Takayasu's arteritis (TA), a granulomatous vasculitis, affects the aorta and its major branches. Glucocorticoids are an effective treatment for patients with active TA, but some patients fail to achieve or maintain remission with the conventional therapy, and side effects resulting from long-term glucocorticoid therapy are potentially serious. Anti-tumor necrosis factor-alpha agents, such as infliximab, may be efficient in patients with refractory TA. We report on a 24-year-old female patient with refractory TA who was treated successfully with infliximab. Clinical remission was induced as determined by repeated 18F-fluoro-2-deoxy-D-glucose positron emission tomography scans combined with assay of serological inflammatory markers.
Antibodies, Monoclonal ; Aorta ; Female ; Glucocorticoids ; Humans ; Necrosis ; Positron-Emission Tomography ; Takayasu Arteritis* ; Vasculitis ; Young Adult ; Infliximab

Objective: The study aimed to ascertain the clinical manifestations of inflammatory arthritis accompanying tuberculosis (TB) for the differential diagnosis. Methods: We retrospectively reviewed patients with active TB who presented with inflammatory arthropathy at Seoul Medical Center. Among 2,872 patients with active TB infection, 47 had inflammatory arthropathy: 14 had crystal-induced arthropathy; 12, TB arthritis; 12, Poncet’s disease (PD); 8, Rheumatoid arthritis (RA); and 1, septic arthritis. The clinical characteristics and laboratory and radiographic findings of each group were analyzed. Results: In TB arthritis, weight-bearing joints were more commonly affected than the elbow and wrist joints. When compared to TB arthritis, PD demonstrated a significantly higher proportion of polyarthritis and involved both large and small-to-mediumsized joints. The duration of arthritis symptoms after anti-TB treatment was significantly shorter in patients with PD (56 days vs. 90 days, p=0.028). When compared to PD, RA flares during active TB infection involved only small-to-medium-sized joints rather than a mixed distribution (62.5% vs. 16.7%, p=0.035). Patients with PD more commonly had fever at onset and showed a good response to nonsteroidal anti-inflammatory drugs alone or were in remission within 3 months after anti-TB treatment. The presence of rheumatoid factor or anti-cyclic citrullinated peptide and radiographic progression after 12 months was frequently observed in patients with RA flares. Conclusion The differential diagnosis of inflammatory arthritis accompanying active tuberculosis infection is challenging. Comprehensive history taking and physical examination, synovial fluid analysis, and a high level of clinical suspicion are essential to avoid delayed diagnosis and to reduce the significant morbidity involved.

OBJECTIVES: To investigate the clinical findings upon initial diagnosis and extraglandular manifestations in Korean patients with primary Sjogren's syndrome (pSS). METHODS: We collected clinical and laboratory data from 238 pSS patients enrolled at Seoul National University Hospital, Seoul National University Bundang Hospital and Seoul Medical Center from March 2011 to December 2014. All patients met the American-European Consensus Group criteria for pSS. RESULTS: Upon initial diagnosis, sicca symptoms (xerophthalmia or xerostomia) as the chief complaint were only observed in 129 (54.2%) pSS patients, while extraglandular manifestation was more common as the chief complaint in male patients or those with younger age (<40 years) than female or older patients (both p<0.05). Extraglandular manifestations were found in 178 (74.8%) patients, with musculoskeletal manifestations being most common (53.8%). Peripheral neuropathy in pSS patients was associated with Raynaud phenomenon and elevated serum total immunoglobulin G (IgG) levels (both p<0.05). Serum beta2-microglobulin (beta2-M) levels were significantly correlated with European League against Rheumatism (EULAR) Sjogren's syndrome disease activity index, erythrocyte sedimentation rate and serum total IgG (all p<0.001), and were higher in patients with extraglandular manifestations than those without (p<0.05). Serum C3 levels were decreased in patients with extraglandular manifestation, compared to those without (p<0.05). Malignant lymphoma was found in Korean pSS patients (1.7%) and associated with elevated serum beta2-M levels (p<0.0001). CONCLUSION: Extraglandular manifestations were common in pSS patients and may be a diagnostic tool for male or younger pSS patients. Serum beta2-M levels can be useful markers for monitoring pSS patients.
beta 2-Microglobulin ; Consensus ; Diagnosis ; Erythrocyte Indices ; Female ; Humans ; Immunoglobulin G ; Lymphoma ; Male ; Peripheral Nervous System Diseases ; Raynaud Disease ; Rheumatic Diseases ; Seoul ; Sjogren's Syndrome*

OBJECTIVE: To quantify autonomic dysfunction in fibromyalgia patients compared to healthy controls using heart rate variability (HRV). METHODS: Sixteen patients with fibromyalgia and 16 healthy controls were recruited in this case control study. HRV was measured using the time-domain method incorporating the following parameters: total heartbeats, the mean of intervals between consecutive heartbeats (R-R intervals), the standard deviation of normal to normal R-R intervals (SDNN), the square root of the mean squared differences of successive R-R intervals (RMSSD), ratio of SDNN to RMSSD (SDNN/RMSSD), and difference between the longest and shortest R-R interval under different three conditions including normal quiet breathing, rate controlled breathing, and Valsalva maneuver. The severity of autonomic symptoms in the group of patients with fibromyalgia was measured by Composite Autonomic Symptom Scale 31 (COMPASS 31). Then we analyzed the difference between the fibromyalgia and control groups and the correlation between the COMPASS 31 and aforementioned HRV parameters in the study groups. RESULTS: Patients with fibromyalgia had significantly higher SDNN/RMSSD values under both normal quiet breathing and rate controlled breathing compared to controls. Differences between the longest and shortest R-R interval under Valsalva maneuver were also significantly lower in patients with fibromyalgia than in controls. COMPASS 31 score was negatively correlated with SDNN/RMSSD values under rate controlled breathing. CONCLUSION: SDNN/RMSSD is a valuable parameter for autonomic nervous system function and can be used to quantify subjective autonomic symptoms in patients with fibromyalgia.
Autonomic Nervous System ; Case-Control Studies ; Fibromyalgia* ; Heart Rate* ; Heart* ; Humans ; Parasympathetic Nervous System ; Respiration ; Sympathetic Nervous System ; Valsalva Maneuver

Peripheral nervous system dysfunction is a rare complication in Henoch-Schonlein purpura, but it tends to recover spontaneously without treatment. A 78-year-old man who had ankylosing spondylitis presented with Henoch-Schonlein purpura associated with progressive sensorimotor polyneuropathy. He was diagnosed with chronic inflammatory demyelinating polyneuropathy, which did not improve despite intravenous immunoglobulin therapy. We describe a case of Henoch-Schonlein purpura, accompanied by chronic inflammatory demyelinating polyneuropathy in a patient with ankylosing spondylitis.
Aged ; Humans ; Immunization, Passive ; Peripheral Nervous System ; Polyneuropathies ; Polyradiculoneuropathy ; Purpura, Schoenlein-Henoch ; Spondylitis, Ankylosing

A 46-year-old man was admitted to our hospital because of a fever of unknown origin. The patient had a skin rash, arthralgia, and hepatosplenomegaly. Laboratory tests showed leukemoid leukocytosis, a high serum ferritin level (31,200 ng/mL), and abnormal liver function tests. He was initially diagnosed with adult-onset Still's disease (AOSD). However, imaging studies incidentally detected a cold abscess involving the 11th thoracic vertebra. A biopsy of the abscess revealed histologically granulomatous caseous necrosis, positive for M. tuberculosis by polymerase chain reaction. We present a patient with musculoskeletal tuberculosis (TB) that clinically mimicked AOSD. In TB-endemic countries, extrapulmonary TB should be included in the differential diagnosis of AOSD.
Abscess ; Arthralgia ; Biopsy ; Diagnosis, Differential ; Exanthema ; Ferritins ; Fever of Unknown Origin ; Humans ; Leukocytosis ; Liver Function Tests ; Middle Aged ; Mycobacterium tuberculosis ; Necrosis ; Polymerase Chain Reaction ; Spine ; Still's Disease, Adult-Onset* ; Tuberculosis*

Polyarteritis nodosa (PAN) has a broad spectrum of clinical presentation, since it affects small and medium-sized muscular arteries with microaneurysm formation, aneurysmal rupture with hemorrhage, thrombosis, and, consequently, organ ischemia or infarction. Although skeletal muscle involvement is well documented in patients with PAN, it can mimic more common diseases, and cause confusion and delays in diagnosis. PAN muscular involvement may have limited or early systemic forms with a benign course and excellent clinical response to corticosteroid therapy. Herein, we describe the clinical course and outcome of four unusual cases of PAN manifested by acute onset of pain and pitting edema in both lower extremities; in addition, we reviewed the relevant literature.
Aneurysm ; Arteries ; Diagnosis ; Edema* ; Hemorrhage ; Humans ; Infarction ; Ischemia ; Lower Extremity* ; Muscle, Skeletal ; Musculoskeletal Pain ; Polyarteritis Nodosa* ; Rupture ; Systemic Vasculitis ; Thrombosis

Primary Sjogren's syndrome (pSS) is characterized by chronic inflammation and dysfunction in exocrine organs; however, it also has protean clinical features, including neuropsychiatric symptoms. A major neurological manifestation is peripheral neuropathy and involvement of the central nervous system is uncommonly described in pSS. A 52-year-old female was admitted because of depression, dysarthria, gait abnormality, and memory disturbance, which had developed over two months, and was diagnosed as pSS. She was treated successfully with high-dose glucocorticoid and cyclophosphamide pulse therapy without recurrence during the follow-up period of two years. Herein, we describe the first Korean case of pSS presenting with rapidly progressive cognitive impairment along with a review of the literature.
Central Nervous System ; Cyclophosphamide ; Depression ; Dysarthria ; Female ; Follow-Up Studies ; Gait ; Humans ; Inflammation ; Memory ; Middle Aged ; Neurologic Manifestations ; Peripheral Nervous System Diseases ; Recurrence ; Sjogren's Syndrome*

BACKGROUND/AIMS: It is unknown whether pulmonary rehabilitation (PR) is an effective intervention to manage coal-worker pneumoconiosis (CWP). We evaluated the efficacy and safety of an individualized PR program in 53 patients with CWP hospitalized in two medical institutions. METHODS: The PR program consisted of upper and lower extremity exercises to improve exercise endurance and skeletal musculoskeletal strength. All subjects performed treadmill and ergometer exercise with steady loading weights three times/week for 12 weeks. The following tests were performed before and after the study to investigate the efficacy of the PR program: modified Borg scale, pulmonary function test, mid-thigh circumference, maximum muscular strength, 6-min walk distance (6MWD), and the St. George's Respiratory Questionnaire (SGRQ), Korean version. RESULTS: Forty patients (75.5%) completed their PR programs. They improved significantly on the modified Borg scale, mid-thigh circumference, maximum muscular strength, 6MWD (all p < 0.000), and SGRQ (p = 0.007); however, no significant improvement was observed on the pulmonary function test. A significant improvement in dyspnea (p = 0.004) and 6MWD (p = 0.002) was observed in 12 patients with forced expiratory volume in 1 sec < 60%. The PR program with smoking cessation resulted in significantly more improvement on the 6MWD (p < 0.0001) and the SGRQ score (p = 0.002), as compared to those of patients who did not quit smoking. CONCLUSIONS: Our results show that an individualized 12-week PR program improves exercise capacity and quality of life for patients with CWP.
Dyspnea ; Exercise ; Exercise Therapy ; Forced Expiratory Volume ; Humans ; Lower Extremity ; Pneumoconiosis* ; Quality of Life ; Surveys and Questionnaires ; Rehabilitation* ; Respiratory Function Tests ; Smoke ; Smoking ; Smoking Cessation ; Weights and Measures