Pitfalls in diagnosing SS

Q: All of the following conditions EXCEPT ONE may bar patients from diagnosis of Sjögren's syndrome (SS) and participation in SS studies or therapeutic trials because of overlapping clinical features or interference with criteria tests? - select one

A) Positive Anti-Ro/SSA

B) History of head and neck radiation 

C) Active hepatitis C infection 

D) Sarcoidosis

E) Amyloidosis

Answer: A

Positive Anti-Ro/SSA actually is a part of diagnosis of SS. The five major components to diagnose SS are:

• Labial salivary gland with focal lymphocytic sialadenitis and focus score of ≥1 foci/4 mm2
• Anti-Ro/SSA positive
• Ocular staining score ≥5 (or van Bijsterveld score ≥4) in at least one eye
• Schirmer test ≤5 mm/5 minutes in at least one eye
• Unstimulated whole saliva flow rate ≤0.1 mL/minute

Prior diagnosis of any of the following conditions would exclude diagnosis of SS and participation in SS studies or therapeutic trials because of overlapping clinical features or interference with criteria tests:

• History of head and neck radiation treatment
• Active hepatitis C infection (with positive PCR)
• Acquired immunodeficiency syndrome
• Sarcoidosis
• Amyloidosis
• Graft-versus-host disease
• IgG4-related disease

#rheumatology

References:

1. Daniels TE, Cox D, Shiboski CH, et al. Associations between salivary gland histopathologic diagnoses and phenotypic features of Sjögren's syndrome among 1,726 registry participants. Arthritis Rheum 2011; 63:2021.

2. Whitcher JP, Shiboski CH, Shiboski SC, et al. A simplified quantitative method for assessing keratoconjunctivitis sicca from the Sjögren's Syndrome International Registry. Am J Ophthalmol 2010; 149:405.

3. Van Bijsterveld OP. Diagnostic tests in the Sicca syndrome. Arch Ophthalmol 1969; 82:10.

4. Navazesh M, Kumar SK, University of Southern California School of Dentistry. Measuring salivary flow: Challenges and opportunities. J Am Dent Assoc 2008; 139 Suppl:35S.

5. Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: A consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 2017; 76(1):9-16.