Bullous pemphigoid is an autoimmune, subepidermal blistering disease commonly seen in the elderly. It is known as autoimmune bullous dermatoses with an estimated prevalence of 1 in 40,000 and shows no gender or racial predilection. It has an autoimmune nature with immunoglobulin (Ig) G and/or C3 complement targeting hemidesmosomal antigens located in the epidermal basement membrane zone. Drug-induced pemphigoid, although rare, can occur in patients taking penicillamine, furosemide, captopril, penicillin, or sulfasalazine, but infliximab-induced pemphigoid has not been reported. We experienced a case of infliximab-induced pemphigoid in 54-year-old woman with a 17-year history of rheumatoid arthritis.
Aged ; Antibodies, Monoclonal ; Arthritis, Rheumatoid ; Basement Membrane ; Blister ; Captopril ; Complement C3 ; Female ; Furosemide ; Humans ; Infliximab ; Immunoglobulins ; Middle Aged ; Pemphigoid, Bullous ; Penicillamine ; Penicillins ; Prevalence ; Skin Diseases, Vesiculobullous ; Sulfasalazine

Neuromyelitis optica (NMO) is an idiopathic, severe inflammatory demyelinating disease of the central nervous system targeting optic nerves and the spinal cord. It is characterized by acute bilateral visual loss (optic neuritis), acute transverse myelitis, and tends to spare brain early in the disease course. NMO can occur as an isolated condition or secondary to infection, toxin exposure, and autoimmune disease including systemic lupus erythematosus (SLE), sarcoidosis, and Behcet's disease. We experienced a case of SLE with myelitis and recurrent optic neuritis in a 28-year-old woman who presented with recurrent visual disturbance and sudden onset of paraplegia, and report here on this case along with a review of the relevant literature.
Autoimmune Diseases ; Brain ; Central Nervous System ; Demyelinating Diseases ; Female ; Humans ; Lupus Erythematosus, Systemic ; Myelitis ; Myelitis, Transverse ; Neuromyelitis Optica ; Optic Nerve ; Optic Neuritis ; Paraplegia ; Sarcoidosis ; Spinal Cord

Infliximab, which is indicated for refractory rheumatoid arthritis (RA), ankylosing spondylitis (AS) and Crohn's disease, has cutaneous adverse events including skin rash, urticaria, pruritus, and lupus-like eruption. Psoriasis induced by infliximab is very rare. In Korea, it is infrequently reported in Crohn's disease or RA and never reported in AS. We encountered a case of psoriasis induced by infliximab treatment for AS, and report here on this case along with a review of the relevant literature.
Arthritis, Rheumatoid ; Crohn Disease ; Exanthema ; Korea ; Pruritus ; Psoriasis* ; Spondylitis, Ankylosing* ; Urticaria ; Infliximab

OBJECTIVE: Cardiac manifestations are well recognized complication of ankylosing spondylitis (AS). They include aortic incompetence, conduction defects, mitral valve disease, pericarditis and cardiomyopathy. There was one study to evaluate the change of aortic elasticity in AS patient and the association between the aortic strain and duration of AS, Bath Ankylosing Spondylitis Disease Activity Index (BASDAI). We designed this study to determine whether aortic elasticity changes in Korean AS patients and is associated with the duration of AS or BASDAI. METHODS: 18 AS patients without cardiovascular involvement and 18 sex and age- matched healthy subjects were enrolled in the study. Aortic strain and distensibility was calculated from aortic diameters measured by echocardiography and blood pressure measured by sphygmomanometry. RESULTS: The mean aortic strain and mean aortic distensibility in AS group indicated that there was not any correlation with those of control group, based on the statistical analysis. Moreover, there was no statistical correlation between the means of aortic strain, aortic distensibility and the duration or BASDAI of AS. CONCLUSION: In patients with AS without cardiac involvement, the aortic elasticity was not decreased than that of control group, and aortic strain and distensibility were not correlated with the duration or BASDAI of AS.

OBJECTIVE: Although previous trials suggested a relationship between neutrophil-lymphocyte ratio (NLR), platelet-lymphocyte ratio (PLR), and systemic inflammatory response, clinical utility of NLR and PLR in rheumatoid arthritis (RA) is not well defined. This study was conducted to assess the efficiency of NLR and PLR as an inflammatory index in patients with RA. METHODS: A total of 107 patients with newly diagnosed RA who had never used steroid and a control group of 50 age- and gender-matched healthy subjects whose high sensitive C-reactive protein (hsCRP) was within normal range were included. Those with cerebrovascular diseases, diabetes, malignancies, or any cardiovascular diseases were excluded from both groups. The patients were divided into two groups according to the Disease Activity Score of 28 joints (DAS28). Group 1 included patients with a DAS28 score of 3.2 and lower (low disease activity) and group 2 included patients with a score higher than 3.2 (moderate to high disease activity). RESULTS: NLR and PLR in the patient group were 2.99±2.04, 170.90±86.49, significantly higher than that of the control group. NLR and PLR in group 2 were 4.16±2.50, 225.23±93.21, significantly higher than those of group 1 patients (2.26±1.22, 137.15±61.92). NLR and PLR both showed correlation with rheumatoid factor, hsCRP, serum albumin, Korean Heath Assesment Questionnaire, and DAS28. CONCLUSION: These data showed a positive correlation between NLR or PLR level and RA disease activity, suggesting that NLR or PLR can be used as an additional inflammatory marker in patients with RA.
Arthritis, Rheumatoid* ; C-Reactive Protein ; Cardiovascular Diseases ; Equidae ; Humans ; Joints ; Reference Values ; Rheumatoid Factor ; Serum Albumin

Gitelman syndrome is a condition caused by a mutation of the thiazide sensitive Na-Cl cotransporter gene on the distal convoluted tubule. It results in a variety of clinical features, including hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis. It is often diagnosed in asymptomatic adults presented with unexplained hypokalemia; however, it is sometimes associated with muscular cramps, numbness, fatigue, weakness, or paralysis. We experienced a case of rheumatoid arthritis accompanied by Gitelman syndrome, presented with hand tremor. We diagnosed her using renal clearance study and genetic analysis. Here, we report our experiences regarding this case along with a literature review.
Adult ; Alkalosis ; Arthritis, Rheumatoid* ; Fatigue ; Furosemide ; Genetic Testing ; Gitelman Syndrome* ; Hand ; Humans ; Hypesthesia ; Hypokalemia ; Muscle Cramp ; Paralysis ; Solute Carrier Family 12, Member 3 ; Thiazides ; Tremor

Neurogenic bladder in systemic lupus erythematosus (SLE) has been considered to occur rarely. Myelopathy, cyclophosphamide treatment, vasculitis have been implicated as the causes of the bladder pathology in patients with SLE. In this report, we describe a 46-year- old female with SLE who simultaneously combined with the neurogenic bladder. The diagnosis of neurogenic bladder, attributed to peripheral neuropathy, was made on the basis of cystometrography and clinical symptom. Steroid therapy couldn't improve the clinical manifestation of neurogenic bladder despite the amelioration of the other lupus symptom.
Cyclophosphamide ; Diagnosis ; Female ; Humans ; Lupus Erythematosus, Systemic* ; Pathology ; Peripheral Nervous System Diseases ; Spinal Cord Diseases ; Urinary Bladder ; Urinary Bladder, Neurogenic* ; Vasculitis

OBJECTIVE: Fibromylagia (FM) is a rheumatic disorder characterized by widespread bodily pain, tenderness, and fatigue. It has a major impact on the quality of life including the quality of marital life. The objectives of this study were to assess the quality of marital life in patients with FM in Korea and to identify the correlation of the quality of marital life with disease severity. MEHTODS: Subjects were the patient group, composed of 50 married patients with FM, and a comparison group composed of 50 healthy married adults with similar demographic characteristics. The quality of marital life was measured using the Korean-Marital Satisfaction Inventory (K-MSI). The K-MSI consists of two valid scales, one global affective scale, and eleven additional scales measuring specific dimensions of relationship distress. The clinical parameters were assessed by Korean Fibromyalgia Impact Questionnaire (KFIQ) and tender point, respectively from 50 patients with FM. RESULTS: Of the 50 subjects, 48 (96%) were women with mean (+/-SD) age of 52.6(+/-8.8) years. The mean (+/-SD) duration of disease was 25.7 (+/-19.8) months. The mean (+/-SD) scores of KFIQ and tender point count were 52.9 (+/-14.9) and 13.1 (+/-1.6), respectively. The T-score of most scales of K-MSI of FM patients were higher than that of healthy controls (p<0.01). The scores of KFIQ were positively correlated with the eight scales of K-MSI and negatively correlated with one scale (Role orientation) of K-MSI. The tender point count was positively correlated with only one of scale (Conflict with in-law) of K-MSI. CONCLUSION: The results suggest that the quality of marital life in Korean patients of FM is lower than healthy subjects. In a simple correlation analysis, KFIQ was a meaningful variable correlated with the quality of marital life.
Adult ; Fatigue ; Female ; Fibromyalgia* ; Humans ; Korea ; Quality of Life ; Surveys and Questionnaires ; Weights and Measures

Diffuse idiopathic skeletal hyperostosis (DISH) is a condition characterized by the calcification and ossification of soft tissue, and can lead to severe cervical kyphosis, presenting with a spectrum of physical complaints including dysphagia, hoarseness, stridor, aspiration pneumonia, and dyspnea due to airway compromise. Restrictive ventilatory impairment is very rare. We encountered a 73-year-old man with DISH presenting with progressive dysphagia and dyspnea over a few months. The symptoms were evaluated with a video fluoroscopy swallowing study and pulmonary function tests (PFT). The PFT revealed restrictive ventilatory impairment. A neck magnetic resonance imaging (MRI) study showed anterior cervical osteophytes causing upper airway compromise and compression of the esophagus. Osteophytes were removed surgically and the patient improved clinically. Here, we describe the case with a literature review.
Aged ; Deglutition ; Deglutition Disorders* ; Dyspnea ; Esophagus ; Fluoroscopy ; Hoarseness ; Humans ; Hyperostosis, Diffuse Idiopathic Skeletal* ; Kyphosis ; Magnetic Resonance Imaging ; Neck ; Osteophyte* ; Pneumonia, Aspiration ; Respiratory Function Tests ; Respiratory Sounds

BACKGROUND: Aberrations of cardiovascular regulation have been reported in patients who suffer with fibromyalgia (FM). Abnormalities of the cardiovascular autonomic regulation, as well as the correlation between coronary heart disease and depression, have been considered to be the causative factors. The clinical features of transient left apical ballooning syndrome with the patients under acute stress have been clearly described, but the effect of chronic stress such as FM on the myocardium is unknown. We investigated the cardiac strain in FM patients by strain imaging with using the 2D grayscale images, and we quantified the regional myocardial deformation properties. METHODS: We investigated 30 consecutive postmenopausal women (mean age: 48+/-8 years) who satisfied the criteria for fibromyalgia with atypical chest pain by performing standard and 2-dimensional strain echocardiography (2DS). Those patients with hypertension, coronary heart disease or diabetes were excluded. The global and segmental longitudinal deformation parameters of the LV from 3 apical views were analyzed, and the patients underwent a manual tender point survey for determining the number of tender points and tender point counts, and the patients completed the fibromyalgia impact questionnaire (FIQ), the brief fatigue inventory (BFI), and Beck depression inventory (BDI). RESULTS: The global longitudinal LV strain was significantly reduced in the FM patients with a high FIQ score (>40) as compared to the patients with a low FIQ score (-18.61% vs. -22.72%). Also, both the global and segmental longitudinal LV strains were negatively associated with fatigue or the tender point counts. However, there was no significant association between depression and the LV strain. CONCLUSION: This study showed the reduced myocardial longitudinal deformation in FM patients. This suggested that strain imaging is a feasible approach to assess the regional ventricular function in FM patients.
Chest Pain ; Coronary Disease ; Depression ; Echocardiography ; Fatigue ; Female ; Fibromyalgia ; Humans ; Hypertension ; Myocardium ; Surveys and Questionnaires ; Sprains and Strains ; Takotsubo Cardiomyopathy ; Ventricular Function