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Is there a connection between Sjögren’s syndrome and hearing loss?

FA Manian MD, MPH, , , , , , , , Leave a comment

While Sjögren’s syndrome (SS) is well known to cause dry eyes, mouth, skin and nose (“sicca”), its association with hearing loss is often underrecognized by patients and clinicians.  In fact, hearing loss has been reported in as many as one-third or more of patients with SS.1-4

A 2023 systematic review and meta-analysis of 11 observational studies found a 52% pooled prevalence of any type of hearing loss in SS patients (mean age 52 years). Even after excluding studies with moderate (vs low) publication bias, the prevalence remained high (37%).1  A subgroup analysis based on sensorineural, conductive and mixed types of hearing loss (irrespective of severity) found a prevalence of 43%, 5% and 2%, respectively, among SS patients.

A 2020 cross-sectional study involving 30 patients with SS and polyneuropathy undergoing auditory function testing (by pure tone audiometry, Freiburg speech comprehension audiometry, transient evoked otoacoustic emissions and brainstem evoked response audiometry) found hearing loss by pure tone audiometry in 33% of patients with severity ranging from mild (in 60%) to severe (in 10%).2 Overall 80% of patients showed pathological test results on audiometric testing. Interestingly, in those with hearing loss on pure tone audiometry, 80% had asymmetric results.

A potential mechanism for sensorineural hearing loss in SS is the involvement of central nervous system. Of note, other autoimmune diseases such as rheumatoid arthritis and systemic lupus erythematosus have also been associated with hearing loss, possibly related to the vasculitis of vasa nervorum or vasa vasorum.1 Indeed, immunosuppressants such as corticosteroids, cyclophosphamide, azathioprine and mycophenolate mofetil have been used in the treatment of sensorineural hearing loss associated with autoimmune disorders. Dryness of Eustachian tube has been suggested as risk factor for the development of conductive hearing loss.1

Bonus Pearl: Did you know that SS is the second most common autoimmune rheumatic disease affecting an estimated 4 million cases in the USA alone?3

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References

  1. V, PartalidouS, Siolos P, et al. Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis. Rheumatology 2023;43:233-44. Prevalence of hearing loss in patients with Sjögren syndrome: a systematic review and meta-analysis – PubMed (nih.gov)
  2. Seeliger T, Bonig L, Witte T, et al. Hearing dysfunction in patients with Neuro-sjögren: a cross-sectional study. Ann Transl Med 2020;8:1069. Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study – PubMed (nih.gov)
  3. Yang TH, Xirasagar S, Cheng YF, et al. Increased prevalence of hearing loss, tinnitus and sudden deafness among patients with Sjögren’s syndrome. RMD Open 2024;10:e003308. Increased prevalence of hearing loss, tinnitus and sudden deafness among patients with Sjögren’s syndrome – PubMed (nih.gov)
  4. Kim KS, Kim HS. Successful treatment of sensorineural hearing loss in Sjögren’s syndrome with corticosteroid. Korean J Intern Med 2016;31:612-615. Successful treatmenParaschou t of sensorineural hearing loss in Sjögren’s syndrome with corticosteroid – PubMed (nih.gov)

 

Disclosures/Disclaimers: The listed questions and answers are solely the responsibility of the author and do not necessarily represent the official views of Mercy Hospital-St. Louis, Massachusetts General Hospital, Harvard Catalyst, Harvard University, their affiliate academic healthcare centers, or its contributors. Although every effort has been made to provide accurate information, the author is far from being perfect. The reader is urged to verify the content of the material with other sources as deemed appropriate and exercise clinical judgment in the interpretation and application of the information provided herein. No responsibility for an adverse outcome or guarantees for a favorable clinical result is assumed by the author. Thank you!

Is there a connection between Sjögren’s syndrome and hearing loss?

My patient with rheumatoid arthritis might have been exposed to tuberculosis. Does immunosuppressive therapy affect the results of interferon gamma release assay (IGRA) testing for latent tuberculosis?

FA Manian MD, MPH, , , , , , , , , , , , , , , , , , , Leave a comment

The weight of the evidence to date suggests that immunosuppressive therapy, including steroids, other oral immunosuppressants and anti-tumor-necrosis factor (TNF) agents, may negatively impact IGRA results.1

In some ways the finding of false-negative IGRA in the setting of immunosuppression is intuitive since many immunosuppressive agents are potent inhibitors of T cells and interferon-gamma response. 1,2 Despite this, the initial reports have been somewhat conflicting which makes a 2016 meta-analysis of the effect of immunosuppressive therapy on IGRA results in patient with autoimmune diseases (eg, rheumatoid arthritis, lupus, inflammatory bowel disease) particularly timely. 1

This meta-analysis found a significantly lower positive IGRA results among patients on immunosuppressive therapy ( O.R. 0.66, 95% C.I. 0.53-0.83). Breakdown by IGRA test showed a significant association between QuantiFERON-TB Gold In-Tube and lower positive results and a trend toward the same with T-SPOT though the latter did not reach statistical significance with fewer evaluable studies (O.R. 0.81, 95% C.I 0.6-1.1).   Breakdown by type of immunosuppressant showed significantly negative impact of corticossteroids, other oral immunosuppressants, and anti-TNF agents for all. Some studies have reported daily steroid doses as low as 7.5 mg-10 mg may adversely impact T-cell responsiveness in IGRA. 3,4

So, whenever possible, testing for latent TB should be performed before immunosuppressants are initiated.

Bonus Pearl: Did you know that an estimated one-third of the world’s population may have latent TB?

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References

  1. Wong SH, Gao Q, Tsoi KKF, et al. Effect of immunosuppressive therapy on interferon gamma release assay for latent tuberculosis screening in patients with autoimmune diseases: a systematic review and meta-analysis. Thorax 2016;71:64-72. https://thorax.bmj.com/content/thoraxjnl/71/1/64.full.pdf
  2. Sester U, Wilkens H, van Bentum K, et al. Impaired detection of Mycobacterium tuberculosis immunity in patents using high levels of immunosuppressive drugs. Eur Respir J 2009;34:702-10. https://erj.ersjournals.com/content/34/3/702
  3. Kleinert S, Kurzai O, Elias J, et al. Comparison of two interferon-gamma release assays and tuberculin skin test for detecting latent tuberculosis in patients with immune-mediated inflammatory diseases. Ann Rheum Dis 2010;69:782-4. https://ard.bmj.com/content/69/4/782
  4. Ponce de Leon D, Acevedo-Vasquez E, Alvizuri S, et al. Comparison of an interferon-gamma assay with tuberculin skin testing for detection of tuberculosis (TB) infection in patients with rheumatoid arthritis in a TB-endemic population. J Rheumatol 2008;35:776-81. https://www.ncbi.nlm.nih.gov/pubmed/18398944
My patient with rheumatoid arthritis might have been exposed to tuberculosis. Does immunosuppressive therapy affect the results of interferon gamma release assay (IGRA) testing for latent tuberculosis?

How do I interpret an elevated serum C-reactive protein (CRP) and normal erythrocyte sedimentation rate (ESR) or vice-versa?

FA Manian MD, MPH, , , , , , , , , , , , Leave a comment

Discordance between serum CRP and ESR is not uncommon (1,2). This phenomenon may be due to a variety of factors including the fact that the kinetics of these two tests is quite different, as discussed in another P4P Post.

In a study of CRP/ESR discordance (defined as results differing by 2 or 3 quartiles) in adults, a high CRP/low ESR profile was more likely to be associated with  urinary, GI, blood stream, and pulmonary infections, myocardial infarction, and venous thromboembolism and less likely to be associated with bone and joint infections (1).

In the same study, a high ESR/low CRP was associated with connective tissue diseases, such as systemic lupus erythematosus and strokes (1).

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References

1. Feldman M, Aziz B, Kang GN, et al. C-reactive protein and erythrocyte sedimentation rate discordance: frequency and causes in adults. Translational Research 2013;161:37-43. https://www.ncbi.nlm.nih.gov/pubmed/22921838

2. Colombet I, Pouchot J, Kronz V. Agreement between erythrocyte sedimentation rate and C-reactive protein in hospital practice. Am J Med 2010;123:864.e7-863.e13.https://www.ncbi.nlm.nih.gov/pubmed/20800157

Disclosures: The listed questions and answers are solely the responsibility of the author and do not necessarily represent the official views of Mercy Hospital-St. Louis or its affiliate healthcare centers, Mass General Hospital, Harvard Medical School or its affiliated institutions. Although every effort has been made to provide accurate information, the author is far from being perfect. The reader is urged to verify the content of the material with other sources as deemed appropriate and exercise clinical judgment in the interpretation and application of the information provided herein. No responsibility for an adverse outcome or guarantees for a favorable clinical result is assumed by the author. Thank you!

How do I interpret an elevated serum C-reactive protein (CRP) and normal erythrocyte sedimentation rate (ESR) or vice-versa?

My 70 year old male patient with recent hip fracture has developed fevers with sterile pyuria. How do I interpret the sterile pyuria in this patient?

FA Manian MD, MPH, , , , , , , , , , , , , , , , , , Leave a comment

Although historically sterile pyuria (SP) has been associated with genitourinary (GU) tuberculosis, there are many more common causes to consider in the hospitalized patient (1-3).    

Recent antibiotic exposure (within past 2 weeks) in the setting of UTI is one of the most frequent causes.  Prostatitis is also an often overlooked cause.  Sexually transmitted diseases such as gonorrhea and Chlamydia trachomatis should also be considered in at risk patients. Hospitalized patients with systemic infections outside of the GU tract (e.g. pneumonia, appendicitis, diverticulitis) may also have SP (1-3). High prevalence of SP (>70%) has been reported among patients with appendicitis or diverticulitis (2). 

Non-infectious causes include current or recent catheterization of bladder, urinary stones, stents, GU malignancy, papillary necrosis,  Kawasaki’s disease, autoimmune diseases (eg, SLE) and analgesic nephropathy. 

I would start with repeating the u/a as 50% of sterile pyuria may be transient (3). If repeat u/a still shows pyuria, a prostate exam in our elderly male is indicated to exclude prostatitis. 

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References:

  1. Wise GJ, Schlegel PN. Sterile pyuria. N Engl J Med 372;11:1048-54. https://www.nejm.org/doi/pdf/10.1056/NEJMra1410052
  2.  Goonewardene S, Persad R. Sterile pyuria: a forgotten entity. Ther Adv urol 2015; 7:295-298.  https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4549703/ 
  3. Hooker JB, Mold JW, Kumar S. Sterile pyuria in patients admitted to the hospital with infections outside of the urinary tract. J Am Board Fam Med 2014;2&:97-103. https://www.jabfm.org/content/27/1/97.long#T1 

Disclosures: The listed questions and answers are solely the responsibility of the author and do not necessarily represent the official views of Massachusetts General Hospital, Harvard Catalyst, Harvard University, its affiliate academic healthcare centers, or its contributors. Although every effort has been made to provide accurate information, the author is far from being perfect. The reader is urged to verify the content of the material with other sources as deemed appropriate and exercise clinical judgment in the interpretation and application of the information provided herein. No responsibility for an adverse outcome or guarantees for a favorable clinical result is assumed by the author. Thank you!

My 70 year old male patient with recent hip fracture has developed fevers with sterile pyuria. How do I interpret the sterile pyuria in this patient?