Promoting optimal care for Sjogren's, a common, serious, systemic disease.
SJOGREN'S BY ORGAN & SYSTEM
Key Concepts
This page provides links to information and resources organized by organ and/or system directly impacted by Sjogren’s, i.e., systemic manifestations, not SICCA.
This page includes some comorbidities, which are conditions that occur in higher rates in people with Sjogren’s but are not caused directly by the disease.
Sjogren's manifestations are caused directly by the disease. Manifestations are disease states that are associated with signs (measurable/observed) and symptoms (what the patient feels). Some manifestations have no obvious symptoms. A few have no obvious signs.
Always check the Resources Page on the Sjogren’s Foundation website for information, specifically the “Brochures and Resource Sheets” and "Information for your Doctor" sections.
Use the GLOSSARY to look up the meaning of terms you may not be familiar with. The glossary explains what terms mean in the context of Sjogren’s.
To learn more about how find information and navigate this website and blog, read HOW TO USE SJOGREN’S ADVOCATE.
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Organs & systems affected by Sjogren's currently covered by this page:
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Lung (Pulmonary)
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ORGANS AND SYSTEMS
(General info, then by alphabetical order)
General: Sjogren's review
What's New in Sjogren's Disease - video presentation by Dr. Vasco Romao (2024)
General: Fatigue, cognitive dysfunction, widespread pain
Fatigue - video presentation my Dr. Wan-Fai Ng (2024)
Digestive System [Gastrointestinal (GI)]
The entire GI tract, from the mouth to the anus, can be impacted by Sjogren’s and its comorbidities. Even though the mouth is part of the GI tract, it is usually discussed separately in Sjogren’s literature under sicca, dental, or oral.
GASTROINTESTINAL MANIFESTATIONS
LIVER DISEASE AND SJOGREN’S
CELIAC DISEASE, GLUTEN, AND YOUR HEALTH (celiac disease is a common comorbidity)
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Primary Biliary Cholangitis (PBC)
A comorbidity that impacts about 1 in 20 people with Sjogren’s.
Heart
People with Sjogren’s are at increased risk of heart disease. Heart problems in people with Sjogren’s are usually comorbidities, but occasionally they can be caused directly by Sjogren’s.
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Immune System
Sjogren’s is caused by a disordered immune system, which has relationships with every other system in the body, especially the neurological (nervous) system.
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Immunology Explained - A website from the American Association of Immunologists
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IMMUNE SYSTEM VIDEOS (brief and easy to understand)
SO WHAT IS SJOGREN’S, REALLY?
Learn what we know and don’t know about the immune system and Sjogren’s.
Kidney (Renal)
One out of ten Sjogren’s patients has clinically overt (obvious) kidney disease. Up to half (50%) have abnormal kidney function when all patients are screened, including those without obvious symptoms (154, 241).
Tubulointerstitial nephritis (TIN) is the most common type of kidney disease diagnosed in Sjogren’s. It is detectable in 10-40% of Sjogren’s patients. There are often no symptoms early on, but TIN may present with renal tubular acidosis (RTA) manifestations (usually “distal RTA”, or dRTA).
Distal Renal Tubular Acidosis (dRTA)
dRTA can cause:
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electrolyte abnormalities,
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kidney stones,
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osteomalacia / bone pain / pathological fractures,
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low potassium may cause profound muscle weakness or even paralysis,
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nephrogenic diabetes insipidus, a condition that causes frequent urination, dehydration, and lightheadedness.
Every Sjogren’s patient should be routinely screened for kidney disease.
Renal involvement is underestimated by research studies because the ESSDAI, a tool used to measure systemic disease activity, “does not include TIN in its subclinical form (when there are only changes in urinary concentration or isolated electrolyte abnormalities). Although less severe, these findings also indicate an active disease with evolutionary potential”. (241)
Please note: There are no established screening guidelines for Sjogren's kidney disease yet. However, I plan to research expert recommendations and will share them in a future post.
Glomerulonephritis
Impacts 2-4% of people with Sjogren’s and requires urgent medical attention (42, 79). There is an association with cryoglobulinemia (241).
Lung (Pulmonary)
Over half of people with Sjogren’s (50-70%) have one or more types of lung disease, including interstitial lung disease pulmonary hypertension, bronchiolitis, bronchiectasis, lymphoma, and others. See WHAT YOU SHOULD KNOW ABOUT SJOGREN'S LUNG DISEASE for links to lung disease resources, including Clinical Practice Guidelines and self-advocacy tips.
PULMONARY HYPERTENSION (PH)
Pulmonary hypertension, usually in the form of pulmonary arterial hypertension (PAH) in Sjogren’s, is one of the most severe types of lung disease. PH/ PAH can be life-threatening. Until recently, this was considered extremely rare.
Neurological: Overview
Introductory information on peripheral and central nervous system manifestations of Sjogren’s by Johns Hopkins Rheumatology.
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Overview - video presentation by Dr. Alessia Alunno (2024) xxxx
How Sjogren's Can Influence Your Brain and Nerves- video by rheumatologist, Dr. Diana Girnita
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The Sjogren’s Foundation Clinical Practice Guidelines, Peripheral Nervous System are expected to be published in 2024. This will be a major resource for clinicians and Sjogren’s patients.
Neurological: Central Nervous System (CNS)
The central nervous system is comprised of the brain and spinal cord. CNS involvement is usually described as impacting about 1 in 20 (5%) of people with Sjogren's.
However, CNS Sjogren's studies do not include cognitive dysfunction, which is reported by more than half of Sjogren's patients in multiple studies. Despite the profound impact on quality of life, researchers are just beginning to study this cognitive dysfunction and describe its impact. I plan to address this and other neglected areas of Sjogren's.
Migraines and other chronic severe headaches are also reported by many people with Sjogren's. These may be direct CNS Sjogren's manifestations, but are not usually recognized as such.
Introduction to CNS Sjogren's by Dr. Brandon Law. He points out the importance and sometimes difficult task for distinguishing Sjogren's from multiple sclerosis.
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Neurological: Peripheral Nervous System (PNS)
Neuropathies are caused by damage or dysfunction of the peripheral nerves. Neuropathies can be found in more than half of people with Sjogren’s. In the 2021 Living with Sjogren’s survey conducted by the Sjogren's Foundation, 61% (about 6 out of 10) of the 3622 patients surveyed reported neuropathy symptoms.
Journal articles often state lower prevalence rates of neuropathy because the most common neuropathies in Sjogren's, small fiber neuropathy (SFN) and dysautonomia (autonomic disorders), are usually not assessed by researchers or clinicians. SFN cannot be detected by routine neurological examination and testing and most researchers remain unfamiliar with dysautonomia. Symptoms of SFN and dysautonomia are often misattributed to fibromyalgia, anxiety, or “functional neurological disorder”.
Other PNS manifestations include large fiber neuropathies, demyelinating disease (e.g., chronic inflammatory demyelinating polyneuritis [CIDP]), cranial neuropathies (e.g., trigeminal neuropathy), mononeuritis multiplex, sensory ganglionopathy, sensorimotor polyneuropathies, and more.
Neuropathies & Quality Of Life
Fatigue, lightheadedness, cognitive dysfunction, and widespread pain
are priority concerns for most people living with Sjogren's.
These vexing quality of life issues are often caused, at least in part, by neuropathy,
but are often misattributed to fibromyalgia and/or psychological disorders.
The following blog posts address this misattribution.
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SJOGREN’S SYMPTOMS CAN LOOK A LOT LIKE FIBROMYALGIA
NEUROPATHY: MAYBE IT’S ALL CONNECTED
Autonomic Disorders (Dysautonomia)
Please see the resource page for DYSAUTONOMIA / POTS.
Thyroid
Autoimmune thyroid disease is a common comorbidity impacting 15-30% of people with Sjogren’s.
Hormones Demystified is an endocrinology blog with many posts about thyroid disease and tests.
Small Fiber Neuropathy (SFN)​
​Small fiber neuropathy and Sjogren's - Excellent, short review article
Small fiber neuropathy testing and information - A patient-friendly library created by a neuropathology company, Therapath.
Small Fiber Neuropathy for the Rheumatologist - For self-advocacy, highlight the following in the Evaluation paragraph,
"In patients with Sjögren’s disease, neurologic symptoms may precede the diagnosis of Sjögren’s. Notably, Sjögren’s disease is associated with several disorders of the peripheral nervous system, and small fiber neuropathy is among the most common. Patients with small fiber neuropathy and Sjögren’s are more commonly seronegative and, thus, lack anti-SSA and anti-SSB autoantibodies.
Sensory neuronopathy (neur-ON-opathy, not “neuropathy” aka dorsal root ganglionopathies) - Ataxia and asymmetric (patchy) non-length-dependent or generalized sensory deficits are clinical hallmarks.
Updated 04-27-2024