A 34-year-old female – lower back pain and dry eye – 13/03/2022

Discussion of the case

The 34 year female patient presented with complaints of lower back pain of inflammatory nature and dryness of eyes, mouth, and skin for the past one year. She had pain bilaterally in the soles, Achilles tendons, and medial and lateral epicondyles of elbow joint since 2 months. The history also revealed the presence of bloody stools for 20 days but no frequent diarrhea was reported. She had a previous incidence of urinary tract infection treated with antibiotics. She had a maternal history of lower back pain. She was diagnosed to have seizure disorder and was on anti-epileptics for the past 5 years. Vitals and systemic examination did not reveal any abnormality.

Previous investigations indicate that her ESR was 40 mm per hour, TSH- 1.5 IU, and RF- 35 IU/ml; while the rest of the parameters were normal. Whereas re-evaluation conducted in our institution had revealed that the patient was positive for ANA and anti-RO, while RF was negative. CRP and ESR were normal.

Clinical examination conducted in our institution had shown that she had inflammatory backache with recent-onset enthesitis and UTI.  She had dry eyes and was on teardrops.  But there was no dry mouth or joint symptoms. Her inflammatory parameters were normal and there was no evidence of sacroiliitis in the pelvis radiograph.

The disease history of 1-year duration indicates the rare possibility of persistent infections. However, the patient had an episode of UTI one month before, and the occurrence of inflammatory back pain and enthesitis prior to UTI, which are suggestive of seronegative spondyloarthropathies (SpA). The patient fulfills the European Spondyloarthropathy Study Group (ESSG) for classifying SpA (table 1).¹

Table 1: ESSG criteria for characterizing SpA

Fig. 2: ASAS classification criteria for axial and peripheral spondyloarthritis²

(A) ASAS classification criteria for axial spondyloarthritis; (B) ASAS classification criteria for peripheral spondyloarthritis. *Sacroiliitis on imaging refers to definite radiographic sacroiliitis according to the modified New York criteria or active sacroiliitis on MRI according to the ASAS definition. **Peripheral arthritis: usually predominantly lower limbs and/ or asymmetric arthritis; enthesitis: clinically assessed; dactylitis: clinically assessed. SpA: spondyloarthritis.

A positive HLA-B27 can help in classifying the patients with SpA.³ Comparison of the clinical features of sarcoidosis, Sjogren's syndrome, and SpA (table 2) shows that the SpA does not explain the presence of dry eyes from the past one year.⁴

Table 2:  Comparison of the clinical features of sarcoidosis, Sjogren's syndrome, and SpA

The conjoint occurrence of polyarthritis, enthesitis, and dry eye can be explained by sarcoidosis. There are no specific criteria to diagnose sarcoidosis. The possibility of sarcoidosis can be ruled out since the patient's chest radiograph and serum ACE were normal and there was no lymphadenopathy.

The other possibility is Sjögren's syndrome. The classification criteria to be followed for Sjögren's syndrome is listed in table 3.⁵

Table 3: ACR-EULAR Classification Criteria for primary Sjögren’s syndrome (pSS)

The classification of SS applies to any individual who meets the inclusion criteria, does not have any condition listed as exclusion criteria, and who has a score ≥ 4 when summing the weights from the following items:

The patient partially fulfills the above-mentioned criteria. In a large series reported from France, the musculoskeletal features of Sjogren's syndrome were seen in 10% of the study participants. Five of the patients had an axial distribution with sacroiliitis and inflammatory back pain.⁶ This finding clearly explains the inflammatory back pain noted in the present case. The patient’s ANA profile, ophthalmologically-proven dry eyes, and Ro52 positivity suggest the possibility of Sjogren's syndrome. Enthesitis is also noted very rarely in such patients.  A possible overlap of SpA and Sjogren's can be avoided through HLA-B27, which was negative in the current case.

Final diagnosis

Primary Sjogren's syndrome

Follow-up

The patient responded well to the treatment of methotrexate and hydroxychloroquine, and a significant reduction in articular/eye symptoms was noted.

Learning points

• Patients with Sjogren's syndrome can sometimes have non-specific inflammatory pain symptoms
• Tests like HLA- B27 can assist in excluding diagnosis.

References

1. Lipton S, Deodhar A. The new ASAS classification criteria for axial and peripheral spondyloarthritis: promises and pitfalls. International Journal of Clinical Rheumatology. 2012 Dec 1;7(6):675.
2. Poddubnyy D. Classification vs diagnostic criteria: the challenge of diagnosing axial spondyloarthritis. Rheumatology. 2020 Oct;59(Supplement_4):iv6-17
3. Rudwaleit M, van der Heijde D, Landewé R, Akkoc N, Brandt J, Chou CT, et al. The Assessment of Spondylarthritis International Society classification criteria for peripheral spondyloarthritis and for spondyloarthritis in general. Ann Rheum Dis. 2011 Jan;70(1):25-31.
4. Kobak S. Sarcoidosis: a rheumatologist's perspective. Ther Adv Musculoskelet Dis. 2015 Oct;7(5):196-205.
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. Arthritis Rheumatol. 2017;69(1):35-45. doi:10.1002/art.39859.
6. Fauchais AL, Oattara B, Gondram G et al. Articular manifestations in primary Sjogren's syndrome: clinical significance and prognosis of 188 patients. The Rheumatology 2010;49:1164–1172.