PRIMARY CARE PROVIDERS:

STEPS TO DIAGNOSIS

This Steps to Diagnosis 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 Sjogen'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 SJOGRENS TRIAD" 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. Ranges from mild to severe. 

2. Widespread muscle and/or joint pain. Anyone with a diagnosis of fibromyalgia.  

 

Even if a patient has a diagnosis of thyroid disease or fibromyalgia, these conditions do not rule out Sjogren’s. Take a second look! Most fibromyalgia symptoms in Sjogren's patients are direct Sjogren's features. 

 

3. Sicca- Dry, irritated eyes and/or mouth.  Patients often do not recognize or mention sicca symptoms.  See Step 2 for Sicca Screening tool.  

The Triad

Red flagsSjogren’s should be high in the differential     

  • Parotitis/sialadenitis- persistent or recurrent

  • MALT lymphoma, especially of major salivary glands

  • Interstitial lung disease

  • Interstitial kidney disease 

  • Distal Rental tubular acidosis; hypokalemia

  • Primary biliary cholangitis

  • Autoimmune hepatitis

  • Leukopenia, thrombocytopenia, normocytic anemia

  • Hypergammaglobulinemia

  • Cutaneous vasculitis or annular erythema/cutaneous lupus

  • NMO, or MS-type symptoms without typical MRI or CSF findings

  • Esophageal dysmotility/difficulty swallowing

  • POTS and other CV dysautonomias may present with dizziness, tachycardia. 

red flag.jpg

Common presenting primary complaints 

  • Severe fatigue, often associated with widespread pain

  • “Brain fog”-difficulty concentrating, functioning at work

  • Chronic cough, often dry 

  • Recurrent/ chronic sinusitis

  • GERD, GI pain, chronic nausea, “irritable bowel,” constipation, SIBO

  • Recurrent oral/ vaginal candidiasis

  • Vaginal dryness/ painful intercourse

  • Interstitial cystitis

  • Constitutional symptoms: fever, enlarged lymph nodes, unintentional weight loss

  • Dry, irritated eyes, blurred vision

  • Dry mouth, severe or unexplained dental caries

  • Neuropathies:  Painful sensory neuropathies, and others

  • Dysautonomias such as POTS

  • Raynaud’s phenomenon

  • "Fibromyalgia"

It is essential to keep a high index of suspicion in adult women who have multiple seemingly unrelated complaints. Sjogren's often has an incomplete presentation, especially early in the disease.

STEP 2 : SCREEN FOR SICCA 

Always screen for sicca in the setting of ongoing unexplained fatigue, widespread pain, red flags, or common presenting complaints

Patients may not recognize sicca symptoms as abnormal or important. They often fail to mention sicca symptoms to PCPs. Sicca is more complex than just dryness. If patients answer NO to “do you have dry eyes or dry mouth?” ask the following quick questions:

                     EYE

Gritty, "sand in eyes" sensation?

Foreign body sensation?

Pain, burning

Blurring, blinking to correct vision?

Restasis, Xiidra or artificial tears > tid?

                     ORAL

Trouble speaking?

Need liquids to swallow food?                                                                   

Food or pills get stuck?     

     

Severe, unexplained caries?                          

80% of Sjogren’s patients have sicca at presentation. This means that 20% do not. (3)

Conversely, not all sicca is due to Sjogren’s. Other causes include medications, radiation treatment, diabetes, HCV, HIV, sarcoidosis, amyloidosis, IgG-4 syndrome and others. Fibromyalgia is often associated with a dry eye sensation, or may be the diagnosis given to patients with incomplete Sjogren's presentations. Rheumatology expertise is helpful to sort through the possibilities. 

STEP 3 : PHYSICAL EXAMINATION 

  • Skin - overt signs of dryness. Cutaneous vasculitis or annular erythema/ subcutaneous lupus.  Raynaud’s phenomenon

  • Head and neck - Palpate for adenopathy, parotid swelling/masses

  • Oral - saliva under tongue, dental decay, bright red tongue often seen with candida (vs. cottage cheese appearance). 

  • 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 may indicate interstitial lung disease.

  • GI - diffuse, mild tenderness common. Palpate RUQ and epigastric area for focal tenderness (less common)

  • GU - tenderness w/ bladder palpation

  • Neuro - Note gait, sensory changes hands and feet. If tachycardia, or reports of syncope or pre-syncope, arrange follow-up evaluation for CV dysautonomias.

  • Ortho -  joints, esp. hands and feet, for tenderness and swelling.   

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)

Order Initial Sjogren’s labs if Sjogren’s triad or red flags. Also consider when common presenting complaints are: 

  • Scattered, multisystem

  • Persistent or recurring

  • Associated with fatigue, pain and/or sicca
     

 A positive family history of any autoimmune disease should tip the scale toward evaluation.

Initial Sjogren's Labs

  •  ANA, SSA, SSB, CCP, RF, ESR, CRP    (titers not ~ disease severity)    

  •  CBC with diff and platelets,  serum potassium, LFTs

  •  UA, Random urine protein/Cr

  •  HIV, HEP C       

  •  Minor salivary gland biopsy (MSGB)- if SSA  negative- Usually via rheumatology consult.

STEP 5: Referral 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 as well as look for other rheumatologic diseases that often co-exist with Sjogren's. Most rheumatologists prefer completion of preliminary lab work prior to referral. They typically often order additional tests for further evaluation. 

 

The role of Classification Criteria vs. clinical diagnosis   

Unfortunately, a significant minority of rheumatologists remain out of touch with present-day Sjogren’s diagnosis and treatment. Some are unaware of the limitations of the Classification Criteria and use them as a stringent requirement for diagnosis. As a referring provider, it is important that you understand that Classification Criteria are NOT diagnostic criteria. Sjogren's is a clinical diagnosis that is supported by, but cannot be rule out by, the tests used for classification.

Sjogren’s patients may be quite ill. Sjogren's is common, serious, and always systemic. Many patients develop serious complications. They deserve timely diagnosis and specialty care.  

I often I 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.  If a patient runs into these roadblocks, they should seek a second opinion from a Rheumatologist who is more experienced with Sjogren’s.

Updated 3-10-21