Sjogren’s Disease: Autoimmune dry eye Part 3
Part 3
Sjogren’s Disease is associated with other autoimmune diseases in 52% of cases – so once I identify a patient with Sjogren’s, I always refer to a rheumatologist to assist with further diagnosis and treatments. A group of these “concurrent” diseases include:
- Rheumatoid Arthritis (attacks joints and can cause eye problems like “iritis”)
- Scleroderma (a skin disease resulting in thickened, inflamed skin)
- Celiac disease (true gluten allergy, where G.I. diseases including cancer can be triggered)
- Autoimmune Hepatitis (liver disease)
- Neuromyelitis Optica (mimics Multiple Sclerosis, but treatment is different)
- Autoimmune Thyroid diseases
- Primary Biliary Cirrhosis (another liver/GI disease)
- Systemic Lupus Erythematosus (SLE) – a complicated autoimmune disorder that can cause many diseases involving every major body system.
Some of the manifestations directly related to Sjogren’s include:
- Renal Tubular Acidosis (a kidney disease)
- Interstitial Nephritis (another kidney disease)
- Cystic Lung disease
- Cryoglobulinemic Vasculitis (where inflammation in the blood vessels can cause a gamut of body-wide illnesses).
A bit more scary, is the increased risk of Non-Hodgkin’s Lymphoma, a rare cancer of the body’s lymphocytes (part of our natural defense system). The Arthritis Foundation has a good page on this here: https://www.arthritis.org/health-wellness/about-arthritis/related-conditions/other-diseases/non-hodgkins-lymphoma-with-sjogrens-syndrome
From that source comes this:
“Not everyone with Sjögren’s has the same risk. Symptoms and factors that raise the likelihood of developing NHL include:
Enlarged salivary glands, especially if they stay swollen for months or years
Persistently swollen lymph nodes (glands) in the neck, armpit or groin
Pinpoint or button-sized areas of bleeding in the skin (usually on the lower legs) called petechiae or purpura
Nerve damage known as peripheral neuropathy
Poor blood flow to the fingers, toes, ears or knees, called Raynaud’s phenomenon
Abnormal proteins called cryoglobulins and/or gamma globulins in the blood
The number of factors you have is key in determining your risk. In people with fewer than two of these factors, the risk for NHL is less than 4%. In those with three to six of these factors, it’s nearly 40%.”
This is another reason I like to refer to a rheumatologist, as they are keyed into these (potentially many,) Sjogren’s-related issues and are in a better position to diagnose and properly refer or treat them, if they are found present.
A clip from an American College of Rheumatology meeting abstract has a good description of how rheumatologists (and other medical professionals dealing with Sjogren’s Disease) will look at quantifying signs and symptoms of the disease. The abstract I’m referring to can be found here:
When investigating how much a disease is affecting an individual, it is helpful to have a systematic way of quantifying disease activity – in part to know if that patient is progressing in a way that is better or worse, but also to help validate studies to know if a particular treatment is truly helping populations of patient with the disease. One of the greatest challenges of such studies about Sjogren’s patients, is that they are a diverse group – some of whom have multiple (other) autoimmune diseases, that there are many ways the “same disease” can affect people - and all of whom can have variable degrees of the disease (from a little to a lot) that can vary over time.
We can stratify Sjogren’s to help determine who is best eligible for disease-modifying therapies – as most therapies will target specific portions of Sjogren’s signs and symptoms – and any of them can also have significant side-effects, so patients with less illness will usually want less potentially toxic treatments. The primary disease symptoms are classified as:
- Dryness
- Fatigue
- Pain
And by a Low vs High Symptom “burden” – i.e.:
- Dry with low pain
- Dry with high pain
Disease activity can be categorized:
- Dryness (sicca) alone – without body-wide disease activity or enlargement of the saliva or tearing glands (18%)
- Predominantly dry, but with mild to moderate general body-wide disease activity (especially joint pain, blood disorders and/or gland enlargement = 74%)
- High body-wide disease activity with or without dryness (8%)
On top of this, can be multiple forms of disease affecting peripheral nerves. This is because Sjogren’s can also primarily attack these smaller nerves and affect our sense of balance, our ability to sense or feel things – or can result in a normal ability to “sense” even while depleting the tiny nerve fibers in skin and in the surface of the eye (leading to surface problems and can wreak havoc on those already suffering with dry eyes).
Next week, I’ll focus on more specific issues associated commonly with Sjogren’s and some of the advances in treatments that are underway…