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

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

Focal dystonia is a neurological condition affecting a muscle or group of muscles in a specific part of the body, leading to involuntary muscular contractions. This condition is often treated with medications including muscle relaxants and injections of botulinum toxin. However, some cases do not respond to normal modes of treatment. Deep brain stimulation (DBS) can be a therapeutic option for patients who are resistant to medical treatment. We report a case of fibromyalgia accompanied by focal hand dystonia, where unilateral DBS improved the patient's focal dystonic movement. We also present a review of the relevant literature.
Botulinum Toxins ; Deep Brain Stimulation* ; Dystonia* ; Dystonic Disorders ; Fibromyalgia* ; Hand ; Humans ; Muscle Contraction ; Muscles

Thrombotic thrombocytopenic purpura (TTP) is a blood coagulation disorder that damages numerous organs, including the kidney, heart and brain. Features indicative of TTP include thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, kidney failure and fever. Infections, toxins, pregnancy and, rarely, autoimmune diseases are all known to be associated with TTP. We encountered a rare case of rheumatoid arthritis accompanied by TTP following tympanoplasty. The patient's confusion, thrombocytopenia and renal failure all improved after plasmapheresis and high-dose glucocorticoid therapy, but she eventually expired due to sepsis. We report on this case herein and also review the relevant literature.
Anemia, Hemolytic ; Arthritis, Rheumatoid* ; Autoimmune Diseases ; Blood Coagulation Disorders ; Brain ; Fever ; Heart ; Kidney ; Neurologic Manifestations ; Plasmapheresis ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic* ; Renal Insufficiency ; Sepsis ; Thrombocytopenia ; Tympanoplasty

OBJECTIVE: Although a series of trials support that rheumatoid arthritis (RA) is associated with increased atherosclerosis, the link between microvascular structural changes and the disease activity of RA has not been clarified. We measured changes in the retinal microvasculature using fundus fluorescein angiography (FAG) and investigated the association between the retinal vasculature and clinical parameters of RA. METHODS: Seventy-five RA patients and sixty healthy control were included. Morphometric and quantitative features in the capillary images including retinal vascular signs and vessel diameters were measured with fundus photography and FAG. RA activity was assessed based on high sensitivity C-reactive protein (hsCRP), disease activity score with 28 joints (DAS 28), and health assessment questionnaire (HAQ). RESULTS: Central retinal arteriolar equivalents (CRAE) was 118.1+/-31.3 microm in RA patients and 123.8+/-19.9 microm in control subjects, showing the tendency of retinal arteriolar narrowing in patients with RA but without statistical significance. The mean central retinal venular equivalents (CRVE) was 162.4+/-26.4 microm which was significantly higher than that of control group (144.1+/-23.1 microm, p<0.001). The prevalence of AVN was 34.7%, and significantly higher in RA group. Among retinal findings, the presence of early pinpoint hyperfluorescence and areas of delayed choroidal perfusion correlated with hsCRP. Age, disease duration, DAS 28, HAQ, and rheumatoid factor (RF) had no effect on CRAE and CRVE. In multivariate analysis, only hsCRP was found to be associated with wider venular caliber. CONCLUSION: Retinal venular widening was more common in RA patients. Retinal venular diameter had significant correlation with disease activity of RA. Retinal imaging is a comparative method for the assessment of microvascular findings of RA patients.
Arthritis, Rheumatoid ; Atherosclerosis ; C-Reactive Protein ; Capillaries ; Choroid ; Fluorescein ; Fluorescein Angiography ; Humans ; Joints ; Microvessels ; Multivariate Analysis ; Perfusion ; Photography ; Prevalence ; Rheumatoid Factor ; Surveys and Questionnaires

Apical ballooning syndrome (ABS), also referred to as stress cardiomyopathy, is characterized by acute left ventricular dysfunction following a stressful situation. Diagnosis of ABS is made in the following scenarios: transient hypokinesia or dyskinesia of the left ventricular segment, absence of obstructive coronary disease, new electrocardiogram abnormalities, absence of recent significant head trauma, pheochromocytoma, myocarditis, and hypertrophic cardiomyopathy. Prognosis is usually favorable since the wall motion abnormality returns to normal within days, and certainly within the first month. We encountered a case of SLE with apical ballooning on echocardiography in a 44-year-old woman. She was suffering from severe left ventricular dysfunction that has persisted on 5 year follow-up echocardiography. We report this case along with a review of the relevant literature.
Adult ; Cardiomyopathy, Hypertrophic ; Coronary Disease ; Craniocerebral Trauma ; Diagnosis ; Dyskinesias ; Echocardiography ; Electrocardiography ; Female ; Follow-Up Studies ; Humans ; Hypokinesia ; Lupus Erythematosus, Systemic ; Myocarditis ; Pheochromocytoma ; Prognosis ; Takotsubo Cardiomyopathy ; Ventricular Dysfunction, Left

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

Serotonin syndrome, an adverse drug reaction, is a consequence of excess serotonergic agonism of central nervous system receptors and peripheral serotonergic receptors. Serotonin syndrome has been associated with large numbers of drugs and drug combinations, and serotonin-norepinephrine reuptake inhibitor-induced serotonin syndrome is rare. It is often described as a sign of excess serotonin ranging from tremor in mild cases to delirium, neuromuscular rigidity, and hyperthermia in life-threatening cases. Diagnosis is based on the symptoms and patient's history, and several diagnostic criteria have been developed. We experienced a rare case of fibromyalgia accompanied by tremor, hyperreflexia, spontaneous clonus, muscle rigidity, and diaphoresis after 10 days of single use of duloxetine 30 mg. Only one case of serotonin syndrome resulting from administration of duloxetine has been reported in Korea, however that case resulted from co-administration of fluoxetine. We report here on this case along with a review of the relevant literature.
Central Nervous System ; Delirium ; Diagnosis ; Drug Combinations ; Drug-Related Side Effects and Adverse Reactions ; Duloxetine Hydrochloride* ; Felodipine ; Fever ; Fibromyalgia* ; Fluoxetine ; Humans ; Korea ; Muscle Rigidity ; Reflex, Abnormal ; Serotonin Syndrome* ; Serotonin* ; Tremor