PRIMARY CARE PROVIDERS:
STEPS TO DIAGNOSIS
This page arose out of Dr. Sarah Schafer's teaching experience with primary care providers (PCPs). Many PCPs have expressed the need for practical steps for use in the clinic setting. The teaching curriculum was created with the support of several rheumatologists with Sjogren's expertise. This page is not an "official" guideline approved by the Sjogren's Foundation, any medical board, or rheumatology organization.
Clinicians should use their best judgement, taking into account each patient’s unique needs as well as the medical system that they work in. In an ideal world, this content would be taught routinely during medical education.
STEP 1: Recognize Sjogren's
Scattered and sometimes vague symptoms such as the Sjogren's Triad of fatigue, pain, and dryness, are often the earliest symptoms. There are many exceptions, as noted below.
Have a high index of suspicion when adults, particularly women, present with greater than 3 months of:
1. Flu-like fatigue that ranges from mild to severe.
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2. Widespread muscle and/or joint pain.
Sjogren's is often misdiagnosed as fibromyalgia. Clinicians should look for Sjogren's in anyone labeled with fibromyalgia. Learn more here.
3. Sicca, especially dry, irritated eyes and/or mouth. Patients often do not recognize or mention sicca symptoms. Clinicians must know to ask about sicca symptoms, although 20% with Sjogren's do not present with obvious or measurable sicca.
See Step 2 for sicca screening questions.
A high index suspicion for Sjogren's should occur in people with the above symptoms (the "Sjogren's Triad"), people with Red Flags, and people with the following autoimmune diseases:
~ other autoimmune rheumatic diseases such as rheumatoid arthritis, systemic lupus erythematosus, and systemic sclerosis. Sjogren's should never be called "secondary Sjogren's".
~ autoimmune thyroid disease
~ celiac disease
People with these conditions should be carefully monitored for Sjogren's and vice versa.
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The Sjogren's Triad
Red Flags – Sjogren’s should be high in the differential
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Persistent or recurrent parotitis/sialadenitis
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MALT lymphoma, especially of major salivary glands
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Interstitial lung disease
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Interstitial kidney disease
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Distal Renal tubular acidosis; hypokalemia
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Primary biliary cholangitis
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Autoimmune hepatitis
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Leukopenia, thrombocytopenia, normocytic anemia
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Hypergammaglobulinemia
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Cutaneous vasculitis or annular erythema/cutaneous lupus
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NMO or MS-type symptoms without typical MRI or CSF findings
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Esophageal dysmotility/difficulty swallowing
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POTS and other CV dysautonomias may present with dizziness, tachycardia.
Common presentations
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Severe fatigue, often associated with widespread pain
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Sleep disorders, lack of refreshing sleep
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“Cognitive dysfunction ("Brain fog)”-difficulty concentrating, functioning at work
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Chronic cough, often dry
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Recurrent/ chronic sinusitis
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GERD, GI pain, chronic nausea, “irritable bowel,” constipation, SIBO
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Recurrent oral/ vaginal candidiasis
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Vaginal dryness/ painful intercourse
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Interstitial cystitis/bladder pain syndrome
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Constitutional symptoms: fever, enlarged lymph nodes, unintentional weight loss
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Dry, irritated eyes, blurred vision
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Dry mouth, severe or unexplained dental caries
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Neuropathies: Painful sensory neuropathies, and others
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Autonomic disorders (Dysautonomia) such as POTS (often undiagnosed)
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Raynaud’s phenomenon
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"Fibromyalgia"
It is essential to keep a high index of suspicion for Sjogren's in adult women who report multiple seemingly unrelated symptoms.
Sjogren's often has an incomplete presentation, especially early in the disease. Symptoms often start decades before Sjogren's is diagnosed.
STEP 2 : Screen For Sicca
Always screen for sicca in the setting of ongoing unexplained fatigue, widespread pain, red flags, or common presenting complaints.
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Patients may not recognize sicca symptoms as abnormal or important. They often fail to mention sicca symptoms to PCPs. SICCA is more than just dryness. If patients answer no when you ask if they have dry eyes or a dry mouth, follow-up with these questions.
Screening Questions For Ocular Sicca
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Do you have a gritty, "sand in eyes" sensation?
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Do you have a foreign body sensation?
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Do you experience pain and/or burning in your eyes?
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Do you find your vision blurring and/or do you need to blink to correct your vision?
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Do you use Restasis, Xiidra, or artificial tears more than 3 times a day?
Screening Questions For Oral Sicca
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Do you have trouble speaking?
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Do you need liquids to swallow food?
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Do food or pills get stuck in your throat?
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Do you have severe, unexplained caries?
Eighty percent of Sjogren’s patients have sicca at presentation. This means that 20% do not (3).
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Conversely, not all sicca is due to Sjogren’s. Other causes include medications, radiation treatment, diabetes, HCV, HIV, sarcoidosis, amyloidosis, IgG-4 disease and others. Rheumatology expertise is helpful to sort through the possibilities.
STEP 3: Physical Examination
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Skin - overt signs of dryness. Cutaneous vasculitis or annular erythema/subcutaneous lupus. Raynaud’s phenomenon
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Head and neck - Palpate for adenopathy, parotid swelling/masses
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Oral - saliva under tongue, dental decay, bright red tongue often seen with candida (vs. cottage cheese appearance).
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Eyes - most changes are seen using special dyes and slit lamp exam.
Blepharitis/ conjunctival erythema may be noted on gross exam. -
Heart and lungs - crackles at the lung base may indicate interstitial lung disease.
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GI - diffuse, mild tenderness common. Palpate RUQ and epigastric area for focal tenderness (less common)
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GU - tenderness w/ bladder palpation, vaginal dryness and/or pain
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Neuro - Note gait, sensory changes hands and feet. If tachycardia, or reports of syncope or pre-syncope, arrange follow-up evaluation for CV dysautonomias.
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Ortho - joints, especially hands and feet, for tenderness and swelling.
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Note: Sjogren’s patients tend to look well, even when they feel quite ill. Routine physical examination may be completely normal.
STEP 4: Initial Labs (suggested)
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Labs may vary based on the patient and the institution.
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Order initial Sjogren's labs if the Sjogren's triad or red flags are present.
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Sjogren's should always be considered in patients with multiple, seemingly unrelated symptoms, especially if they have any features in the Sjogren's.
See All in Your Head? to learn more why assuming symptoms are psychological/functional without a thorough evaluation is a great disservice to patients. ​
A positive family history of any autoimmune disease should tip the scale toward evaluation.
Initial Labs That Support A Diagnosis Of Sjogren's
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ANA, SSA, SSB, CCP, RF, ESR, CRP (titers not ~ disease severity or activity)
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CBC with diff (differential) and platelets, serum potassium, LFTs
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UA, Random urine protein/Cr
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HIV, HEP C
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If SSA-negative, minor salivary gland biopsy (MSGB), usually ordered via rheumatology consult.
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STEP 5: Refer To A Rheumatologist
Even if your patient has a textbook Sjogren’s presentation and a positive SSA, a rheumatologist with Sjogren’s expertise should confirm the diagnosis and look for other rheumatologic diseases. Most rheumatologists prefer completion of preliminary lab work prior to referral. They typically often order additional tests for further evaluation.
Sjogren's is important to diagnose. It is a COMMON, SERIOUS, SYSTEMIC disease, never "just sicca". Many patients develop serious complications. People with Sjogren's deserve timely diagnosis and multidisciplinary care
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Unfortunately, somerheumatologists remain out of touch with present-day Sjogren’s diagnosis and treatment. Some misuse the Classification Criteria (a research tool) as diagnostic criteria. There are no diagnostic criteria. Sjogren's is a big picture diagnosis.
Learn more:
Why is it so hard to get diagnosed?
12 Reasons People with Sjogren's Don't get Diagnosed
SSA and Sjogren's
A note from Dr. Schafer...
I often hear stories from other patients who are (incorrectly) told that they can’t have the disease without a positive test for Sjogren’s antibodies.
Even with a Sjogren’s diagnosis, some patients are told that they don’t need special care, or that the disease is not serious. The Myths About Sjogren's page can help you counter misinformation, which remains widespread.
If you run into these roadblocks, try to find a from a rheumatologist who is more experienced with Sjogren’s.
See the Myths About Diagnosis page to advocate for a proper evaluation.
Updated 04-09-2024