Recurrent focal myofasciitis of Behçet syndrome mimics infectious myofasciitis: a case report

Behçet syndrome (BS) is a chronic inflammatory disease with multiorgan manifestations. However, muscular involvement in BS has rarely been reported. Herein, we report the case of a 30-year-old male with BS who had recurring pain and swelling of the lower legs. The patient was administered antibiotics on several occasions as the condition was misinterpreted to be infectious myositis. Magnetic resonance imaging revealed myofascial involvement with focal necrotic lesions, and muscle biopsy revealed acute suppurative myositis with perivascular infiltration of polymorphonuclear leukocytes. His symptoms improved after treatment with corticosteroids. Azathioprine and colchicine therapy was beneficial for preventing further relapse after short-term corticosteroid treatment. Therefore, BS should be considered in the differential diagnosis of focal suppurative myofasciitis.

Comparison of the 2017 EULAR/ACR Criteria with Clinicoserologic Criteria for the Classification of Idiopathic Inflammatory Myopathies in Korean Patients

Purpose@#To investigate correlations between myositis-specific autoantibodies (MSA) or myositis-associated antibodies (MAA) and clinical features, thereby demonstrating the utility of clinicoserologic classification in idiopathic inflammatory myopathies (IIM) patients. @*Materials and Methods@#We conducted a multicenter study of 108 adult patients (age ≥18 years) who were diagnosed with IIM by Peter and Bohan criteria or 2004 European Neuromuscular Centre (ENMC) criteria. Clinical data were obtained by medical record review. Immunoblot assay with Euroline strip (EUROIMMUN, Germany) was performed using the sera of dermatomyositis (DM, n=56), polymyositis (PM, n=45), amyopathic DM (n=5), DM sine dermatitis (n=1), and immune mediated necrotizing myopathy (n=1) patients. Patients were classified based on two classifications: 2017 EULAR/ACR and novel clinicoserologic classification. @*Results@#According to 2017 EULAR/ACR criteria, DM and PM were the most and the second most frequent entities. Overlap myositis was the major entity of IIM, and the frequency of PM was significantly lower when applying clinicoserologic classification criteria. Sixty-nine (63.9%) patients had one or more MSA, and 61 (56.5%) patients had one or more MAA. Interstitial lung disease was closely associated with anti-MDA5 and anti-ARS, and DM-specific skin lesions were frequently observed in patients with antiTIF1γ, anti-SRP, and anti-MDA5. @*Conclusion@#The clinicoserologic criteria based on MSA/MAA positivity could reflect more precise clinical features of IIM. Establishment of a laboratory system routinely available to screen for MSA/MAA status will be beneficial to provide precise diagnosis and proper management of IIM patients.

Comparison of the 2017 EULAR/ACR Criteria with Clinicoserologic Criteria for the Classification of Idiopathic Inflammatory Myopathies in Korean Patients

Purpose@#To investigate correlations between myositis-specific autoantibodies (MSA) or myositis-associated antibodies (MAA) and clinical features, thereby demonstrating the utility of clinicoserologic classification in idiopathic inflammatory myopathies (IIM) patients. @*Materials and Methods@#We conducted a multicenter study of 108 adult patients (age ≥18 years) who were diagnosed with IIM by Peter and Bohan criteria or 2004 European Neuromuscular Centre (ENMC) criteria. Clinical data were obtained by medical record review. Immunoblot assay with Euroline strip (EUROIMMUN, Germany) was performed using the sera of dermatomyositis (DM, n=56), polymyositis (PM, n=45), amyopathic DM (n=5), DM sine dermatitis (n=1), and immune mediated necrotizing myopathy (n=1) patients. Patients were classified based on two classifications: 2017 EULAR/ACR and novel clinicoserologic classification. @*Results@#According to 2017 EULAR/ACR criteria, DM and PM were the most and the second most frequent entities. Overlap myositis was the major entity of IIM, and the frequency of PM was significantly lower when applying clinicoserologic classification criteria. Sixty-nine (63.9%) patients had one or more MSA, and 61 (56.5%) patients had one or more MAA. Interstitial lung disease was closely associated with anti-MDA5 and anti-ARS, and DM-specific skin lesions were frequently observed in patients with antiTIF1γ, anti-SRP, and anti-MDA5. @*Conclusion@#The clinicoserologic criteria based on MSA/MAA positivity could reflect more precise clinical features of IIM. Establishment of a laboratory system routinely available to screen for MSA/MAA status will be beneficial to provide precise diagnosis and proper management of IIM patients.

Predicting Imaging Remission in Rheumatoid Arthritis: a Case-control Ultrasound Study

Background@#To elucidate the achievement rates of imaging remission and to examine the characteristics associated with imaging remission status among patients with rheumatoid arthritis (RA) who have attained clinical remission. @*Methods@#Ninety-seven patients with RA patients who had attained clinical remission, defined by DAS28-ESR < 2.6 were enrolled. Power Doppler ultrasonography (PDUS) was performed on 16 joints and 2 tendons, including the first to third metacarpophalangeal, second and third proximal interphalangeal, radiocarpal (RC), second and third metatarsophalangeal joints, and extensor carpi ulnaris tendons. They were graded based on a dichotomous assessment. The clinical and laboratory data of patients who had attained imaging remission were compared to those of patients who had attained only clinical remission. @*Results@#The imaging remission rate was 51.5% in patients who had attained clinical remission. Forty-seven patients (48.5%) were PDUS positive. Power Doppler was detected most frequently in the right RC joint (n = 40). PDUS positive patients had higher evaluator global assessment (EGA) scores (P < 0.001) than PDUS negative patients. PDUS positive patients also had higher clinical disease activity index and simplified clinical disease activity index scores than PDUS negative patients. Patients who had attained imaging remission had lower pain scores and used nonsteroidal anti-inflammatory drugs less frequently. Patients who had attained imaging remission had higher rheumatoid factor (RF) and anti-cyclic citrullinated peptide levels. A low EGA score was found to be a predictor of imaging remission achievement among patients who had attained clinical remission. @*Conclusion@#Only 51.5% of the patients with RA who had attained clinical remission were also in imaging remission. Patients who had attained imaging remission had lower EGA scores and higher RF levels than patients who had attained only clinical remission.

Impact of sleep quality on clinical features of primary Sjögren's syndrome

BACKGROUND/AIMS@#This study aimed to investigate the inf luence of poor sleep quality on clinical features of primary Sjögren’s syndrome (pSS).@*METHODS@#Sleep quality was cross-sectionally assessed using the Pittsburgh Sleep Quality Index (PSQI), and demographic, clinical, and laboratory data were collected from 115 Korean patients with pSS. The patients completed questionnaires on the European League Against Rheumatism (EULAR) SS Patient Reported Index (ESSPRI), quality of life (EuroQOL five dimensions questionnaire [EQ-5D]), fatigue (fatigue severity score [FSS]), and depression (Beck Depression Inventory [BDI] II]). Symptoms and patient global assessment (PGA) were evaluated with a 100-mm visual analogue scale (VAS). The EULAR sicca score (ESS), ESSPRI, and EULAR SS Disease Activity Index (ESSDAI) were calculated at study enrollment.@*RESULTS@#Fifty-three patients (46.1%) had poor sleep quality and 32.4% of 71 patients without depression were poor sleepers. Poor sleepers had a significantly lower EQ-5D or ESSDAI and a significantly higher FSS, BDI-II, PGA, ESS, ESSPRI, or VAS scores for extra-glandular symptoms than good sleepers. Neutrophil and lymphocyte counts were significantly higher and immunoglobulin G levels tended to decrease in poor sleepers. Additionally, PSQI was negatively correlated with EQ-5D and ESSDAI and positively with ESS, FSS, BDI-II, PGA, VAS scores for their symptoms, and ESSPRI. Multivariate analysis revealed that poor sleep quality remained the independent determinants of the unsatisfactory symptom state (ESSPRI ≥ 5).@*CONCLUSIONS@#Our results showed that poor sleep quality could significantly affect the patient-oriented outcomes and physician-reported activity index of pSS patients through the various effects of sleep quality on the psychological or somatic symptoms and the immune system.

Causes of Chronic Hip Pain Undiagnosed or Misdiagnosed by Primary Physicians in Young Adult Patients: a Retrospective Descriptive Study

BACKGROUND: Hip pain is a common musculoskeletal complaint in general practice. Although comprehensive diagnostic approach on hip pain is mandatory for adequate treatment, un- or mis-diagnosis is not rare in primary care. The aim of this study was to analyze descriptively un- or mis-diagnosed hip pain cases referred from primary care to a tertiary hospital, especially in young adults ≤ 50 years old. METHODS: We retrospectively analyzed a consecutive cohort of 150 patients (≤ 50 years old) with chronic hip pain (≥ 6 weeks), which was not diagnosed or misdiagnosed based on the information provided on the referral form. RESULTS: Overall an average 32 cases/month were referred due to hip pain without a diagnosis or with an incorrect diagnosis. Among them, 150 patients were enrolled in this study and 146 (97.3%) could be allocated to a specific disease by using data from routine clinical practice. Four common final diagnoses were femoroacetabular impingement (FAI) syndrome (55.3%), hip dysplasia (HD, 13.3%), referred pain from the lumbar spine (9.3%), and spondyloarthritis (SpA, 7.3%). In patients with FAI syndrome, 37 (44.0%) had pincer-type FAI and 33 (39.8%) had combined-type. Although the pain site or gender was not tightly clustered, the distribution of final diagnosis was significantly different according to hip pain location or gender. Especially, SpA or HD was not observed in younger women subgroup or elder men subgroup, respectively, when stratified by the mean age of participants. CONCLUSION: Most (> 80%) young patients with hip pain, a difficult issue to diagnosis for many primary physicians, had FAI syndrome, HD, spine lesions, and SpA. This study could give a chance to feedback information about cases with un- or mis-diagnosed hip pain, and it suggests that primary physicians need to be familiar with the diagnostic approach for these 4 diseases.

Acute Eosinophilic Monoarthritis in a Patient with Toxocariasis / 대한내과학회지

Eosinophilic synovial effusion is rarely observed in patients with inflammatory nature of synovial fluid, and the differential diagnosis includes parasitic arthritis. Toxocariasis is the one of the most common forms of helminthiasis worldwide and has been reported as a common cause of peripheral blood eosinophilia in Korea. However, joint involvement has been rarely reported in adults with toxocariasis in the English-language literature. Here, we report the first Korean case of a female presenting with acute monoarthritis with an increased number of eosinophils in the peripheral blood and synovial fluid, who was finally diagnosed with toxocariasis.

A Case of Acute Calcium Pyrophosphate Crystal Arthritis in the Lumbar Facet Joint

Calcium pyrophosphate dihydrate deposition disease most commonly presents with acute arthritis of the peripheral joints. Infrequently, a mass effect of this disease can cause axial symptoms, such as spinal stenosis, radiculopathy, or myelopathy. Herein, we report on the first Korean case of acute arthritis in the lumbar facet joint due to calcium pyrophosphate dihydrate crystal deposition disease. A 73-year-old female presented with acute fever, severe lumbago, and knee arthralgia, 11 days after partial parathyroidectomy. Plain radiographs showed multiple chondrocalcinosis, while a bone scan, computed tomography, and magnetic resonance imaging showed right L5-S1 facet arthritis. In synovial fluid from the facet and knee joints, positively birefringent calcium pyrophosphate dihydrate crystals were observed under polarized light microscopy. Under the diagnosis of acute calcium pyrophosphate dihydrate crystal arthritis (formerly known as 'pseudogout') in the facet joint, an intra-articular triamcinolone injection was administered, which resulted in dramatic improvement of the symptoms within 24 hours.

Three Cases of Spondyloepiphyseal Dysplasia Tarda in One Korean Family

Spondyloepiphyseal dysplasia (SED) tarda is an inherited skeletal arthropathy. Because SED tarda involves the joints and resemble the clinical findings of chronic arthropathies, this disease is frequently misdiagnosed as juvenile idiopathic arthritis (JIA). We report here on three patients (father and his two daughters) in one family with SED tarda. All patients had back pain and polyarthralgia. Their radiographs revealed typical changes for SED tarda including platyspondyly and dysplastic bone changes. This rare disease has major clinical importance in that it is similar with JIA or rheumatoid arthritis.

Isolated Lenticulostriate Artery Aneurysm Rupture in a Patient with Behcet's Disease

Behcet's disease (BD) is characterized by recurrent oro-genital ulcers, skin lesions, and intraocular inflammation, but can also affect various internal organs. Vascular BD usually presents with luminal stenosis, thrombosis, or aneurysm formation in aorta and peripheral arteries. However, intracranial artery involvement has been uncommonly reported in patients with BD and BD cases with lenticulostriate artery aneurysm have been rarely described in the English-language literature. We hereby reported the first case of a Korean BD patient presenting with a ruptured lenticulostriate artery aneurysm, who received medical treatment, and reviewed the literature on reported cases of BD with intracranial aneurysms.

A Case of Mycobacterium szulgai Lung Disease in Patient with Healed Tuberculosis / 결핵및호흡기질환

Mycobacterium szulgai is a rare nontuberculous mycobacterium found in Korea. It is an opportunistic pathogen and is usually isolated from patients with a history of alcoholism, chronic pulmonary disease, or an immunocompromising condition. We present here a case of M. szulgai isolated from a patient with a history of pulmonary tuberculosis. A 54-year-old man was admitted with dyspnea and febrile sensation. He had a history of pulmonary tuberculosis which occurred 30 years earlier and treatment with anti-tuberculosis medication. His chest computed tomography scan showed cavitary consolidation in both upper lungs. A sputum acid-fast bacilli (AFB) smear was positive and anti-tuberculous medication was started. However, a polymerase chain reaction for mycobacterium tuberculosis was negative and anti-tuberculous medication was stopped. M. szulgai was isolated on 3 separate sputum and bronchial wash fluid AFB cultures. He was treated with clarithromycin, rifampicin, and ethambutol. After 1 month, a sputum AFB smear and culture became negative and no additional M. szulgai were isolated during a 16-month treatment.

A Case of Aborted Sudden Cardiac Death during Exercise Associated with an Anomalous Origin of Right Coronary Artery / 대한내과학회지

There are many causes of sudden cardiac arrest. The main cause of sudden cardiac death (SCD) is coronary heart disease. However, the frequency of coronary heart disease is much lower in sudden cardiac arrest occurring below the age of 30-40. Congenital anomalous origin of the coronary arteries is a rare, but well-described, cause of myocardial ischemia and sudden death in young adults. Here, we report the case of a 23-year-old man with sudden cardiac arrest due to ventricular fibrillation associated with an anomalous origin of the right coronary artery. The patient was diagnosed using multi-detector computed tomography and successfully treated with surgical correction.