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

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

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

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

BACKGROUND/AIMS: Although trials have suggested an association between osteoporosis and cardiovascular disease (CVD), the relationship between fracture risk and cardiovascular disease is not well defined. Here, we examined whether subjects with a higher risk of fracture also share an increased likelihood of developing CVD. METHODS: This study included 477 subjects; patients with a history of diabetes, chronic hepatopathy, nephritic syndrome, or any cardiovascular diseases were excluded. We used dual energy X-ray absorptiometry to assess the bone mineral density (BMD) of the lumbar spine and femur, and calculated fracture risk based on the Fracture Risk Assessment (FRAX) score. The Framingham risk score (FRS) was used to estimate cardiovascular risk. RESULTS: Of the 477 subjects, 222 had osteopenia and 150 had osteoporosis; the remaining 105 had a normal BMD. In men, no significant differences were observed in systolic blood pressure (SBP), diastolic blood pressure, low-density lipoprotein, high-density lipoprotein (HDL), and triglyceride (TG) between groups. Men with osteoporosis were generally older, and had significantly higher total cholesterol (TC). In women, age and FRS were significantly higher in the osteoporosis group. In the multivariate analysis, age, SBP, TC, HDL, TG, and FRAX were all significantly associated with FRS. CONCLUSIONS: These data suggest that patients with a higher risk of fracture are also at greater risk of developing CVD, indicating a possible mechanistic link between CVD and osteoporosis.
Absorptiometry, Photon ; Blood Pressure ; Bone Density ; Bone Diseases, Metabolic ; Cardiovascular Diseases ; Cholesterol ; Female ; Femur ; Humans ; Lipoproteins ; Male ; Multivariate Analysis ; Osteoporosis ; Risk Assessment* ; Spine ; Triglycerides

Renal tubular acidosis (RTA) refers to a group of disorders involving transport defects in bicarbonate reabsorption or hydrogen excretion. Features like metabolic acidosis with a normal anion gap, neurological symptoms, and electrolyte imbalances indicate RTA. Kidney transplantation, cirrhosis, sickle cell anemia, medications, and autoimmune diseases, particularly Sjogren's syndrome and rheumatoid arthritis, are related to RTA. We encountered a rare case of a patient with systemic lupus erythematosus accompanied by RTA secondary to tacrolimus administration, who had muscle weakness and paralysis. Her symptoms improved after discontinuing tacrolimus and correcting the acidosis and potassium levels. Here, we report on this case and review the relevant literature.
Acid-Base Equilibrium ; Acidosis ; Acidosis, Renal Tubular* ; Anemia, Sickle Cell ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Fibrosis ; Humans ; Hydrogen ; Kidney Transplantation ; Lupus Erythematosus, Systemic* ; Muscle Weakness ; Paralysis ; Potassium ; Sjogren's Syndrome ; Tacrolimus*

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

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

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