Modern Rheumatology Case Reports, 8(2), 2024, 259–263

DOI: [Link]
Advance Access Publication Date: 7 February 2024
Case Report

A case of juvenile-onset ankylosing spondylitis effectively

treated with tumour necrosis factor-alpha inhibitor agents
Akira Sakaguchi, Naoki Kondo *, Rika Kakutani, Eiji Kinoshita, Yasufumi Kijima and
Hiroyuki Kawashima
Division of Orthopedic Surgery, Department of Regenerative and Transplant Medicine, Niigata University Graduate School of Medical and
Dental Sciences, Chuo-Ku, Niigata, Japan
*Correspondence: Naoki Kondo; Naokikondo1214@[Link]; Division of Orthopedic Surgery, Department of Regenerative and Transplant Medicine, Niigata
University Graduate School of Medical and Dental Sciences, 1-757, Asahimachi-dori, Chuo-Ku, Niigata 951-8510, Japan.

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ABSTRACT
A 15-year-old girl had experienced hip pain at 11 years of age. At 15 years of age, the patient complained of persistent generalised pain. Her
rheumatoid factor and serum matrix metalloproteinase-3 levels were below standard values; there were no inflammatory responses, and the
human leukocyte antigen test was negative for B27 and positive for B52 and B62. The bath ankylosing spondylitis disease activity index (BASDAI)
value was 8.0 at the time of induction and 3.1 at 6 months after the introduction of adalimumab (at a dose of 40 mg). The BASDAI value improved
with an increase in the dose of adalimumab to 80 mg at 8 months after the initial introduction of adalimumab (at 40 mg), although it remained
at 4.8 at 16 months after the dose increase. The BASDAI value was 2.6 at 6 months, 2.7 at 1 year, and 1.8 at 1.5 years after the introduction of
infliximab, indicating that the patient had progressed well without any adverse events. Based on this case, juvenile ankylosing spondylitis is a
differential diagnosis for low back pain and generalised pain since childhood. Tumour necrosis factor (TNF) inhibitors were promptly introduced
in this case, although it took 4 years from the initial presentation. TNF inhibitors were effective in treating juvenile ankylosing spondylitis in the
present case without any adverse events. This case is notable because juvenile onset ankylosing spondylitis is one of the reasons for severe
lumbago since childhood and because TNF inhibitors were administered promptly after diagnosis.
KEYWORDS: Generalised pain; back pain; juvenile onset ankylosing spondylitis; tumour necrosis factor inhibitors; efficacy

Introduction general body pain, particularly in the ankle joints and the left
Ankylosing spondylitis (AS) is an immune-mediated inflam- hip joint.
matory disease with axial manifestations [1]. Patients expe- The patient had persistent bilateral hip-joint pain at
riencing AS symptoms at ≤16 years of age are diagnosed 11 years of age, but pain in the hip joints transiently improved
with juvenile-onset AS (JoAS). Patients with JoAS typically within 1 year. Since then, she experienced pain in multiple
have more peripheral joint involvement, both clinically and local regions, such as the neck, lumbar spine, and all four
radiographically, and more root joint involvement, such as limbs including the hip region and ankle joint. She visited our
involvement of the hip and shoulder joints [2]. In the Japanese clinic with suspected juvenile idiopathic arthritis.
population, the incidence of AS has been reported to be Laboratory findings revealed no inflammatory response
2.6/100,000 (0.0026%) [3]. Among patients with AS, the (C-reactive protein level, 0.06 mg/dL; erythrocyte sedimen-
prevalence of JoAS has been reported to vary between 9% tation rate, 5 mm/h). Additionally, anti-cyclic citrullinated
and 21% in Caucasian populations [4]. peptide antibody and rheumatoid factor levels were under
JoAS is considered a differential diagnosis for lumbar pain their respective sensitivities. Antinuclear antibody and anti-
since childhood. Here, we present a case of JoAS, which took RNP antibody tested negative. The patient’s serum matrix
more than 4 years to diagnose and wherein the administration metalloproteinase-3 level was 10 ng/mL (within normal lim-
of tumour necrosis factor (TNF) inhibitors was effective in its) (Table 1). The human leukocyte antigen (HLA) test was
treating the condition. positive for B52 and B62 but negative for B27.
At the initial visit, the patient had joint pains in her left
hip and ankle, but joint swelling was not detected and she
Case presentation did not have fever. Signs of skin rash, lymphadenopathy,
A 15-year-old Japanese girl presented at our facility (Division or splenomegaly were also not detected. Sonography was
of Orthopaedic Surgery, Niigata University Medical and Den- performed for pain in her ankle joint and Achilles tendon.
tal General Hospital, Niigata, Japan) in 2015 with persistent Both grayscale and pulse Doppler signals were grade 0 in the

Received 26 July 2023; Accepted 29 January 2024

© Japan College of Rheumatology 2024. Published by Oxford University Press.
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Table 1. Laboratory findings at each visit.
260

Parameter Unit Normal VISIT1 VISIT2 VISIT3 VISIT4 VISIT5 VISIT6 VISIT7 VISIT8 VISIT9
Months for the −1 0 6 9 10 24 29 38 41
time course
BASDAI N/A 8 3.1 4.4 2.7 4.8 2.6 6.6 3.7
ASDAS-CRP N/A 3 1.6 2.3 1.7 2.4 1.6 3.1 1.9
Blood WBC /μl 3590–9640 3930 4180 5370 8290 6520 5940 5890 6750 6350
Neutrophil % 41–75 48.6 59.3 60.3 57.5 52 48.2 48.9 47.1 46
Lymphocyte % 21–51 37.7 27.3 30.2 33.2 37.1 40.4 39.2 43.1 40.6
Eosinophil % 0.2–8.4 4.6 5.5 3.9 3.3 4 3.9 4.4 3.6 3.8
Basophil % 0.2–1.8 1 0.5 0.6 0.1 0.6 0.8 0.5 0.4 0.5
Monocyte % 3–8 8.1 7.4 5 5.9 6.3 6.7 7 5.8 9.1
RBC ×104 /μL 386–492 466 466 459 457 456 449 417 455 428
Hb g/dL 11.6–14.8 13.7 13.6 13.7 13.4 13.4 13.8 12.9 14.3 13.6
Ht % 35.1–44.4 40.5 40.7 41 39.7 39.1 40.1 37.8 42.3 39.7
Plt ×104 /μL 15.8–34.8 29.5 26.8 28.5 27.5 27.7 26.9 25.8 26.9 27.2
Total protein g/dL 6.6–8.1 6.9 7.3 7.3 7 7.2 7.3 6.9 7.4 7.2
Albumin g/dL 4.1–5.1 4.1 4.2 4.3 4.2 4.1 4.2 3.9 4.2 3.9
AST U/L 13–30 24 20 21 24 19 19 16 23 21
ALT U/L 7–23 39 18 21 27 18 11 8 23 12
LDH U/L 124–222 169 159 174 187 163 142 132 194 152
γ-GTP U/L 9–32 15 14 14 12 12 12 10 11 11
BUN mg/dL 8–20 13 15 10 10 12 13 13 16 15
Creatinine mg/dL 0.46–0.79 0.69 0.59 0.59 0.58 0.7 0.63 0.69 0.78 0.67
T-Chol mg/dL 142–248 184 159 179 159 168 191 178 174 160
Na mmol/L 138–145 140 140 139 137 138 138 140 140 139
K mmol/L 3.6–4.8 3.9 3.7 3.8 3.3 3.7 3.8 3.4 3.7 4
Cl mmol/L 101–108 105 107 106 106 106 104 105 105 105
Ca mg/dL 8.8–10.1 9.2 9.2 9.4 9.3 9.3 9.4 9.2 9.5 9.3
iP mg/dL 2.7–4.6 4 2.7 3.5 3.6 3.4 3.2 3.3 3.7 3.3
T-Bil mg/dL 0.4–1.5 0.6 0.5 0.6 0.5 0.6 0.7 0.5 0.5 0.4
ESR mm/hr 3–15 5 7 7 4 5 4 4 3 5
MMP3 ng/mL 16.1–56.8 10 10 13.4 10 10.3 10 10 11.7 18.6
RF IU/mL <5 <5.0 <5.0 <5 <5 <5 <5 <5 <5 <5
CRP mg/dL <0.14 0.06 0.25 0.14 0.24 0.27 0.09 0.2 0.21 0.22
Urine
Proteinuria ± ± ± ± 1+ – ± ± –
Urinary sugar
Specific gravity 1.028 1.03 1.022 1.024 1.024 1.006 1.027 1.027 1.017
pH 6 6 6 6 6.5 6 6.5 6 6.5
Keton body
White blood cell 1+ 1+
Hematuria

VISIT1, the initial visit; this visit was defined as one month (−1) before the administration of adalimumab. VISIT2, at the administration of adalimumab of 40 mg biweekly (time, 0 months). VISIT3, at 6 months
after VISIT2; improvement was observed by adalimumab therapy (40 mg biweekly). VISIT4, at 9 months after VISIT2, indicating the point that adalimumab was dosed up to 80 mg biweekly. VISIT5, at 12 months
after VISIT2; the improvement of disease activity was observed by adalimumab therapy (80 mg biweekly). VISIT6, at 24 months after VISIT2, indicating the point of worsening of disease activity under adalimumab
(80 mg biweekly) and the switch to infliximab (250 mg). VISIT7, at 29 months after VISIT2; the improvement of disease activity was observed with 250 mg of infliximab therapy. VISIT8, at 38 months after VISIT2,
indicating the point of worsening under 250 mg of infliximab and dosed up to 300 mg. VISIT9, at 41 months after VISIT2; the improvement was observed with 300 mg of infliximab therapy.
Abbreviations; WBC: white blood cell; RBC: red blood cell; Hb: haemoglobin; Ht: haematocrit; Plt: platelet; AST: aspartate aminotransferase; ALT: arginine aminotransferase; LDH: lactate dehydrogenase; ALP:
alkaline phosphatase; γ-GTP: gamma-glutamyl transpeptidase; BUN: blood urine nitrogen; T-Chol: total cholesterol; Na: sodium; K: kalium; Cl: chloride; Ca: calcium; iP: inorganic phosphorus; T-Bil: total-bilirubin;
ESR: erythrocyte sedimentation rate; MMP-3; matrix metalloproteinase-3; RF: rheumatoid factor; CRP: C-reactive protein.
Sakaguchi et al.

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A case of juvenile-onset ankylosing spondylitis effectively treated with tumour necrosis factor-alpha inhibitor agents 261

cervical spine (no ankylosing changes), hip joints, or ankle

joints (no enthesitis) (Figure 2).
From the findings of persistent lumbago for more than
3 months, X-ray findings of sacroiliac joints that were both
grade 3, and based on the modified New York criteria (1984)
[7] for AS, the patient’s condition fulfilled the diagnostic crite-
ria for AS (the clinical symptom #1; lumbar pain that persisted
more than 3 months and #2 radiological findings; bilateral
sacroiliac joints were both grade 3), and she was diagnosed
accordingly. The age of onset was <16 years; hence, the patient
was finally diagnosed with JoAS.
Figure 1. Lumbar and sacroiliac joint findings on plain radiography and CT.
The patient was initially treated with nonsteroidal
Bamboo spine finding is not detected on the anteroposterior lumbar
spine view (a). Spinal syndesmophyte and enthesitis are not detected on anti-inflammatory drugs (NSAIDs) and salazosulfapyridine
the lateral lumbar spine view (b). In bilateral sacroiliac joints, partial (1000 mg/day) because she had peripheral joint pain, although
ankylosing in each upper region is detected (c) and determined as ‘grade salazosulfapyridine was not covered under medical insurance

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3’ in the modified New York diagnostic criteria for ankylosing spondylitis. for JoAS. However, her multiple arthralgia and lumbar pain
CT images demonstrate that bone erosions are detected (white did not improve (defined VISIT1, 1 month before the admin-
arrow-heads) without joint space narrowing (d).
istration of adalimumab). Then, she was administered 40 mg
adalimumab biweekly (VISIT2, at the time of the adminis-
tration of the first TNF inhibitor). Before the administration
of adalimumab, the bath ankylosing spondylitis disease activ-
ity index (BASDAI) [7] value was 8.0, and the ankylosing
spondylitis disease activity score-C-reactive protein (ASDAS-
CRP) [7, 8] was 3.0. The values decreased to 3.1 (BASDAI)
and 1.6 (ASDAS-CRP) 6 months after adalimumab adminis-
tration (VISIT3, at 6 months). However, her ankle-joint pain
persisted, and the BASDAI value and ASDAS-CRP increased
to 4.4 and 2.3, respectively (VISIT4, at 9 months). Therefore,
adalimumab was administered biweekly at a maximum dose
of 80 mg. Subsequently, the BASDAI value improved to 2.7,
and the ASDAS-CRP improved to 1.7 (VISIT5, at 12 months).
However, the values worsened again to 4.8 (BASDAI) and
2.4 (ASDAS-CRP) 15 months after treatment with 80 mg of
adalimumab (VISIT6, 24 months).
The patient’s treatment was switched to infliximab at
Figure 2. Plain radiographs of cervical spine, hip joints, and bilateral ankle 5 mg/kg (250 mg in total). After 5 months, the BASDAI value
joints. Spinal ankylosing lesions are not detected in her cervical spine (a,
improved to 2.6, and the ASDAS-CRP improved to 1.6
b). Hip deformity and destruction are note detected (c). Arthritis of ankle
joints or enthesitis of the Achilles tendon and plantar tendon are not
(VISIT7, at 29 months). Nonetheless, the values worsened to
present (d, e, f, and g). 6.6 (BASDAI) and 3.1 (ASDAS-CRP) 14 months after treat-
ment with 250 mg of infliximab; therefore, the dose was
slightly increased to 300 mg (VISIT8, at 38 months). Subse-
semiqualitative score [5]. No pulse Doppler abnormality was quently, the BASDAI value and ASDAS-CRP improved to 3.7
detected in the bilateral Achilles tendon insertions, suggesting and 1.9, respectively, after 3 months (VISIT9, at 41 months)
no tendinitis. (Figure 3 and Table 1). No adverse events were observed
Because joint swelling was not mentioned in the medi- during the clinical course of the disease. Additionally, no pro-
cal record, with negative findings in ultrasonography and no gressive radiographic findings, such as syndesmophytes or
fever, juvenile idiopathic arthritis was ruled out. ankylosing, were observed in the lumbar and cervical lateral
Bone scintigraphy and magnetic resonance imaging were views, even after 3 years of treatment with TNF inhibitors.
not used for diagnosis. Regarding related symptoms and find- In summary, the present case was treated in accordance
ings of systemic lupus erythematosus, the patient had no with the ASAS-EULAR recommendations of 2016 [9]. In
erythema such as butterfly rash and discoid, photodermatosis, Phase I, NSAIDs was used. However, BASDAI was more than
and intraoral ulcer. Pleuritis, pericarditis, persistent protein- 4; thus, in Phase II, salazosulfapyridine was used for periph-
uria, and neuropathy were not detected. Haemolytic anaemia, eral joint pain. Moreover, TNF inhibitors were administered.
leukopenia of <4000, decrease of lymphocytes to <1500, and Adalimumab was first used because its administration by
decrease of platelets to <10,000 were not observed. Finally, subcutaneous injection took less time than infliximab admin-
SLE was ruled out because the present case did not fulfil the istration by intravenous injection. However, adalimumab was
SLE classification [6]. insufficient for controlling disease activity of this case; there-
The anteroposterior view of lumbar plain radiographs and fore, in Phase III, infliximab was used as the second TNF
computed tomography (CT) scans of the pelvis showed grade inhibitor.
3 bilateral sacroiliitis (localised bone erosion and osteosclero- The patient and her parent provided written informed
sis) (Figure 1). No remarkable findings were detected in the consent to publish the details of her case.
262 Sakaguchi et al.

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Figure 3. Clinical course before and after the administration of tumour necrosis factor inhibitors. At the initial visit (VISIT1, defined as ‘-1 month’), Bath
ankylosing spondylitis disease activity index (BASDAI) was 8.0 and ankylosing spondylitis disease activity score-C-reactive protein (ASDAS-CRP) was
3.0, respectively. Adalimumab, 40 mg biweekly, was initially administered (VISIT2, ‘0 months’), decreasing BASDAI from 8.0 to 3.1 and ASDAS-CRP from
3.0 to 1.6 after 6 months (VISIT3; time, 6 months). Her ankle pain was not relieved; therefore, up to 80 mg of adalimumab was administered biweekly
(VISIT4; time, 9 months). Subsequently, BASDAI improved from 4.4 to 2.7 and ASDAS-CRP improved from 2.3 to 1.7 (VISIT5; time, 12 months). However,
BASDAI worsened again to 4.8, and ASDAS-CRP worsened to 2.4 on 15 months after administration of 80 mg of adalimumab (VISIT6; time, 24 months).
Hence, the treatment was switched to a regimen of 5 mg/kg (250 mg in total) of infliximab (VISIT6). After 5 months, BASDAI improved to 2.6 and
ASDAS-CRP improved to 1.6 (VISIT7; time, 29 months). However, the values worsened to 6.6 (BASDAI) and 3.1 (ASDAS-CRP) 14 months after
administration of 250 mg of infliximab (VISIT8; time, 38 months); therefore, the dose of infliximab increased slightly to 300 mg. Then, BASDAI and
ASDAS-CRP improved to 3.7 and 1.9, respectively, after 3 months (VISIT9; time, 41 months). The black dashed line shows a BASDAI value of 4.0, and the
black dotted line shows an ASDAS-CRP value of 2.1. Each line shows the border of low disease activity. V; Visit.

Discussion We previously reported a case of a 15-year-old boy with

Based on the case reported herein, JoAS should be considered JoAS, who had a chief complaint of lumbago and for whom
in the differential diagnosis of lumbar pain or systemic axial no peripheral or root joint symptoms were detected. He
and peripheral joint pain from infancy (younger age). was HLA-B27 positive. The administration of prednisolone
There are many diseases related with multiple joint pain (5 mg/day) and salazosulfapyridine (1000 mg/day) was effec-
and lumbar pain in 15-year-old girl that need to be ruled out tive, and the CRP level completely improved in the patient’s
in this case. The differential diagnosis was difficult because clinical course [11].
many diseases are likely candidates, such as juvenile idiopathic In the present case, the age of onset was 15 years, and
arthritis, systemic lupus erythematosus, lumbar disc her- the patient tested negative for HLA-B27 but positive for
nia, lumbar spondylolysis, psoriatic arthritis, palmoplantar HLA-B52 and B62. Radiographic findings indicated Grade 3
pustulosis-associated arthritis, and chronic recurrence mul- bilateral sacroiliac joint involvement, which was supported by
tifocal osteomyelitis. Thus, it took approximately 4 years to CT findings. Compared with that of axial lesions, the onset
diagnose the present case as being a case of JoAS. Nonethe- pattern of JoAS often involves the peripheral joints [2]. In the
less, despite the difficulties in diagnosis, we diagnosed this case present case, although the radiographic findings showed no
as JoAS from the clear findings described above and according abnormalities in the foot or hip, these regions were painful.
to the modified New York diagnostic criteria for AS. Therefore, it is plausible that arthritis was present in the foot
JoAS is defined as AS, and its onset (appearance of symp- and hip joints.
toms) occurs at less than 16 years of age. It is more common in The patient experienced pain, and her BASDAI value
male individuals, and the presence of HLA-B27 is frequently was elevated to 8.0, refractory to NSAIDs, intravenous
observed [2]. The HLA-B27 positivity rate was reported to acetaminophen, and salazosulfapyridine. We did not admin-
be the highest in the group of younger males with JoAS ister methotrexate because methotrexate monotherapy is
[2]. On the other hand, HLA-B27 is less frequently posi- strongly discouraged for the management of sacroiliitis. In
tive in Japanese AS patients (29.2%) than in non-Japanese addition, glucocorticoids are recommended only as bridging
Asian patients (80%) and non-Asian patients (49.9%) [10]. therapy. Intra-articular glucocorticoid injection of sacroiliac
However, there have been no reports on the positivity rate joints is conditionally recommended as an adjunct therapy
of HLA-B52 and HLA-B62 in Japanese patients with AS or [12]. Notably, in the present case, TNF inhibitors were
juvenile AS. administered 4 years after the onset of the disease. The TNF
A case of juvenile-onset ankylosing spondylitis effectively treated with tumour necrosis factor-alpha inhibitor agents 263

inhibitors, adalimumab and infliximab, were both effective in Ethical approval

this case. Not applicable.
Adalimumab and infliximab are approved drugs in the
treatment of JoAS in Japan. Additionally, certolizumab pegol,
etanercept, and golimumab are permitted worldwide. A recent References
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Conflict of interest controlled randomized trial with adalimumab for treatment of
juvenile onset ankylosing spondylitis (JoAS): significant short term
None declared. improvement. Arthritis Res Ther 2012;14:R230.
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Funding in patients with juvenile-onset spondyloarthritis. Arthritis Res Ther
No funding was acquired for this manuscript. 2022;24:187.