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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 17  |  Issue : 3  |  Page : 715-717

Delayed diagnosis of ankylosing spondylitis


Department of Orthopedics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Wardha, Maharashtra, India

Date of Submission14-Jul-2022
Date of Decision03-Sep-2022
Date of Acceptance20-Sep-2022
Date of Web Publication2-Nov-2022

Correspondence Address:
Dr. Nareshkumar Satyanarayan Dhaniwala
Department of Orthopedics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Medical Sciences (Deemed to be University), Sawangi, Wardha - 442 107, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jdmimsu.jdmimsu_303_22

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  Abstract 


Ankylosing spondylitis (AS) is a chronic inflammatory condition affecting the axial skeleton, enthesis, and peripheral joints and is included in a group of disorders termed spondyloarthropathy. Its diagnosis is based on clinical and radiological features. This article reports an atypical case of AS in a 24-year-old female presenting with bilateral hip arthritis without any back symptoms. The report discusses the causes of delayed diagnosis and the relevance of thorough clinical examination.

Keywords: Ankylosing spondylitis, delayed diagnosis, sacroiliitis, spondyloarthropathy


How to cite this article:
Dhaniwala NS, Prasad S, Dhaniwala MN, Salwan A. Delayed diagnosis of ankylosing spondylitis. J Datta Meghe Inst Med Sci Univ 2022;17:715-7

How to cite this URL:
Dhaniwala NS, Prasad S, Dhaniwala MN, Salwan A. Delayed diagnosis of ankylosing spondylitis. J Datta Meghe Inst Med Sci Univ [serial online] 2022 [cited 2023 Feb 4];17:715-7. Available from: http://www.journaldmims.com/text.asp?2022/17/3/715/360207




  Introduction Top


Ankylosing spondylitis (AS) is a systemic autoimmune condition causing inflammation of sacroiliac joints, vertebrae, and large peripheral joints. Inflammation of a tendon or ligament insertion at the bone (enthesis) is also common. It is one of the most common seronegative spondyloarthropathies.[1]

The common age group of presentation is from 15 to 45 years and it commonly affects males more than females in a 3:1 ratio.[2] More than 80% of patients develop the symptoms before the age of 30 years.[1] The condition is said to be genetically related to the human leukocyte antigen (HLA-B 27) seen in 90% of patients. It develops through complex interactions between genetic background and environmental factors. The primary target of the immune response is at the cartilage/bone interface and enthesis. Inflammation is followed by fibrosis and calcification of ligaments. Male patients have more structural changes, including bamboo spine than female patients.

The clinical spectrum varies from a common presentation of back pain and stiffness in the lower back and pelvis to uncommon involvement of large joints, enthesis, anterior uveitis, and inflammatory bowel disease. Peripheral arthritis is usually mono- or oligoarticular, more commonly involving lower limb joints. The spinal involvement ascends to involve the upper part of the spine along with costovertebral joints restricting chest expansion, rotation, and flexion–extension of the lumbar spine. Syndesmophytes and ankylosis are characteristic features, seen on radiographs after some months to many years. Hip and shoulder joints get affected in about 20% of patients. In advanced stages, the spine is ankylosed with a round kyphotic deformity. The diagnosis of AS is based on Modified New York Criteria.[3] This case report describes a delayed diagnosis of AS in a young female who presented with involvement of both hips only without any back pain or deformity.


  Case Report Top


A 24-year-old Indian unmarried female presented to the orthopedics outpatient department in our tertiary care hospital with chief complaints of difficulty in walking along with pain in both hips for 3 years. Initially, she experienced spontaneous pain in her left hip and a mild fever for a few days. The pain was insidious in onset and gradually progressive. She clinically had left hip arthritis. She underwent left hip aspiration and was initially treated with analgesic, rest, and hip exercises. C-reactive protein (CRP) was 24 and rheumatoid arthritis (RA) factor was negative. No definite diagnosis could be established on culture and smear examination of the aspirated fluid. The patient was empirically treated with antitubercular drugs for 1.5 years. She improved after this and was asymptomatic for 6 months. Later on, there was a recurrence of pain in the left hip with the development of pain in her right hip also. She was unable to bear weight and unable to squat and sit cross-legged. In the past, she had pain in her right hip 5 years ago which had improved on its own. There was no history of back pain or stiffness. There was no family history of tuberculosis or similar complaints.

Examination revealed the patient to be a short-statured normally built lady with no obvious abnormality. Her respiration was primarily abdominal, chest expansion was nil but air entry into the lungs was adequate. She was not able to sit normally on the bed or chair, could not stand on her own, and managed to walk only with the support of a walker. In the supine position, both her lower limbs remained in the abduction and external rotation attitude more on the left than the right. Both her hips were tender and fibrous ankylosed, the right in 5° external rotation and the left in 30° abduction and 15° external rotation. Both knee movements were 0°–110°. There was no movement detected in the lumbar spine and junctional area. Her neck movements were terminally restricted. Sacroiliac joints were mildly tender. Clinically, the case was diagnosed as AS with gross involvement of the dorsal and lumbar spine along with bilateral hip ankylosis.

Her CRP (20.31) and erythrocyte sedimentation rate (58) were raised while her complete blood count, coagulation profile, liver function, and kidney function tests were within normal range. HLA-B27 antigen was positive.

Her X-ray pelvis showed obliteration of the right-sided sacroiliac joint with irregularity of the left sacroiliac joint in the lower half. It also showed decreased joint space on both hips with periarticular osteopenia and calcification on the superolateral aspect of the left hip [Figure 1]. Her X-ray lumbar spine showed calcification in the anterior part of the L4–L5 disc region and the narrow neural canal [Figure 2]. The classical bamboo spine picture is not seen. The patient was started on indomethacin and exercises of the chest, neck, back, and peripheral joints, and felt relief in pain. She was advised of bilateral total hip replacement to enable her to sit and ambulate in a better manner. She has undergone both hip replacements in a staged manner at a government hospital and is able to sit and ambulate with support.
Figure 1: X-ray pelvis showing decreased joint space on both hips with periarticular osteopenia and calcification on the superolateral aspect of the left hip and involvement of both sacroiliac joints

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Figure 2: X-ray lumbar spine shows calcification in the anterior part of L4–L5 intervertebral disc space with narrowing of neural canal

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Patient's perspective

I was having pain in my hips for a long time. After taking drugs given to me at the hospital I am relieved of pain and difficulty in sitting. After various investigations and repeated physical examinations by doctors, I was told that I am having AS disease which is restricting my both hip and back movements. Total hip replacement was advised for both hips. After this surgery, I am very happy and able to sit comfortably. At present, I am walking with the support of a walker. I am very hopeful of further improvement in my walking ability. I intend to remain in touch with the treating doctors.


  Discussion Top


Ankylosing spondylosis is an uncommon disease in females. Initial studies showed a male-to-female ratio of 10:1,[4] but recent studies are reporting it to be 3:1.[2] A continuous decline in the male-to-female ratio leading to equal prevalence has been reported in Switzerland.[5]

Modified New York criteria[3] include (1). Clinical criteria of lower back pain and stiffness for >3 months improving with exercise but not relieved by rest, (2). Restriction of lumbar spine movement in both sagittal and frontal planes and (3). Restriction of chest expansion relative to normal values along with (4). Sacroilitis > than Grade 2 bilaterally or Grade 3-4 unilaterally should be looked for on X-ray pelvis or MRI. The presence of radiological criteria along with at least one clinical criteria is considered essential for the diagnosis of AS. Our patient had two positive criteria on clinical examination out of the three criteria and sacroiliitis Grade 2 on both sides. Grading of sacroiliac joints includes normal at 0, suspicious changes as 1, minor changes as 2, moderate changes as 3, and ankylosis as 4.[6]

Radiological changes of sacroiliitis may not be apparent in early cases and may delay the diagnosis. Magnetic resonance imaging (MRI) detects the inflammation in these joints earlier than the X-ray appearance.

Our case had a delay in diagnosis because the patient presented with the primary complaints of hip pain although she also had involvement of the lumbar spine, costovertebral joints, and sacroiliac joints which was detected on physical examination by us and supported by radiological changes in the pelvis. She is positive for HLA-B27. The lack of suspicion of AS, in this case, seems to have occurred because the patient is female, and as against the common presentation of back pain and stiffness, she presented with hip pain only. Even to date despite having gross limitation of all movements of the spine in the lumbar and dorsal region, she does not complain of any back pain or sacroiliac pain. This might have led to incomplete examination on the first visit to the hospital and contributed to missing the back pathology and delay in the diagnosis. Improvement with empirically started antitubercular drugs causing relief in pain further added to delay in diagnosis. Our patient has a feeling of stretch in both the gluteal and upper thigh region after walking, indicating sacroiliac involvement.

Many authors have described cases of delayed diagnosis of AS even in the presence of back pain at an atypical site. Patel et al. described a patient with AS who was diagnosed 20 years after his upper back and neck pain.[7]

AS has a strong association with HLA-B 27 which is a normal gene found in 8% of the Caucasian population. The positivity of this gene is not diagnostic for AS but it is a strong predictor.[8] Fallahi S and Jamshidi AR have discussed the diagnostic delay in AS in their study of 163 patients with AS. Their survey showed a diagnostic delay which was longer in patients with enthesitis compared to patients without enthesitis. It was more in HLA-B 27-negative patients compared to HLA-B 27-positive patients. They also noted that educational level was negatively correlated with diagnostic delay.[9] Aggarwal R and Malviya AN have also discussed the diagnostic delay of AS among Indian patients.[10] The main causes of delay were incorrect diagnosis as nonspecific back pain, degenerative disc disease, RA, and tuberculosis of the spine.

Rusman et al. have discussed gender differences and delays in the diagnosis of AS.[11] The physical examination should include neck movements, chest expansion, lumbar spine movement measurement, and sacroiliac joint tests in such cases. Modified New York criteria of diagnosis should be used more frequently in cases of low back pain lasting >3 months. Sudoł-Szopińska et al. in their study on 101 consecutive patients aged 19–71 years (mean age 40.6) concluded that MRI diagnoses sacroiliitis before radiographic changes in the joints. They noted edema and inflammatory changes in 39.5% of patients with normal X-ray pelvis.[12]


  Conclusion Top


A case of AS presented with bilateral hip ankylosis is reported to highlight causes of delay in diagnosis. The role of detailed physical examination and application of Modified New York criteria in the diagnosis of AS is brought out. Any joint pain lasting >3 months should make the treating doctor consider the possibility of AS even in the absence of back pain. AS should be considered a differential diagnosis in cases of large joint inflammatory arthritis, tuberculosis of the hip with or without lower back pain, or sacroiliitis in patients >15 years of age.

Take-away message

AS should always be considered a differential diagnosis in bilateral hip involvement. One must be aware that radiological changes in sacroiliac joints and calcification of longitudinal ligaments in the spine leading to the bamboo spine are very late phenomena and occur more in females than in males. MRI of the pelvis should be considered for early detection of sacroiliac joint involvement in suspected cases of AS.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Braun J, Sieper J. Ankylosing spondylitis. Lancet 2007;369:1379-90.  Back to cited text no. 1
    
2.
Lee W, Reveille JD, Weisman MH. Women with ankylosing spondylitis: A review. Arthritis Rheum 2008;59:449-54.  Back to cited text no. 2
    
3.
Rudwaleit M, van der Heijde D, Landewé R, Akkoc N, Brandt J, Chou CT, et al. The Assessment of Spondyloarthritis International Society classification criteria for peripheral spondyloarthritis and for spondyloarthritis in general. Ann Rheum Dis 2011;70:25-31.  Back to cited text no. 3
    
4.
West HF. Aetiology of ankylosing spondylitis. Ann Rheum Dis 1949;8:143-8.  Back to cited text no. 4
    
5.
Baumberger H, Khan MA. SAT0417 Gradual progressive change to equal prevalence of ankylosing spondylitis among males and females in Switzerland: Data from the Swiss Ankylosing Spondylitis Society (SVMB). Ann Rheumatic Dis 2017;76:929.1-929.  Back to cited text no. 5
    
6.
van der Linden S, Valkenburg HA, Cats A. Evaluation of diagnostic criteria for ankylosing spondylitis. A proposal for modification of the New York criteria. Arthritis Rheum 1984;27:361-8.  Back to cited text no. 6
    
7.
Patel P, Hussain H, Fahey J. Delayed diagnosis of ankylosing spondylitis: A missed opportunity? Cureus 2019;11:e5723.  Back to cited text no. 7
    
8.
Reveille JD, Hirsch R, Dillon CF, Carroll MD, Weisman MH. The prevalence of HLA-B27 in the US: Data from the US National Health and Nutrition Examination Survey, 2009. Arthritis Rheum 2012;64:1407-11.  Back to cited text no. 8
    
9.
Fallahi S, Jamshidi AR. Diagnostic delay in ankylosing spondylitis: Related factors and prognostic outcomes. Arch Rheumatol 2016;31:24-30.  Back to cited text no. 9
    
10.
Aggarwal R, Malaviya AN. Diagnosis delay in patients with ankylosing spondylitis: Factors and outcomes – An Indian perspective. Clin Rheumatol 2009;28:327-31.  Back to cited text no. 10
    
11.
Rusman T, van Vollenhoven RF, van der Horst-Bruinsma IE. Gender differences in axial spondyloarthritis: Women are not so lucky. Curr Rheumatol Rep 2018;20:35.  Back to cited text no. 11
    
12.
Sudoł-Szopińska I, Kwiatkowska B, Włodkowska-Korytkowska M, Matuszewska G, Grochowska E. Diagnostics of sacroiliitis according to ASAS criteria: A comparative evaluation of conventional radiographs and MRI in patients with a clinical suspicion of spondyloarthropathy. Preliminary results. Pol J Radiol 2015;80:266-76.  Back to cited text no. 12
    


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