Signs, symptoms, and manifestations
Sicca (dryness) vs. systemic manifestations
In Sjogren’s disease, a malfunctioning immune system causes damage and dysfunction in many parts of the body. When this impacts the exocrine glands, it creates sicca (dryness) manifestations. Most Sjogren’s manifestations are systemic (non-sicca). Despite this, sicca usually gets the most attention.
Rheumatologists routinely underestimate the systemic manifestations of Sjogren’s. Understanding the difference between manifestations, signs, and symptoms will help you talk with your doctors about Sjogren’s care that goes beyond sicca.
Symptoms are not the same thing as manifestations
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. Some manifestations have no obvious signs. Features can refer to manifestations, signs, or symptoms.
Some systemic manifestations have characteristic signs and symptoms. Example: Raynaud’s phenomenon
Raynaud's Symptoms The patient feels cold/ burning fingers or toes, associated with temporary skin color changes after exposure to cold or stress. Raynaud’s Signs Examination- The clinician observes the color changes in the skin in the clinic. Photo documentation- the patient photographs the color changes to show their doctor. Tests- the rheumatologist observes blood vessel changes using a hand-held microscope.
Some manifestations have characteristic signs and symptoms but lack of clinician awareness may delay diagnosis. Example: Interstitial Lung Disease (ILD) ILD is more common than most rheumatologists realize. It may not get diagnosed in its early stages when it is most treatable.
ILD Symptoms Symptoms may include chronic cough, shortness of breath, exercise intolerance, and chest discomfort. Some patients have no obvious symptoms. Each of these symptoms may have other causes, including other types of lung disease.
ILD Signs Examination- Crackling sounds may heard at the base of the lungs with a stethoscope. Tests- High resolution CT scan (HRCT) and pulmonary function tests (PFTs) are used for diagnosis. “Silent” lung disease (no obvious symptoms) can be detected with these tests.
Some manifestations have no obvious symptoms. This is why it is important to monitor for systemic manifestations, regardless of symptoms. Examples: Low white blood cell count
“Silent” lung or kidney disease
Some manifestations have no obvious signs.
This may happen because
1. the clinician did not examine the part of the body impacted (e.g., listen to the lungs with a stethoscope, inspect the skin and joints). 2. the clinician did not order routine screening tests (e.g., kidney function, CBC). 3. specialized testing is not readily available (e.g., gastrointestinal motility studies, musculoskeletal ultrasound). 4. tests are available but the clinician is not familiar with the condition (e.g., POTS, cognitive dysfunction). 5. medical science has not yet found biomarkers that are specific to a condition, even though it is experienced by a majority of Sjogren’s patients (e.g., fatigue, widespread muscle pain).
Why doesn’t Sjogren’s Advocate have a page that lists symptoms?
Several patients have requested a complete list of Sjogren’s symptoms and what causes them. I use the example of nausea, a common symptom, to show why this is not practical.
13 causes of nausea in Sjogren’s (a partial list) Sjogren’s manifestations - caused directly by the disease Autonomic disorders (e.g., gastroparesis) Migraines (CNS manifestation; some would consider this a comorbidity) Dysbiosis/ small intestine bacterial overgrowth Gastritis Gastroesophageal reflux disease (GERD) Pancreatitis (acute pancreatitis is rare in Sjogren’s; if present, rule out IgG4 related disease) Peritonitis (rare in Sjogren’s; if present rule out systemic lupus erythematosus).
Unrelated to Sjogren’s Pregnancy Food intolerances and food allergies (possibly comorbidities) Gallbladder disease
Each patient has their own unique set of symptoms. Symptoms often have multiple causes that can change over time. It is important to work with your own clinician for individualized evaluation and management.