The effect of gender on disease activity and clinical characteristics in patients with axial psoriatic arthritis.

OBJECTIVES: In this study, we aimed to evaluate the effect of gender on clinical findings, disease activity, functional status and quality of life in patients with axial involvement in Turkey. METHODS: Patients with PsA who met the CASPAR classification criteria were enrolled consequently in this cohort. Turkish League Against Rheumatism (TLAR)-Network was formed with the participation of 25 centres. The demographic variables, fatigue, diagnostic delay, the beginning of peripheral arthritis, enthesitis, dactylitis and spine involvement, inflammatory low back pain, BASFI, HAQ, HAQ-s, visual analogue scale-pain (VAS-pain), anxiety, depression and disease activity parameters (ESR, DAS28, BASDAI) were recorded. Axial involvement was assessed according to clinical and radiological data according to modified New York (MNYC) or Assessment of SpondyloArthritis international Society (ASAS) criteria. RESULTS: A total of 1018 patients with PsA were included in this study. Of the 373 patients with axial involvement, 150 were male (40.2%) and 223 (59.8%) were female. Spondylitis was detected in 14,7% of men and 21,9% of women in all patients. Pain score (VAS) (p < .002), fatigue (p < .001), ESR (p < .001), DAS28 (p < .001), BASDAI score (p < .001), PsAQoL (p < .001), HAQ score (p < ,01), HAQ-S score (p < .001), anxiety (p < .001), depression (p < .024), FACIT (p < .001) and FiRST (p < .001) scores were statistically significantly worse in women than males with axial PsA. However, quality of life was better (p < .001) and PASI score (p < .005) were statistically worse in male patients than in female patients with axial involvement. CONCLUSION: This study has shown that the burden of disease in axial PsA has significant difference between genders. Disease activity, physical disability, functional limitation, depression and anxiety scores were higher in female patients, while quality of life were better and PASI score were higher in male patients. Therefore, we suggest that new strategies should be developed for more effective treatment of axial PsA in female patients.

Enthesitis and its relationship with disease activity, functional status, and quality of life in psoriatic arthritis: a multi-center study.

Psoriatic arthritis (PsA) is an inflammatory arthritis with distinct phenotypic subtypes. Enthesitis is assigned as a hallmark of the disease, given its significant relations to disease activity and quality of life. Our objective is to evaluate the prevalence of enthesitis and its association with some clinical parameters, particularly quality of life, using data from a national registry. Patients with PsA meeting ClASsification criteria for Psoriatic Arthritis (CASPAR) were enrolled by means of a multi-centre Turkish League Against Rheumatism (TLAR) Network Project. The following information was recorded in web-based case report forms: demographic, clinical and radiographic data; physical examination findings, including tender and swollen joint counts (TJC and SJC); nail and skin involvement; Disease Activity Score-28 for Rheumatoid Arthritis with Erythrocyte Sedimentation Rate (DAS 28-ESR); Bath Ankylosing Spondylitis Disease Activity Index (BASDAI); Maastricht Ankylosing Spondylitis Enthesitis Score (MASES); Psoriasis Area Severity Index (PASI); Bath Ankylosing Spondylitis Radiology Index for the spine (BASRI-s); Health Assessment Questionnaire (HAQ); Bath Ankylosing Spondylitis Functional Index (BASFI); Health Assessment Questionnaire for the spondyloarthropathies (HAQ-s); Psoriatic arthritis quality of Life scale (PsAQoL); Short Form 36 (SF-36); Hospital Anxiety Depression Scale (HADS); Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F); and Fibromyalgia Rapid Screening Tool (FiRST) scores. The patients were divided into two groups, namely with and without enthesitis, based on the triple Likert-type physician-reported statement of 'active enthesitis', 'history of enthesitis' or 'none' in the case report forms. Patients with active enthesitis were compared to others in terms of these clinical parameters. A total of 1130 patients were enrolled in this observational study. Of these patients, 251 (22.2%) had active enthesitis according to the clinical assessment. TJC, HAQ-s, BASDAI, FiRST and PsAQoL were significantly higher whereas the SF-36 scores were lower in patients with enthesitis (p < 0.05). Chronic back pain, dactylitis, and tenosynovitis were more frequent in the enthesopathy group (59.4%/39%, 13.1%/6.5% and 24.7%/3.4%, respectively). Significant positive correlations between the MASES score and the TJC, HAQ, DAS 28-ESR, BASDAI, FiRST and PsAQoL scores, and a negative correlation with the SF-36 score were found. When linear regression analysis was performed, the SF-36 MCS and PCS scores decreased by - 9.740 and - 11.795 units, and the FiRST scores increased by 1.223 units in patients with enthesitis. Enthesitis is an important involvement of PsA with significant relations to quality of life determined with PsAQoL and SF-36 scores. Our study found higher frequency of dactylitis and chronic back pain, and worse quality of life determined with SF-36 and PsAQoL scores in patients with enthesitis.

Management of Psoriatic Arthritis: Turkish League Against Rheumatism (TLAR) Expert Opinions.

OBJECTIVES: This study aims to establish the first national treatment recommendations by the Turkish League Against Rheumatism (TLAR) for psoriatic arthritis (PsA) based on the current evidence. PATIENTS AND METHODS: A systematic literature review was performed regarding the management of PsA. The TLAR expert committee consisted of 13 rheumatologists and 12 physical medicine and rehabilitation specialists experienced in the treatment and care of patients with PsA from 22 centers. The TLAR recommendations were built on those of European League Against Rheumatism (EULAR) 2015. Levels of evidence and agreement were determined. RESULTS: Recommendations included five overarching principles and 13 recommendations covering therapies for PsA, particularly focusing on musculoskeletal involvement. Level of agreement was greater than eight for each item. CONCLUSION: This is the first paper that summarizes the recommendations of TLAR as regards the treatment of PsA. We believe that this paper provides Turkish physicians dealing with PsA patients a practical guide in their routine clinical practice.

Turkish League Against Rheumatism Consensus Report: Recommendations For Management of Axial Spondyloarthritis.

OBJECTIVES: This study aims to update 2011 Turkish League Against Rheumatism SpondyloArthritis Recommendations, and to compose a national expert opinion on management of axial spondyloArthritis under guidance of current guidelines, and implantation and dissemination of these international guidelines into our clinical practice. PATIENTS AND METHODS: A scientific committee of 28 experts consisting of 14 rheumatologists and 14 physical medicine and rehabilitation specialists (one of them also has an immunology PhD) was formed. The recommendations, systematic reviews, and meta-analyses including pharmacologic and non-pharmacologic treatment were scrutinized paying special attention with convenient key words. The draft of Turkish League Against Rheumatism opinion whose roof consisted of international treatment recommendations, particularly the Assessment of SpondyloArthritis International Society/European League Against Rheumatism recommendations was composed. Assessment of level of agreement with opinions by task force members was established through the Delphi technique. Voting using a numerical rating scale assessed the strength of each recommendation. RESULTS: Panel compromised on five basic principles and 13 recommendations including pharmacological and nonpharmacological methods. All of the recommendations had adequate strength. CONCLUSION: Turkish League Against Rheumatism expert opinion for the management of axial spondyloArthritis was developed based on scientific evidence. These recommendations will be updated regularly in accordance with current developments.

Comparison of an epicondylitis bandage with a wrist orthosis in patients with lateral epicondylitis.

BACKGROUND:: Lateral epicondylitis is one of the most common causes of elbow pain. It is generally observed in people between the ages of 40 and 50 years, and the dominant extremity is more frequently affected. Various noninvasive methods are frequently used; however, studies investigating the effectiveness of the orthoses are limited and with controversial outcomes. OBJECTIVE:: To investigate the effectiveness of a hand-wrist resting orthosis and an epicondylitis bandage in lateral epicondylitis. STUDY DESIGN:: Randomized controlled trial. METHODS:: In total, 82 patients diagnosed with unilateral lateral epicondylitis were enrolled into the study. Hand-wrist resting orthosis was applied to 45 patients and epicondylitis bandage was applied to 41 patients. Patients were evaluated before treatment and at 6 weeks after treatment with a Visual Analogue Scale; Disabilities of the Arm, Shoulder, and Hand questionnaire; the Short Form 36; and a determination of hand grasping force. RESULTS:: Both groups were similar in terms of age, body mass index, gender, frequency of lateral epicondylitis on the dominant side, dominant extremity, and duration of complaints ( p > 0.05). A statistically significant recovery was detected in both groups between the pre-therapy values and the values at the sixth week in terms of Visual Analogue Scale scores; Disabilities of the Arm, Shoulder, and Hand questionnarie scores; hand grasping forces; and the Short Form 36 mental and physical component scores ( p < 0.05). However, there were no significant differences detected between the two therapy methods before the therapy and at the sixth week of treatment in all parameter values ( p > 0.05). CONCLUSION:: Our results suggest that both epicondylitis bandage and hand-wrist resting orthosis are effective treatment options for lateral epicondylitis-induced pain, functional status, muscle strength, and quality of life. Furthermore, neither treatment option was superior to the other. Large, randomized studies are necessary for more clear statements. CLINICAL RELEVANCE: Epicondylitis bandage and hand-wrist resting orthosis are equally effective treatment options for lateral epicondylitis-induced pain, functional status, muscle strength, and quality of life.

Turkish League Against Rheumatism (TLAR) Recommendations for the Pharmacological Management of Rheumatoid Arthritis: 2018 Update Under Guidance of Current Recommendations.

OBJECTIVES: This study aims to report the assessment of the Turkish League Against Rheumatism (TLAR) expert panel on the compliance and adaptation of the European League Against Rheumatism (EULAR) 2016 recommendations for the management of rheumatoid arthritis (RA) in Turkey. PATIENTS AND METHODS: The EULAR 2016 recommendations for the treatment of RA were voted by 27 specialists experienced in this field with regard to participation rate for each recommendation and significance of items. Afterwards, each recommendation was brought forward for discussion and any alteration gaining ≥70% approval was accepted. Also, Turkish version of each item was rearranged. Last version of the recommendations was then revoted to determine the level of agreement. Levels of agreement of the two voting rounds were compared with Wilcoxon signed-rank test. In case of significant difference, the item with higher level of agreement was accepted. In case of no difference, the changed item was selected. RESULTS: Four overarching principles and 12 recommendations were assessed among which three overarching principles and one recommendation were changed. The changed overarching principles emphasized the importance of physical medicine and rehabilitation specialists as well as rheumatologists for the care of RA patients in Turkey. An alteration was made in the eighth recommendation on treatment of active RA patients with unfavorable prognostic indicators after failure of three conventional disease modifying anti-rheumatic drugs. Remaining principles were accepted as the same although some alterations were suggested but could not find adequate support to reach significance. CONCLUSION: Expert opinion of the TLAR for the treatment of RA was composed for practices in Turkish rheumatology and/or physical medicine and rehabilitation clinics.

The prevalence of infectious agents in patients with systemic sclerosis.

BACKGROUND/AIM: Systemic sclerosis (SSc) is an autoimmune disease characterized by microvascular injury, excessive extracellular matrix deposition, and fibrosis in the skin and internal organs. Bacterial and viral infectious agents have been suspected to be contributing factors in the development and progression of the pathologic features of SSc. MATERIALS AND METHODS: In this study, 30 SSc patients who were admitted to the rheumatology unit of the Konya Training and Research Hospital and 30 healthy controls were included. The presence of 9 different antibodies (IgM and IgG) against Helicobacter pylori, cytomegalovirus (CMV), Epstein-Barr virus (EBV), and parvovirus B19 were investigated in sera samples obtained from the 60 participants using an enzyme-linked immunosorbent assay method. The characteristics of current and past infections with H. pylori, CMV, EBV, and parvovirus B19 were evaluated by determining the seropositivity of the tested bacterial and viral agents. RESULTS: The prevalences of H. pylori, CMV, EBV, and parvovirus B19 were determined to be higher in patients with SSc than in the control group. CONCLUSION: SSc is associated with a higher rate of certain infections, which deserves further investigation in order to assess the role of infections in disease etiology/pathogenesis.

Femoral cartilage thickness measurements in healthy individuals: learning, practicing and publishing with TURK-MUSCULUS.

BACKGROUND AND OBJECTIVES: Measurement of the femoral cartilage thickness by using in-vivo musculoskeletal ultrasonography (MSUS) has been previously shown to be a valid and reliable method in previous studies; however, to our best notice, normative data has not been provided before in the healthy population.The aim of our study was to provide normative data regarding femoral cartilage thicknesses of healthy individuals with collaborative use of MSUS. METHODS: This is across-sectional study run at Physical and Rehabilitation Medicine Departments of 18 Secondary and Tertiary Centers in Turkey. 1544 healthy volunteers (aged between 25-40 years) were recruited within the collaboration of TURK-MUSCULUS (Turkish Musculoskeletal Ultrasonography Study Group). Subjects who had a body mass index value of less than 30 and who did not have signs and symptoms of any degenerative/inflammatory arthritis or other rheumatic diseases, history of knee trauma and previous knee surgery were enrolled. Ultrasonographic measurements were performed axially from the suprapatellar window by using linear probes while subjects' knees were in maximum flexion. Three (mid-point) measurements were taken from both knees (lateral condyle, intercondylar area, medial condyle). RESULTS: A total of 2876 knees (of 817 M, 621 F subjects) were taken into analysis after exclusion of inappropriate images. Mean cartilage thicknesses were significantly lower in females than males (all p< 0.001). Thickness values negatively correlated with age; negatively (females) and positively (males) correlated with smoking. Men who regularly exercised had thicker cartilage than who did not exercise (all p < 0.05). Increased age (in both sexes) and absence of exercise (males) were found to be risk factors for decreased cartilage thicknesses. CONCLUSION: Further data pertaining to other countries would be interesting to uncover whether ethnic differences also affect cartilage thickness. Collaborative use of MSUS seems to be promising in this regard.

The effect of etanercept on a case of amyloidosis secondary to ankylosing spondylitis: results of 2-year follow-up.

Secondary amyloidosis (type AA) is rarely encountered but can be a significant complication of ankylosing spondylitis (AS) and may lead to proteinuria and renal dysfunction. Anti-tumor necrosis factor-alpha (anti-TNFα) agents may be used to induce clinical remission by suppressing systemic inflammation in secondary amyloidosis. The patient described , with the diagnosis of AS, was diagnosed with secondary amyloidosis, despite treatment with disease modifying anti-rheumatic medication. He developed marked proteinuria, renal dysfunction and low levels of serum albumin. Diagnosis of amyloidosis was confirmed by renal biopsy. During a 2-year treatment period with etanercept, an anti-TNFα agent, a definite improvement was determined in all parameters. This case illustrates that in the treatment of secondary amyloidosis related to AS, etanercept, an anti-TNF α agent, can be considered an effective therapeutic option.

The serum levels of resistin in ankylosing spondylitis patients: a pilot study.

Resistin is a recently described adipokine which is a member of cysteine-rich secretory protein family. Although it has been primarily defined in human adipocytes, it has been identified that its level was higher in mononuclear leukocytes, macrophages, spleen, and bone marrow cells. Because ankylosing spondylitis is an inflammatory disease, it is suspected that upregulation of proinflammatory cytokines is effective in its immunopathogenesis. The aim of our study is to determine the serum resistin levels in patients with AS and to research the relationship with disease activity markers. A total of 30 patients with AS and 30 healthy controls were included in this study. Serum resistin concentrations, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), Bath AS Disease Activity Index (BASDAI) were evaluated. In results resistin levels in ankylosing spondylitis group were significantly higher than in control group. But, there was no correlation between resistin and ESR, CRP, BASDAI. In conclusion, higher serum resistin levels in patients with AS compared to healthy subjects give clues that resistin could have a role in the pathogenesis of AS.

Sacroiliac joint involvement in psoriasis.

Psoriasis is a skin disorder that is associated with arthritis. Sacroiliac joint involvement is considered to be less frequent than the other types of psoriatic arthritis. Additionally, the psoriatic sacroiliitis is considered to be asymmetric in general. We aimed to define the frequency and type of sacroiliac involvement in patients with psoriasis. Patients with psoriasis were included the study. Characteristics of skin, nail and articular involvement were noted. Psoriasis area and severity index was calculated. Antero-posterior pelvic X-rays were obtained and graded by two rheumatologists and a radiologist independently. One hundred and thirty-three patients were included. Thirty-seven of patients (27%) have articular involvement symptomatically. The sacroiliac joint involvement was observed in 34 (26%) of patients. More than one-half of sacroiliac involvement was bilateral while less than one-half was in symptomatic patients regarding sacroiliitis. Fifty-seven percentages of all patients have psoriatic nail involvement. Sacroiliac joint involvement did not show any significant association with psoriatic nail involvement or the severity of skin disease. We found higher frequency of sacroiliac joint involvement and bilateral sacroiliitis in patients with psoriasis. This is in contrast to present information about the association of psoriasis and sacroiliitis. These findings need confirmation by further studies and with more sophisticated techniques such as magnetic resonance imaging.

Validation of the Turkish version of the Quebec back pain disability scale for patients with low back pain.

STUDY DESIGN: A reliability and validity study of a translated, culturally adapted questionnaire. OBJECTIVE: The aims of the present study were to translate the Quebec Back Pain Disability Scale (QDS) into Turkish, to perform its cross-cultural adaptation for Turkish patients with LBP, and to investigate its validity and test-retest reliability. SUMMARY OF BACKGROUND DATA: As a widely used scale in the evaluation of patients with low back pain (LBP), the QDS awaits formal translation and validation into Turkish to achieve an equivalent questionnaire and to allow comparability of data. METHODS: The translation and cross-cultural adaptation of the original questionnaire were performed in accordance with published guidelines. Translation and retranslation of the English version of the QDS was performed blindly and independently by 4 different individuals, and adapted by a team. Hundred patients with LBP were included in our study. The physical examinations were evaluated and the Schober test was assessed for a mobility measurement of the spine. The patients were asked to complete a questionnaire booklet containing the Turkish versions of the modified Oswestry Disability Index (ODI) and QDS, and Visual Analog Scale (VAS) measure of pain. All assessments were repeated 24 hours later for all of the patients. Reliability was evaluated using internal consistency and the intraclass correlation coefficient (ICC). Concurrent validity was measured by comparing the Turkish version of the QDS results to VAS and the Schober test scores. Also, for construct validity, the results of the scale were compared with the Turkish version of modified ODI. RESULTS: The QDS showed excellent test-retest reliability as evidenced by the high ICC for 2 test occasions (ICC = 0.9221, P < 0.000). Also, internal consistency was found to be adequate at both assessments with Cronbach's alpha (0.9405 and 0.9537 at day 0 and 1, respectively). There was a positive correlation between QDS and VAS both for day 0 (r = 0.368; P < 0.000) and for day 1 (r = 0.441; P < 0.000). There was no correlation determined in the comparison of the QDS sum scores with Schober testing for day 0; however, significant negative correlations in these parameters were observed for day 1 (r = -0.249 P = 0.014). Also significantly positive correlations were deter- mined between the Turkish version of the QDS and the Turkish version of the modified ODI for both day 0 and day 1 (r = 0.666, P < 0.000, r = 0.681; P < 0.000, respectively). CONCLUSION: The results of our study show that QDS as a functional status questionnaire has been translated into Turkish without losing the psychometric properties of the original version. The Turkish version of the QDS has good comprehensibility, internal consistency, and validity and is an adequate and useful instrument for the evaluation of disability in patients with LBP.

Positiveness of purified protein derivatives in rheumatoid arthritis patients who are not receiving immunosuppressive therapy.

In recent years, biological agents have emerged as the most popular drugs for the treatment of rheumatoid arthritis (RA). The most frightening side effects of biological agents are infections, with tuberculosis being the leader. On account of the fact that biological agents have been used widespread, a number of algorithms have been developed to search latent tuberculosis. Among these algorithms, the most popular is the purified protein derivatives (PPD) test which is based upon late sensitivity reaction. The objective of this trial is to investigate the relevance of PPD response for the disease in RA patients. A total of 149 subjects (80 patients, 69 healthy), 35 RA patients who have not been treated before, 23 RA and 22 AS patients who are candidates for biological agents and being treated with immunosuppressive drugs, and 69 healthy subjects, have been included in this trial. Swelling joints, number of tender joints, visual analog scale, erythrocyte sedimentation rate, C-reactive protein, and rheumatoid factor were recorded. PPD was performed using the Mantoux method and was measured 72 h later. Statistically significant lower PPD values were determined in untreated RA patients compared to PPD values of treated RA and AS patients and healthy subjects. No correlation was determined between disease activity score 28 activity and PPD values in untreated and treated groups. Similarly, there was no correlation between acute phase reactants and PPD. Lower PPD responses in patients not being treated with immunosuppressive are due to the disease itself, rather than to the drugs being used. It is also important to interpret PPD results in early RA patients with suspicion, when it is intended to start anti tumor necrosis factor therapy.

Acromegalic arthropathy of the hip: a case report.

Acromegaly is a chronic and slowly developing endocrinopathy caused by hypersecretion of growth hormone and consequently of insulin like growth factor-1. The arthropathy in acromegaly can affect both axial and peripheral joints and it may present as the earliest clinical symptom of the disease. Patients with acromegaly may have high prevalence of joint related comorbidity and a reduced self perceived quality of life. An early diagnosis is crucial to obtaining the optimal treatment due to the potential reversibility of the lesions in an early stage. The aim of this case report is to draw attention to the possible articular involvement of acromegaly.

Internal iliac artery pseudoaneurysm: an unusual cause of sciatica and lumbosacral plexopathy.

This report describes an unusual case of lumbosacral plexopathy resulting from internal iliac artery pseudoaneurysm. A 50-yr-old woman presented with multiple penetrating trauma to the thorax, abdomen, and left buttock. Several weeks after the injury, severe sciatica and motor dysfunction developed in her left leg. Progressively worsening pain was followed by left foot drop. An electrodiagnostic evaluation suggested a lower lumbosacral plexopathy. Magnetic resonance imaging showed a hematoma extending adjacent to the left lumbosacral plexus. Computerized tomographic angiography revealed a left internal iliac artery pseudoaneurysm. The pseudoaneurysm was excised surgically. In the early postoperative period, the patient reported total relief of pain. Her motor function recovered gradually over several weeks; however, some residual weakness of foot dorsiflexion persisted. With this case presentation, we underscore that an arterial pseudoaneurysm should be remembered as an etiologic possibility of lumbosacral plexopathy and sciatica, especially in patients with history of iatrogenic or accidental trauma. An increased awareness of this rare cause of sciatica and lumbosacral plexopathy may enable early intervention alternatives.

Hyperostosis frontalis interna in a patient with giant cell arteritis.

Hyperostosis frontalis interna (HFI) is a disorder characterized by progressive symmetric thickening of the inner table of the frontal bone of the human skull. HFI may be accompanied by headache and some neuropsychiatric diseases such as epilepsy and dementia. Giant cell arteritis (GCA), also called temporal arteritis, is a systemic inflammatory vasculitis of unknown etiology that affects medium- and large-sized arteries. It affects elderly people and may result in a wide variety of systemic, neurologic and ophthalmologic complications. As no association of HFI and GCA was encountered in the literature, we found it interesting to report a case with both of these clinical entities.

Superior vena cava syndrome associated with Behcet's disease and 18 months' follow up: a case report.

Behcet's disease is a multisystemic disorder that the main pathological defect is vasculitis and superior vena cava syndrome (SVCS) caused by vasculopathy is a rare manifestation of the disease. Although successful results of endovascular surgery were observed in some cases, medical therapy may have priority among the treatment alternatives because of the high risk of surgery due to the lesional localization of the vessel. In this report, a Behcet's disease case with SVCS and 18 months' follow-up data with medical therapy is presented.

Spontaneous pneumothorax in Wegener's granulomatosis: a case report.

Wegener's granulomatosis (WG) is a multiorgan system disease of unknown etiology characterized by granulomatous inflammation, tissue necrosis, and variable degrees of vasculitis in small- and medium-sized blood vessels. In patients with WG, the musculoskeletal system can be involved before other typical symptoms of the disease. Also in WG, pneumothorax can develop as a rare complication. In this paper, we report a case with WG who had early articular involvement in the absence of classic features of illness and had pneumothorax which resulted from the rupture of the sub-pleural nodule. The aim of this work is to increase the awareness of early articular involvement in WG and the pneumothorax possibility especially in cases with cavitated nodules closure to pleura. This case emphasizes that early diagnosis and treatment is very important in avoiding further complications.

A co-occurrence of sarcoidosis and ankylosing spondylitis: a case report.

Sarcoidosis is an inflammatory multi-system disease, which may cause articular involvement. By causing sacroiliitis, it may mimic spondyloarthropathies. A case with a co-occurrence of sarcoidosis and late-onset of typical ankylosing spondylitis was presented and a possible association between these two entities was discussed.

Legg-Perthes disease-like joint involvement and diagnosis delay in Scheie syndrome: a case report.

Mucopolysaccharidosis (MPS) type I is an inherited disease caused by the absence or malfunctioning of lysosomal enzymes. Three subtypes, based on severity of symptoms, were described, and Scheie syndrome (also called MPS I S) is the mildest form. Although there may be some typical extra-articular manifestations, musculoskeletal involvement may be the only presenting sign in the absence of other symptoms in the patients with less severe forms. The patients with MPS I S, especially in attenuated phenotypes, may be sometimes difficult to recognize for physicians not familiar with the disease. With this case presentation, it is aimed to draw attention to this disease, which could be delayed for the correct diagnosis. An increased awareness of the disease may contribute to more accurate diagnosis, and patients may benefit from early intervention.