Rare presentations of Sjogren syndrome

Authors

  • Miet D. Shah Department of Medicine, KJ Somaiya Medical College and Research Centre, Mumbai, Maharashtra, India
  • Atiullah I. Malik Department of Medicine, KJ Somaiya Medical College and Research Centre, Mumbai, Maharashtra, India
  • Niharika H. Gill Department of Medicine, KJ Somaiya Medical College and Research Centre, Mumbai, Maharashtra, India
  • Murtuza N. Ghiya Department of Emergency Medicine, KJ Somaiya Medical College and Research Centre, Mumbai, Maharashtra, India
  • Ashish K. Jain Department of Medicine, KJ Somaiya Medical College and Research Centre, Mumbai, Maharashtra, India

DOI:

https://doi.org/10.18203/2349-3933.ijam20241022

Keywords:

Neuromyelitis optica, Keratoconjunctivitis sicca, Hypokalemic periodic paralysis

Abstract

Sjögren’s syndrome is a chronic systemic autoimmune disease characterized by lymphocytic infiltration of exocrine glands. It most commonly presenting with sicca symptoms. Sicca refers to dryness most often involving the eyes and mouth due to inflammation and resultant pathology of the lacrimal and salivary glands. Up to one-half of affected individuals also develop extra-glandular involvement in organs such as the joints, skin, lungs, gastrointestinal (GI) tract, nervous system, and kidneys. This condition is frequently associated with other autoimmune disorders including rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) - called the secondary Sjogrens syndrome. Evaluation of a patient with suspected Sjogren syndrome should include an evaluation of oral and ocular dryness and function. In addition to the history, this may include the performance of a Schirmer test, slit-lamp exam with vital dye staining, salivary flow rate, and/or nuclear scintigraphic evaluation of the salivary glandular function. Assessment of autoantibodies (ANA, RF, SS-A, and SS-B) should also be performed. Of these, SS-A is probably the most sensitive and specific antibody for Sjogren's but alone is not diagnostic since it may be present in other autoimmune disorders and may be absent in up to a third of Sjogren cases. The most specific single test is a minor salivary gland (lip) biopsy which will demonstrate focal lymphocytic sialadenitis (FLS). Therapies are directed toward replacing moisture at affected glandular sites and suppressing the autoimmune response locally as well as systemically. This activity reviews the evaluation and management of Sjogren syndrome and explains the different rare presentations in which it can present.

 

References

Carsons SE, Patel BC. Sjogren Syndrome. In: StatPearls. Treasure Island (FL): StatPearls Publishing. 2024.

Aiyegbusi O, McGregor L, McGeoch L, Kipgen D, Geddes CC, Stevens KI. Renal Disease in Primary Sjögren's Syndrome. Rheumatol Ther. 2021;8(1):63-80.

Moutsopoulos HM, Mavragani CP. Harrison’s Principles of Internal Medicine 21st Edition. McGraw Hill. 2022.

Dellafiore C, Villa A, Zibera F. Sjögren syndrome: A case report. 2012;15:108-19.

Jayarangaiah A, Sehgal R, Epperla N. Sjögren's syndrome and neuromyelitis optica spectrum disorders (NMOSD)--a case report and review of literature. BMC Neurol. 2014;14:200.

Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for Neuromyelitis Optica. Neurology. 2006;66:1485-9.

Jacob J, McKeon A, Nakashima I, Sato DK, Elsone L, Fujihara K, et al. Current concept of neuromyelitis optica and neuromyelitis optica spectrum disorders. J Neurol Neurosurg Psychiatry. 2013;84:922-30.

Nielsen S, Nagelhus EA, Amiry-Moghaddam M, Bourque C, Agre P, Ottersen OP. Specialized membrane domains for water transport in glial cells: high-resolution immunogold cytochemistry of aquaporin-4 in rat brain. J Neurosci. 1997;17:171-80.

Icoz S, Tuzun E, Kurtuncu M, Durmus H, Mutlu M, Eraksoy M, et al. Enhanced IL-6 production in aquaporin-4 antibody positive neuromyelitis optica patients. Int J Neurosci. 2010;120:71-5.

Uzawa A, Mori M, Masuda S, Kuwabara S. Markedly elevated soluble intercellular adhesion molecule 1, soluble vascular cell adhesion molecule 1 levels, and blood-brain barrier breakdown in neuromyelitis optica. Arch Neurol. 2011;68:913-7.

Kinoshita M, Nakatsuji Y, Moriya M, Okuno T, Kumanogoh A, Nakano M, et al. Astrocytic necrosis is induced by anti-aquaporin-4 antibody-positive serum. Neuroreport. 2009;20:508-12.

Vasquez-Rios G, Westrich DJ Jr, Philip I, Edwards JC, Shieh S. Distal renal tubular acidosis and severe hypokalemia: a case report and review of the literature. J Med Case Rep. 2019;13(1):103.

Downloads

Published

2024-04-26

Issue

Section

Case Reports