OBJECTIVE: In patients with interstitial lung disease (ILD) associated with connective tissue disease (CTD), chronic ILD related symptoms may often dominate the clinical picture or precede systemic findings and thus often be seen by a non-rheumatologist. The purpose of this study was to evaluate the importance of rheumatologic consultation during ILD work up. METHODS: We retrospectively reviewed 64 patients with ILD from a single tertiary center for their clinical and laboratory characteristics, rheumatologic consultation status, and result of the consultation. American College of Rheumatology criteria for classification of each connective tissue disease were utilized. Undifferentiated connective tissue disease (UCTD) was classified by pre-specified criteria. RESULTS: A total of 23 (36%) of the ILD patients had associated CTD. Five (8%) patients had underlying CTD before the diagnosis of ILD, whereas 18 (28%) patients were diagnosed with CTD after the rheumatologic consultation. ILD patients with CTD were predominantly female, had significantly more frequent radiographic diagnoses of nonspecific interstitial pneumonia, increased frequencies of high titer antinuclear antibody positivity, and rheumatoid factor positivity. Rheumatologic consultation was referred in 36 (56%) patients. In 18 (50%) of the referred patients, CTD was diagnosed. In 61% of the patients diagnosed with CTD as a result of rheumatologic consultation, changes in therapy occurred. CONCLUSION: A substantial proportion of patients with ILD are found to have an underlying CTD upon evaluation by a rheumatologist. Since ILD associated with CTD mimics idiopathic interstitial pneumonia, rheumatologic consultation may have a significant impact on the clinical care of ILD.
Antibodies, Antinuclear ; Connective Tissue ; Connective Tissue Diseases ; Female ; Humans ; Idiopathic Interstitial Pneumonias ; Lung Diseases, Interstitial ; Referral and Consultation ; Retrospective Studies ; Rheumatoid Factor ; Rheumatology

Amyopathic dermatomyositis is defined by characteristic cutaneous manifestation of dermatomyositis without evidence of muscle involvement. There is no clinical difference between dermatomyositis and amyopathic dermatomyositis. Pulmonary involvement of dermatomyositis is so frequent than aggressive dianostic and therapeutic approach is needed. Early steroid or immunosuppresive treatment in pulmonary involvement of dermatomyositis gets better prognosis. Intravenous immunoglobulin treatment is an effective and safe alternative when the steroid or immunosuppresive treatment is ineffective or intolerable. We are reporting a case of amyopathic dermatomyositis with interstitial lung disease. This patient was improved with intravenous immunoglobulin treatment.
Dermatomyositis* ; Humans ; Immunoglobulins ; Lung Diseases, Interstitial* ; Prognosis

A 63-year old man developed acute monoarthritis in the dorsum of the left foot. Polarized light microscopy of the synovial fluid from his third metatarsophalangeal joint revealed numerous positively birefringent lipid spherules with a maltese cross appearance. Positively birefringent lipid spherules can be found in association with acute, otherwise unexplained arthritis, and may induce synovial inflammation similar to that seen in other types of crystal-induced arthritis. We report a case of acute monoarthritis in which large numbers of positively birefringent lipid spherules were present in a hyperlipidemic diabetic patient.
Acute Disease ; Arthritis/metabolism* ; Arthritis/complications* ; Birefringence ; Case Report ; Crystallization ; Diabetes Mellitus/blood* ; Human ; Hyperlipidemia/complications* ; Lipids/metabolism* ; Male ; Middle Age ; Synovial Fluid/metabolism*

Sciatic nerve can be injured by various mechanism such as compression, traction during surgery, and direct trauma. This case reports a sciatic neuropathy caused by compression due to hematoma occurring after intramuscular injection in the gluteus medius muscle far from the nerve. In order to avoid occurrence of sciatic neuropathy after buttock injection, the injection was made in the upper outer quadrant of the buttock, but sciatic neuropathy occurred. Sciatic neuropathy can be confused with lumbar radiculopathy, so differential diagnosis is important.

Sciatic nerve can be injured by various mechanism such as compression, traction during surgery, and direct trauma. This case reports a sciatic neuropathy caused by compression due to hematoma occurring after intramuscular injection in the gluteus medius muscle far from the nerve. In order to avoid occurrence of sciatic neuropathy after buttock injection, the injection was made in the upper outer quadrant of the buttock, but sciatic neuropathy occurred. Sciatic neuropathy can be confused with lumbar radiculopathy, so differential diagnosis is important.

OBJECTIVES: To evaluate serum Interleukin 8 (IL-8) as a predictor of disease activity in SLE and to provide insight into the potential role of IL-8 in the pathogenesis of SLE. METHODS: Sixty-four paired sera from the 32 SLE patients and 10 healthy control sera were obtained. Serum IL-8 levels were determined by ELISA technique. Tests for other laboratory parameters, such as circulating immune complex (CIC), C3, C4, ANA, anti-dsDNA, Hb, Hct, leukocyte, lymphocyte, platelet and ESR, were performed for every sample coincidently with assessment of clinical disease activity by the Lahita scales. RESULTS: We found that serum IL-8 levels in SLE patients were significantly higher than those of healthy controls. Serum IL-8 levels significantly correlated with clinical disease activity. Serum IL-8 levels correlated with CIC, but it had no correlation with other laboratory parameters. CONCLUSON: These findings suggest that serum IL-8 can be used as a marker of disease activity in patients with SLE. These results may have implication in the pathogenesis of SLE.
Antigen-Antibody Complex ; Blood Platelets ; Enzyme-Linked Immunosorbent Assay ; Humans ; Interleukin-8* ; Interleukins* ; Leukocytes ; Lupus Erythematosus, Systemic* ; Lymphocytes ; Weights and Measures

OBJECTIVES: To assess the clinical features, pathologic findings, postoperative results and the effects of immunosuppressive therapy in patients with Beh et's disease (BD). METHODS: We reviewed the postoperative course of the 9 BD patients who underwent a total of 17 aortic valve replacement procedures with prosthetic valves. RESULTS: Histological examination of the aortic valve commonly revealed diffuse myxoid degeneration (75 percent). Of 17 valve replacement surgeries, 13 surgeries resulted in complications, such as detachment of the prosthetic valve with perivalvular leakage and dehiscence of the sternotomy wound, within an average of 5 months (range from 1 month to 14 months). The rate of prosthetic valve detachment was 76 percent (13 of 17 surgeries). Four of the 9 patients (44 percent) who underwent aortic valve replacement procedures died of heart failure or infection associated with the detachment of the prosthetic valve, and perivalvular leakage within an average of 9 months. Aortic insufficiency associated with dehiscence of the prosthetic valve developed in 11 of 12 surgical cases (92 percent) with a mechanical valve and 2 of 5 surgical cases (40 percent) with tissue valves. Thirteen of 15 surgeries (87 percent) which were not given postoperative immunosuppressive therapy developed complications, while none of 2 surgeries that used postoperative immunosuppressive therapy with prednisolone (1 mg/kg/day) and azathioprine (100 mg/day) had these complications. CONCLUSION: The rates of prosthetic valve detachment in BD involving aortic valve were higher than those of other diseases. Aortic valve involvement was also one of the poor prognostic factors in BD. Intensive postoperative immunosuppressive therapy and surgical methods may be important factors for postoperative results.
Adult ; Aortic Valve/pathology ; Aortic Valve Insufficiency/*etiology/pathology ; Behcet Syndrome/*complications/drug therapy/pathology ; Female ; Heart Valve Diseases/*complications/pathology/surgery ; Heart Valve Prosthesis Implantation/*mortality ; Human ; Immunosuppression ; Male ; Postoperative Complications ; Prosthesis Failure ; Survival Analysis

Cleidocranial dysplasia (CCD) is an autosomal-dominant disease characterized by the delayed closure of cranial sutures, defects in clavicle formation, supernumerary teeth, and delayed tooth eruption. Defects in the Runt-related transcription factor 2 (RUNX2), a master regulator of bone formation, have been identified in CCD patients. The aim of this study was to identify the molecular genetic causes in a CCD family with delayed tooth eruption.The 23-year-old female proband and her mother underwent clinical and radiographic examinations, and all coding exons of the RUNX2 were sequenced. Mutational analysis revealed a single nucleotide deletion mutation (NM_001024630.4 : c.357delC) in exon 3 in the proband and her mother. The single C deletion would result in a frameshift in translation and introduce a premature stop codon [p.(Asn120Thrfs*24)]. This would result in the impaired function of RUNX2 protein, which may be the cause of delayed eruption of permanent teeth in the family.
Clavicle ; Cleidocranial Dysplasia ; Clinical Coding ; Codon, Nonsense ; Core Binding Factor Alpha 1 Subunit ; Cranial Sutures ; Exons ; Female ; Humans ; Molecular Biology ; Mothers ; Osteogenesis ; Sequence Deletion ; Tooth ; Tooth Eruption ; Tooth, Supernumerary ; Transcription Factors ; Young Adult

The purpose of this study was to examine the prevalence of developmental anomalies of permanent lateral incisor and eruption disturbances and analyze the association between two components. Panoramic radiographs of 3984 patients (aged 5 to 13 years) who visited the Department of Pediatric Dentistry of Seoul National University Dental Hospital between November 2016 and October 2017 were screened.
The prevalence of developmental anomalies of permanent lateral incisors was 10.2%. The most common developmental anomalies were congenitally missing teeth(66.1%), followed by peg lateralis(33.5%). The prevalence of eruption disturbances was 16.5%. Among the patient with developmental anomalies of permanent lateral incisors, associated eruption disturbances were appeared on 31.1% of patients. Peg lateralis(p < 0.001), underdeveloped lateral incisors(p < 0.001) and dens invaginatus(p = 0.004) were associated significantly with eruption disturbance of permanent teeth. Eruption disturbance in patients with peg lateralis and dens invaginatus was most prevalent in maxillary canine. For patients with underdeveloped lateral incisor, eruption disturbance of maxillary central incisor was most frequent.
It is important for patients with developmental anomalies of lateral incisors to detect associated eruption disturbance early through regular checkup. Diagnosis and treatment plan in view of such relationships is important in order to treat appropriately at the optimal time.

No abstract available.
Lupus Nephritis*