A 16-year-old patient with juvenile spondyloarthritis and chronic recurrent multifocal osteomyelitis (CRMO) overlap syndrome was admitted to our department for follow-up examinations. She had been hospitalized at the department a few times before.
The patient had a history of joint and back pain and pain in the left buttock and the left thigh when walking since 2011. Physical examination revealed abnormal gait with sparing of the left leg and signs of peripheral arthritis. On ultrasound, peripheral arthritis, including in the left ankle (Fig. 1), left knee, the first metatarsophalangeal joint of the left foot and the right sternoclavicular joint was confirmed. On X-ray of the spine, rotoscoliosis of the thoracic and lumbar spine was found. Laboratory tests revealed moderately elevated inflammatory markers and the presence of the HLA-B27 antigen.
In December 2012, whole-body MRI revealed bone marrow edema in C7, Th5 and Th6 vertebral bodies, Th6 endplate damage, reduction of Th6 body height and bone marrow edema in the sternoclavicular joint, in the left sacroiliac joint, in the diaphyses of both ilia, in the right navicular bone (Fig. 2) with associated edema of soft tissues and of the left lateral malleolus.
On chest X-ray, the following abnormalities were found: pleuro-diaphragmatic adhesions in the left dome of the diaphragm, significant right thoracic scoliosis, irregular contours and subchondral sclerosis of the edge of the sternal end of the right clavicle (Fig. 3). On X-ray of sacroiliac joints bilateral grade 2 inflammation was found.
At the time, the diagnosis of enthesitis-related juvenile idiopathic arthritis (ERA-JIA) was made, i.e. a form of juvenile idiopathic arthritis (JIA) which also belongs to the category of juvenile spondyloarthropathies (diseases in which peripheral arthritis is combined with spondylitis).
Non-steroid anti-inflammatory drug and sulfasalazine therapy was administered (November-December 2012) and pain was reduced. However, due to the patient's parents' failure to report to the department with their child, consecutive stages of treatment were discontinued. In addition, due to a positive result of a tuberculin test, the girl was hospitalized three times (in 2012, 2013 and 2016) at a respiratory disease and tuberculosis treatment center. Latent tuberculosis was diagnosed.
Further in the disease process (2013-2015) the patient had periods of active peripheral arthritis, sacroiliitis and spondylitis.
Further MRI examinations (2015, 2016) revealed a significant progression of the lesions: vertebral body fractures, bone marrow edema in multiple additional locations: in the right iliac bone body, the right iliac acetabulum, the right pubic bone, the left femoral neck and greater trochanter, the head of the right fibula and the first metatarsal bone of the right foot, massive bone marrow edema in both sacroiliac joints (Fig. 4) and soft-tissue edema at the level of the affected joints.
On MRI, chronic recurrent multifocal osteomyelitis (CRMO) associated with JIA was diagnosed, i.e. an overlap syndrome was present.
On ultrasound of the right foot the following abnormalities were noted: a thickened, intensively vascularized synovial membrane in the first metatarsophalangeal joint, a large erosion filled with vascularized synovial membrane on the medial surface of the first metatarsal bone head (Fig. 5), a small effusion under the fifth metatarsal bone head and a thickened synovial membrane of the bursa (submetatarsal bursitis). On left knee ultrasound effusion in the joint cavity was revealed.
Combined Metex + adalimumab therapy was applied and clinical symptoms resolved and inflammation markers returned to normal.
Since April 2017 the parents did not report with the child to the department again and they discontinued the treatment.
In September 2017 the girl was readmitted to the department due to pain recurrence, particularly in the left leg. Diagnostic imaging revealed further progression of the lesions, including multilevel vertebral body fractures and syndesmophytes in the lumbar spine on X-ray (Fig. 6).