No abstract available.
Cryoglobulinemia* ; Gangrene* ; Monoclonal Gammopathy of Undetermined Significance* ; Paraproteinemias*

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory disease found in many organs including biliary tract, salivary gland, kidney, and lung. Tubulointerstitial nephritis is the most common renal manifestation, but hematologic involvement of IgG4-RD is rare. Here, we report on a case of a 57-year-old male with IgG4-related interstitial nephritis with bicytopenia, which was initially thought to be systemic lupus erythematosus. He presented with proteinuria, anemia, thrombocytopenia, and low complement levels. Histological findings showed an increased number of IgG4-positive plasma cells (>200/high power field), and an elevated IgG4/IgG ratio (>90%). Serum levels of IgG and IgG4 were also increased. This case emphasized the importance of differential diagnosis of IgG4-RD and immune complex glomerulonephritis.
Anemia ; Antigen-Antibody Complex ; Biliary Tract ; Complement System Proteins ; Diagnosis, Differential ; Glomerulonephritis ; Humans ; Immunoglobulin G ; Immunoglobulins* ; Kidney ; Lung ; Lupus Erythematosus, Systemic ; Male ; Middle Aged ; Nephritis, Interstitial* ; Plasma Cells ; Proteinuria ; Salivary Glands ; Thrombocytopenia

Immunoglobulin G4-related disease (IgG4-RD) is a systemic disease, and lymphadenopathy is frequently observed in these patients. Among the 5 subtypes of IgG4-related lymphadenopathy, progressively transformed germinal centers (PTGC)-type IgG4-related lymphadenopathy possesses a unique characteristic that differentiates it from the other 4 subtypes. Here, we report on a rare case of PTGC-type IgG4-related lymphadenopathy accompanying Mikulicz's disease. A 39-year-old female complained of a left cervical mass and bilateral upper eyelid hypertrophy. The serum level of IgG4 was elevated, and computed tomography showed enlargement of the bilateral lacrimal and submandibular glands and left cervical lymph node. Excisional biopsy of a submandibular gland and cervical lymph node was performed, and the histopathologic findings revealed Mikulicz's disease accompanied by PTGC-type IgG4-related lymphadenopathy. After treatment of the patient with oral prednisolone and azathioprine, the patient's appearance improved. To the best of our knowledge, no case of PTGC-type IgG4-related lymphadenopathy has been previously reported in Korea.
Adult ; Azathioprine ; Biopsy ; Eyelids ; Female ; Germinal Center ; Humans ; Hypertrophy ; Immunoglobulin G ; Immunoglobulins* ; Korea ; Lymph Nodes ; Lymphatic Diseases* ; Prednisolone ; Sjogren's Syndrome* ; Submandibular Gland

A 54-year-old male diagnosed with rheumatoid arthritis (RA) was effectively treated with methotrexate and adalimumab. He was admitted with fatigue and right lower back pain which had persisted for 1 month. An enhanced abdominal computed tomography scan showed an ill-defined mass with soft tissue attenuation surrounding the right common iliac artery involving the right middle portion of the ureter. Laparoscopic ureterolysis and biopsy were performed. Microscopic evaluation confirmed the presence of fibroblastic proliferation, with a pleomorphic inflammatory cell infiltrate consisting predominantly of lymphocytes, macrophages, and vascular endothelial cells, without granuloma or neoplastic cells. Therefore, our diagnosis was retroperitoneal fibrosis (RPF)-associated RA. Clinicians should consider the possibility of RPF in patients with RA who experience lower back pain, abdominal pain, or dysuria, and order suitable imaging studies.
Adalimumab ; Abdominal Pain ; Arthritis, Rheumatoid* ; Biopsy ; Diagnosis ; Dysuria ; Endothelial Cells ; Fatigue ; Fibroblasts ; Granuloma ; Humans ; Iliac Artery ; Low Back Pain ; Lymphocytes ; Macrophages ; Male ; Methotrexate ; Middle Aged ; Retroperitoneal Fibrosis* ; Ureter

Cytomegalovirus (CMV), a member of the human herpesvirus group, causes severe disease in immunocompromised patients. In particular, CMV pneumonia can be a life-threatening disease to patients taking immunosuppressive drugs. The radiographic manifestations of CMV are variable and may consist of reticular or reticulonodular patterns, ground-glass opacities, air-space consolidations, or mixed patterns. A cavitary lesion in pneumonia associated with CMV infection is extremely rare. Herein we report on a case of CMV pneumonia which presented with a cavitary lesion and was treated successfully in a systemic lupus erythematosus patient who was taking immunosuppressive drugs.
Cytomegalovirus* ; Humans ; Immunocompromised Host ; Lung* ; Lupus Erythematosus, Systemic* ; Pneumonia*

Giant cell arteritis (GCA) is a systemic vasculitis which typically occurs in persons over 50 years old. GCA is closely related to polymyalgia rheumatica (PMR). A temporal artery biopsy is the gold standard test for the diagnosis of GCA. Recently, there is increasing evidence for the role of 18F-fluorodeoxyglucose positron emission tomography (18F-FDG-PET) in diagnosis of vasculitis. Here, we report on a case of a 67-year-old Korean male who was diagnosed with atypical GCA in subclinical stage concomitant with PMR by 18F-FDG-PET. After treatment, abnormal findings of 18F-FDG-PET were improved.
Aged ; Biopsy ; Diagnosis ; Giant Cell Arteritis* ; Giant Cells* ; Humans ; Male ; Polymyalgia Rheumatica ; Positron-Emission Tomography ; Systemic Vasculitis ; Temporal Arteries ; Vasculitis

Development of ischemic enteritis is rare in patients with systemic lupus erythematosus (SLE). We report on a case of ischemic enteritis with small bowel perforation in a 54-year-old female patient with SLE. She was diagnosed as SLE at 20 years old. Nine months ago, she developed periumbilical pain and was diagnosed with lupus enteritis. She was treated with prednisolone (0.5 mg/d), which was tapered gradually over 6 weeks. Prednisolone was maintained at 12.5 mg once a day. A similar periumbilical pain episode occurred again 7 months ago. Four weeks ago, she visited the emergency room due to diffuse abdominal pain, and abdominal computed tomography showed small bowel obstruction. Gastrointestinal manifestations improved after conservative management. She visited again due to severe abdominal pain for 2 days. She had panperitonitis due to a small bowel perforation and underwent an emergency laparotomy. The surgical specimen revealed ischemic enteritis involving whole bowel wall thickness with perforation. We report on a case of ischemic enteritis with small bowel perforation in a SLE patient diagnosed as lupus enteritis with literature review.
Abdominal Pain ; Emergencies ; Emergency Service, Hospital ; Enteritis* ; Female ; Humans ; Intestinal Perforation ; Laparotomy ; Lupus Erythematosus, Systemic ; Middle Aged ; Prednisolone

Relapsing polychondritis is a rare autoimmune disease involving the cartilaginous structures of the whole body. Its diagnosis can be difficult when the typical clinical features such as auricular chondritis are absent. Here, we report on a case of a 51-year-old female who presented with cough, dyspnea, and polyarthritis. Chest computed tomography showed the diffuse involvement of tracheobronchial cartilage. According to Damiani's criteria, she was diagnosed as relapsing polychondritis even though there was no unique involvement of auricular cartilage, and high dose steroid and immunosuppressive therapy were then started. This case indicated that patients who have tracheobronchial cartilage involvement without definite auricular chondritis should be considered for relapsing polychondritis as a differential diagnosis.
Arthritis ; Autoimmune Diseases ; Cartilage ; Cough ; Diagnosis ; Diagnosis, Differential ; Dyspnea ; Ear Cartilage ; Female ; Humans ; Middle Aged ; Polychondritis, Relapsing* ; Thorax

OBJECTIVE: To evaluate the prevalence and risk factors of upper extremity musculoskeletal diseases (MSDs) among Korean farmers. METHODS: The study was carried out from June 2013 to August 2015 on 850 farmers and 203 non-farmers (controls) in Gyeongnam Province. Physical examinations were performed by rheumatologists, orthopedists, and rehabilitation specialists. Plain radiography, a nerve conduction examination, and magnetic resonance imaging were performed, and the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire was used to assess upper extremity function. RESULTS: Thirty-four different types of upper extremity MSDs were detected in the 1,053 study subjects. The prevalence of any MSD in farmers was 8.96-fold higher than in control (p<0.001). The most obvious difference in prevalence between farmers and non-farmers was hand osteoarthritis (48.2% vs. 4.9%). Mean total DASH score was higher for farmers than non-farmers (14.29+/-13.66 vs. 10.03+/-10.85, p<0.001). Among farmers, myofascial pain syndrome, rotator cuff tear, and epicondylitis were more prevalent among overhead workers (growing persimmons, pears, and grapes) than in non-overhead workers (growing rice and upland crops). The following factors were associated with a rotator cuff tear; older age, overhead work, high waist circumference, and lower level of education. Hand osteoarthritis was found to be associated with older age, a female gender, high waist circumference, and longer total work time. CONCLUSION: The prevalence of upper extremity MSDs is much higher in farmers than non-farmers and greater still for farmers doing overhead work. Various factors contribute to the occurrence of upper extremity MSDs, and thus, the authors suggest an efficient preventive strategy, which involves consideration of type of work and risk factors, be established for farmers to reduce upper extremity MSDs.
Arm ; Diospyros ; Education ; Female ; Hand ; Humans ; Magnetic Resonance Imaging ; Musculoskeletal Diseases* ; Myofascial Pain Syndromes ; Neural Conduction ; Osteoarthritis ; Physical Examination ; Prevalence* ; Pyrus ; Radiography ; Rehabilitation ; Risk Factors* ; Rotator Cuff ; Shoulder ; Specialization ; Tears ; Upper Extremity* ; Waist Circumference

OBJECTIVE: This study assessed the efficacy and safety of secukinumab and adalimumab in patients with active ankylosing spondylitis (AS). METHODS: A Bayesian network meta-analysis was performed with direct and indirect data collected from randomized controlled trials (RCTs) of efficacy and safety of secukinumab 75 mg, 150 mg and adalimumab 40 mg in patients with active AS. RESULTS: Five RCTs (1,483 patients) met the inclusion criteria. The Assessment in Spondyloarthritis International Society response criteria of ≥20% (ASAS20) response rate was significantly higher in the adalimumab 40 mg (Odds ratio [OR], 4.26; 95% credible interval [CrI], 2.09~8.08), secukinumab 150 mg (OR, 3.35; 95% CrI, 1.73~6.56), and 75 mg dose (OR, 2.44; 95% CrI, 1.06~5.05) than with placebo. The ranking probability based on the surface under the cumulative ranking curve (SUCRA) indicated that adalimumab 40 mg had the highest probability of being the best treatment for achieving an ASAS20 response (SUCRA=0.8753), followed by secukinumab 150 mg (SUCRA=0.7051), secukinumab 75 mg (SUCRA=0.4113), and placebo (SUCRA=0.0083). The ASAS40 response rate distribution pattern was similar to the ASAS20 response rate. However, the number of serious adverse events did not differ significantly among the treatment options. CONCLUSION: Secukinumab and adalimumab were effective for the treatment of active AS without causing a significant risk of serious adverse events.
Adalimumab* ; Humans ; Spondylitis, Ankylosing*