Aim to identify outdated terms and make changes to the terminology of spondyloarthritis. Materials and methods. At the first stage of the work, the terms divided into two categories: “outdated” definitions and terms that need to be improved or unified. Subsequently, each member of the Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia (ExSpA) presented by its own definition of the designated term or agreed with the previous term. At the next stage, the existing definitions were put together. After discussion, experts left a term that scored at least 2/3 of the votes. The special opinion of experts was recorded, whose did not coincide with the majority opinion. An open vote was conducted, when defining an “outdated” term with the unanimous decision of all group members, this term was not recommended for further clinical use. Results. The work carried out allowed us to identify a number of terms that are not recommended for use in clinical practice. Number of terms are defined, which should be used when discussing the problem of spondyloarthritis. Conclusion. The Expert Group of Spondyloarthritis at the Association of Rheumatologists of Russia suggests using or, accordingly, not using a number of terms and their definitions in clinical practice.
BackgroundFibrodysplasia ossificans progressive (FOP) is ultra-rare autosomal dominant inherited genetic disorder which is caused by mutation in ACVR1 gene and provokes severe heterotopic ossification (HO) as a «second skeleton disease». Our long-term experience of the largest group of FOP patients in single Russian center makes possible to interpretate clinical findings and imaging as the evidences that FOP may belong to special kind of axial spondyloarthritis. Because of new bones formation process X-Ray and CT scans recognize as the main imaging methods for the FOP. But MRI method may present important information for the understanding of the disease origin in FOP.ObjectivesTo analyze the MRI findings in FOP patients in relation to clinical manifestations, and therapy options.MethodsThe study was based on retrospective and prospective observation and included 40 pts (21 males; 19 females). Diagnosis of FOP was established on typical clinical features and detecting mutation. X-ray, CT and MRI were performed in most patients excluding the severe disabled status of FOP. Some patients failed to perform MRI due to the presence of severe skeletal deformities or artificial metal constructions (because of previous orthopedic operations). Blood assessment has never shown specific elevated markers.ResultsAmong of all 40 FOP patients MRI of sacroiliac joints was performed in 14 patients. Сhronic sacroiliitis was detected in 10/14 pts, including active sacroiliitis in STIR regimen in 6/14 pts by. Also 7/14 pts have MRI evidence of severe coxitis (see Figure 1). This imagines findings and other features of axial involvement (X ray imagines of ankylosis of the facet joints and vertebral bodies by the type of syndesmophytes) allowed us to establish alternative diagnosis of juvenile spondyloarthritis (JAS) in 7 pts and juvenile idiopathic arthritis (JIA) in 5 cases. It was needed for the legal possibility to administer tofacitinib (TOFA) in 12 pts (the youngest pt of 2 y.o, the oldest – 19 y.o) with strong uncontrolled progression of FOP. The dosage of TOFA was up to 5 mg twice a day. We noticed that all 12 pts have severe coxitis with synovia proliferation (with MRI and ultrasound evidence). Duration of TOFA therapy is from 2 to 25 months. Drug tolerance was good in all pts, no AE were registered. New nodes formation immediately stopped in most pts, significant improvement of motions was observed in 10 (83%) pts. Also, we noticed fast regression of sacroiliitis and coxitis activity by MRI in 5 (42%) pts under TOFA therapy. All other patients didn`t have repeated MRI examination yet. So MRI seems to be important method to confirm the anti-inflammatory effects for sacroiliitis and coxitis signs in patients with FOP. It seems to expect that inflammation control may prevent the sacroiliac and hip ankyloses. In spite of the absence of new HO in our 16 y.o. pt, we found continuous intraskeletal ossification between C3-C4, C5-C7 vertebral bodies, facet joints that leads to subtotal stenosis of spinal canal without any neurological symptoms. Because of «protection effect» of external heterotopic ossification severe neurological damage was not developed.Figure 1.Two different patients with severe FOP and similar evidences of sacroiliitis and bilateral hips synovitis by MRIConclusionAccording to MRI findings, hip damage was characterized for all ages of FOP patients. Sacroiliitis we found mostly in patients older 10 y.o. Based on our experience of JAK-inhibitor therapy we can suggest about positive effect and advantages of TOFA over other therapy approaches. Dynamics of MRI imaging may be good option for the confirmation of therapeutic anti-inflammatory effects.Disclosure of InterestsNone declared
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