IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported. The patient has shortness of breath, cough, and sputum after physical activity. Physical examination showed appearance of anemia, yellow staining of skin and sclera, palpable neck and multiple swollen lymph nodes. Laboratory examination, bone marrow biopsy, and lymph node biopsy confirmed the diagnosis. Therefore, clinicians should develop ideas and raise awareness of such diseases.
Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; Autoimmune Diseases ; complications ; Biopsy ; Humans ; Immunoglobulin G ; Immunoglobulin G4-Related Disease ; complications ; diagnosis

Aged ; Autoimmune Diseases ; diagnosis ; Female ; Humans ; Male ; Middle Aged ; Pancreatitis ; diagnosis ; drug therapy ; surgery ; Prednisone ; therapeutic use

The clinical manifestations of autoimmune pancreatitis (AIP) are diffuse pancreatic swelling, diffuse irregular narrowing of the main pancreatic duct, and increased serum IgG or positive serum autoantibody. Clinically, AIP can be improved dramatically with oral steroid therapy. In this report, we describe a 62-year-old woman diagnosed as autoimmune pancreatitis six years after onset of jaundice, who presented with uncontrolled blood glucose levels. The laboratory tests revealed obstructive jaundice, and the computed tomography of the pancreas showed pancreatic swelling. After six years of onset, she was diagnosed with AIP and successfully treated with steroid treatment.
Autoimmune Diseases/*diagnosis/drug therapy ; Female ; Humans ; Immunoglobulin G/blood ; Jaundice/*diagnosis ; Middle Aged ; Pancreatitis/*diagnosis/drug therapy ; Steroids/therapeutic use ; Tomography, X-Ray Computed

Adult ; Anti-Inflammatory Agents ; therapeutic use ; Autoimmune Diseases ; diagnosis ; drug therapy ; Female ; Graves Ophthalmopathy ; diagnosis ; drug therapy ; Humans ; Magnetic Resonance Imaging ; Triamcinolone Acetonide ; therapeutic use ; Young Adult

We report the first case of Susac syndrome in Koreans, in a 23-yr-old female patient who presented with sudden visual loss and associated neurological symptoms. Ophthalmic examination and fluorescein angiography showed multiple areas of branch retinal artery occlusion, which tended to recur in both eyes. Magnetic resonance imaging showed dot-like, diffusion-restricted lesions in the corpus callosum and left fornix, and audiometry showed low-frequency sensory hearing loss, compatible with Susac syndrome. She received immunosuppressive therapy with oral steroid and azathioprine. Three months later all the symptoms disappeared but obstructive vasculitis have been relapsing. This patient demonstrated the entire clinical triad of Susac syndrome, which tends to occur in young females. Although this disorder has rarely been reported in Asian populations, a high index of suspicion is warranted for early diagnosis and timely treatment.
Autoimmune Diseases/diagnosis/drug therapy ; Azathioprine/administration & dosage/*therapeutic use ; Brain/blood supply/pathology ; Female ; Hearing Loss ; Humans ; Immunotherapy ; Magnetic Resonance Imaging ; Republic of Korea ; Retinal Artery Occlusion/diagnosis/drug therapy/pathology ; Susac Syndrome/*diagnosis/*drug therapy/pathology ; Young Adult

The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin.
Male ; Humans ; Middle Aged ; Autoantibodies ; Myositis/diagnosis* ; Autoimmune Diseases ; Muscle, Skeletal/pathology* ; Hydroxymethylglutaryl-CoA Reductase Inhibitors/therapeutic use* ; Necrosis/pathology* ; Muscular Diseases/drug therapy*

Autoimmune pancreatitis is a distinct disease characterized by the presence of autoantibodies and hypergammaglobulinemia, inflammation of the pancreatic parenchyma, and irregular stricture of the pancreatic duct. The involvement of distal common bile duct is frequently observed, but intrahepatic bile duct involvement is very rare, which seem to have similar feature to primary sclerosing cholangitis. We report a case of the patient with autoimmune pancreatitis combined with extensive involvement of extrahepatic and intrahepatic bile duct, which had a favorable response to steroid therapy.
Aged ; Autoimmune Diseases/complications/*diagnosis/drug therapy ; Bile Ducts, Extrahepatic/*radiography ; Bile Ducts, Intrahepatic/*radiography ; Humans ; Male ; Pancreatitis/complications/*diagnosis/radiography ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome involving defective apoptosis in which the pathways regulating the termination of immune and inflammatory responses are disrupted. Fever, cytopenia, splenomegaly, and hemophagocytosis are typical findings of this syndrome. HLH can be induced by genetic disorders (familial) or secondary causes. While familial HLH is rare, secondary causes include infection, autoimmune disease, and malignancy in adults. Adult onset HLH may be confused with or misdiagnosed as sepsis or macrophage activation syndrome due to similar clinical manifestations and laboratory findings. Consequently, it is difficult to diagnose HLH promptly to initiate adequate immunosuppressive treatment or chemotherapy. A pediatric HLH treatment protocol such as HLH-2004 or multi-agent chemotherapy can be given to adults after adjusting the drug dosage and type. After the initial treatment, refractory or reactivated patients should undergo allogenic hematopoietic stem cell transplantation as soon as possible to improve survival. Clinical trials should determine more suitable therapeutic options for adults with HLH.
Adult* ; Apoptosis ; Autoimmune Diseases ; Clinical Protocols ; Diagnosis* ; Drug Therapy ; Fever ; Hematopoietic Stem Cell Transplantation ; Humans ; Lymphohistiocytosis, Hemophagocytic* ; Macrophage Activation Syndrome ; Sepsis ; Splenomegaly

Posterior reversible leukoencephalopathy syndrome (PRES) is characterized by transient headache, altered mental functioning, seizures, and loss of vision associated with findings of predominantly posterior cerebral lesions on imaging studies. Magnetic resonance imaging typically shows bilateral hyperintensity on T2 weighted imaging and fluid attenuated inversion recovery imaging, predominantly in the parieto-occipital region. The common etiologies of PRES include eclampsia, renal impairment, immunosuppressive treatment, cancer chemotherapy, autoimmune diseases, and hypertension. The prognosis is usually benign when adequate treatment is initiated immediately. Otherwise, delay in diagnosis and treatment may lead to permanent neurological sequelae. We report on the case of a 24-year-old man who presented with the characteristics of PRES with acute carbon monoxide poisoning.
Autoimmune Diseases ; Carbon Monoxide Poisoning* ; Carbon Monoxide* ; Carbon* ; Diagnosis ; Drug Therapy ; Eclampsia ; Female ; Headache ; Hypertension ; Leukoencephalopathies* ; Magnetic Resonance Imaging ; Poisoning ; Posterior Leukoencephalopathy Syndrome ; Pregnancy ; Prognosis ; Seizures ; Young Adult

Posterior reversible leukoencephalopathy syndrome (PRES) is characterized by transient headache, altered mental functioning, seizures, and loss of vision associated with findings of predominantly posterior cerebral lesions on imaging studies. Magnetic resonance imaging typically shows bilateral hyperintensity on T2 weighted imaging and fluid attenuated inversion recovery imaging, predominantly in the parieto-occipital region. The common etiologies of PRES include eclampsia, renal impairment, immunosuppressive treatment, cancer chemotherapy, autoimmune diseases, and hypertension. The prognosis is usually benign when adequate treatment is initiated immediately. Otherwise, delay in diagnosis and treatment may lead to permanent neurological sequelae. We report on the case of a 24-year-old man who presented with the characteristics of PRES with acute carbon monoxide poisoning.
Autoimmune Diseases ; Carbon Monoxide Poisoning* ; Carbon Monoxide* ; Carbon* ; Diagnosis ; Drug Therapy ; Eclampsia ; Female ; Headache ; Hypertension ; Leukoencephalopathies* ; Magnetic Resonance Imaging ; Poisoning ; Posterior Leukoencephalopathy Syndrome ; Pregnancy ; Prognosis ; Seizures ; Young Adult