimaginable with almost any other common, serious, well–characterized disease. There are structural reasons for this disconnect, and probably a component of resistance to change.
How can this happen? One Sjogren’s expert put it this way: “They put Sjogren’s in the too-hard basket.” Sjogren’s patients are incredibly complex. Doing a good job takes a lot of time, creativity, and trial and error. It is frustrating to patients and rheumatologists alike that response to treatments can be unpredictable and that benefits tend to be modest at best. There are no easy algorithms that make treatment decisions simple.
Because there are few continuing education programs about Sjogren’s, clinicians who stay up to date usually do so on their own initiative. It is much easier to go along with the outdated view of Sjogren’s as mostly a nuisance dryness disease than it is to face the complicated reality that Sjogren’s is Always Systemic.
Unless a patient is lucky enough to find a Sjogren’s-savvy rheumatologist, they need to learn a lot about the disease in order to advocate for comprehensive care. Patients, most of whom are burdened by disabling physical fatigue and brain fog, should not need to learn detailed medical knowledge to get the care they need. Many simply do not have the background, time, or resources to do this. This series of blog posts will hopefully make this a bit more manageable for patients.
NEXT UP: Knowing how your rheumatologist approaches Sjogren’s is key to communicating about the care you need. The next blog post will discuss how to determine your rheumatologist’s view of Sjogren’s.