HOME > EDUCATION > TRAINING > Sjogren's Syndrome (SS)

Sjogren's Syndrome (SS)

Contributor: Amy Cannella, MD, FACP

+ CASE 1

A previously healthy 32 year-old woman presents with complaints of six months of worsening dry eyes and dry mouth. She feels a gritty sensation in her eyes, and has been unable to wear her contact lenses. She states her dentist is concerned because she has developed multiple dental caries. She also feels some fullness in her cheeks. She has been very fatigued and achy. Her physical exam reveals dry mucous membranes, dental caries at the gum line and swelling in her parotid glands. A Shirmer’s test is done and results in 3 mm of bilateral tear wetting in 5 minutes. The remainder of the examination is normal. Laboratory testing is done and the ANA is 1:640 (by immunofluorescence), and the anti-Ro (SSA) and anti-La (SSB) antibodies are strongly positive. A diagnosis of primary Sjogren’s syndrome (SS) is made.

What are the key features that lead to a diagnosis of SS?

  • Patients with primary SS commonly present with sicca symptoms (dryness), both ocular and oral. Persistent dry eyes and/or dry mouth (daily for over three months), without an alternative explanation are very important clues in this diagnosis. Patients describe a sandy or gravel sensation in their eyes. In addition to dryness (xerostomia), common oral complaints include salivary gland swelling, an increase in dental caries at the gum-line, and the need to use liquid to aid in swallowing.
  • Ocular signs, such as a positive Schirmer’s test can help give objective evidence for ocular dryness. A Schirmer’s test can be done in the office and consists of gently placing sterile filter paper between the eye and the lower lid and measuring tear production. A positive test results in < 5 mm of wetting in 5 minutes. Other tests for ocular dryness include a Rose Bengal stain to evaluate for devitalized corneal tissue, and a fluorescein test to measure tear break up time.
  • Tests can also be done for xerostomia. These include salivary gland scintigraphy, which gives a dynamic measure of salivary gland function by looking at radiotracer uptake. In addition, whole gland sialometry and the Saxon test measure the rate of saliva production.
  • The presence of antibodies to ANA, Ro (SS-A) and La (SS-B) are commonly found in patients with primary SS. Multiple studies have reported differing frequencies, but in general the presence of one or more of these antibodies are seen in over half of affected patients.
  • A minor salivary gland biopsy is done with a punch biopsy of the lower lip to support a diagnosis of SS. A scoring system is employed by the pathologist, looking for focal collections of lymphocytes.

+ CASE 2

A 56 year old woman with a past medical history of hypertension presents with palpable purpura on her lower legs, and a painful sensation in her right foot with the inability to dorsiflex the foot. She also describes a painful triphasic color change in her fingers upon cold exposure, consistent with Raynaud’s phenomenon. With further questioning, she states she has had years of dry eyes and dry mouth. On physical examination, she is edentulous, has dry mucous membranes, has palpable purpura on her lower legs and has a right foot drop. Her Shirmer’s test results in 1 mm of bilateral tear wetting in 5 minutes. Her ANA, Rheumatoid factor, anti-Ro (SSA) and anti-La (SSB) are strongly positive. Other serologies are negative. She has positive cryoglobulins and a monoclonal protein on serum protein electrophoresis. A sural nerve biopsy shows vasculitis. A diagnosis of Sjogren’s syndrome with vasculitis is made.

What additional extra-glandular manifestation of Sjogren’s Syndrome is concerning in this case?

This patient has likely had long-standing SS, which places her at risk for the development of Non-Hodgkins Lymphoma (NHL). The lifetime risk for developing NHL in SS is 5%, which is markedly higher than that of the general population. Risk factors for the development of NHL include the length of time with the disease, positive anti-Ro (SSA) and La (SSB) antibodies, positive rheumatoid factor (RF), cutaneous vasculitis, type II cryoglobulins, and peripheral neuropathy. Patients with SS will have a monoclonal gammopathy up to 22% of the time, and it may be a harbinger of NHL. In this case, the monoclonal gammopathy, vasculitis, serologic positivity and cryoglobulinemia warrant a referral to hematology for evaluation for lymphoma.

Patient Care

  1. Identify common patient complaints suggestive of the diagnosis of SS, including both oral (xerostomia) and ocular (xerophthlalmia) symptoms and glandular swelling.
  2. Recognize that patients with xerostomia may have problems with chewing and swallowing food, dental caries (at the gum line) and oral candidiasis.
  3. Consider the differential diagnosis for sicca symptoms and evaluate the patient for diseases which mimic SS, such as HIV, HCV, sarcoidosis and lymphoma.
  4. Recognize the association of SS with other autoimmune conditions, including RA, scleroderma and SLE.
  5. Identify the visceral manifestations of SS and the significant morbidity for patients with end-organ involvement
  6. Identify comorbid conditions that can worsen sicca symptoms in SS, such as smoking, medications and vitamin deficiency.
  7. Recognized the different treatment approaches for glandular and extraglandular manifestations of Sjogren’s syndrome.

Medical Knowledge

  1. Identify the epidemiology of SS, including primary and secondary forms.
  2. Recognize the pathogenic mechanisms, including genetic and environmental factors that may lead to the development of Sjogren’s syndrome.
  3. Distinguish the lacrimal and salivary glandular pathology of focal lymphocytic infiltration involved with oral and ocular symptoms of the disease.
  4. Recognize that the classification criteria for SS have been recently revised.
  5. Identify and understand extra-glandular manifestations of the disease, including arthritis, pulmonary disease, distal renal tubular acidosis, thyroiditis, hepatitis, neuropathy and cutaneous vasculitis.
  6. Identify laboratory test abnormalities, including nonspecific ANA and RF and more specific auto-antibodies (anti-Ro and anti-La).
  7. Describe the commonly used tests for diagnosis of SS, including a minor salivary gland biopsy, Shirmer’s test , Slit Lamp examination, Rose Bengal staining, and sialography.
  8. Recognize the risk of developing lymphoma in SS, and the associated harbingers of malignancy (reduction of RF titer, glandular swelling).

Interpersonal and Communication Skills

  1. Discuss the disease and potential complications with patients in a way that incorporates patients’ perspectives.
  2. Utilize web based resources to help educate patients about SS.
  3. Outline a non-pharmacologic treatment regimen for different facets of SS.
  4. Outline a targeted pharmacologic treatment regimen and understand the side effects of therapy.
  5. Recognize the risk of fetal heart block in women with anti-Ro/La antibodies and understand how to discuss this with patients.

Professionalism

  1. Recognize the effects of a chronic disease on the patient, the family and quality of life
  2. Recognize the importance of patient privacy, informed consent and equal care
  3. Provide adequate time and accessibility to address patient concerns
  4. Demonstrate integrity and honesty in discussing patient care issues and management with the patient and family

Practice Based Learning

  1. Utilize the American-European Consensus Group Classification Criteria for Sjogren’s Syndrome to aid in the diagnosis.
  2. Integrate and apply information from the history and physical, laboratory and diagnostic testing to make informed decisions about patient care
  3. Set learning goals in SS diagnosis and management
  4. Utilize web-based resources for the most current information on diagnosis and treatment of SS.
  5. Develop a willingness to learn from errors and use errors in a constructive way to learn and improve the system for patient care.
  6. Systems Based Practice
  7. Recognize that the diagnosis can be challenging and referral to a rheumatologist is helpful.
  8. Recognize the need for interdisciplinary management, including close follow-up with ophthalmology and dental providers.
  9. Identify barriers to the delivery of optimal patient care for patients with SS and offer improved ideas for delivering care.
  10. Identify patient barriers to the treatment plan and be sensitive to both cultural and financial obstacles in delivering patient care.
  11. Demonstrate an awareness of the impact of diagnostic and pharmacologic recommendations on the health care system, including insurance companies, physician and patient.

Keywords: Sjogren's Syndrome, Sicca Syndrome, Xerostomia, Xerophthalmia, Inflammatory Arthritis, Anti-Ro (SSA) Antibody, Anti-La (SSB) Antibody

Key References