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  • 1.

    【2hr】Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma: An Adjunctive Diagnostic Role for Monitoring EBV Viremia?

    包量

    机译 自身免疫性淋巴增生综合征和爱泼斯坦-巴尔病毒相关淋巴瘤：监测EBV病毒血症的辅助诊断作用？
    摘要：Background. Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of lymphocyte homeostasis due to defects in FAS-mediated apoptosis. ALPS is characterized by childhood onset of chronic lymphadenopathy and splenomegaly, autoimmunity, an expanded population of double-negative T cells (DNTCs), and an increased risk of lymphoma. This propensity for lymphoma in ALPS is not well understood. It is possible that lymphomagenesis in some of these patients may result from Epstein-Barr virus (EBV) infection exploiting the defective T-cell surveillance resulting from impaired FAS-mediated apoptosis. Case Presentation. We report the first case, to our knowledge, of lymphoma in a patient with ALPS that was clinically heralded by progressively increasing EBV viremia. We discuss its practical implications and the possible immune pathways involved in the increased risk for EBV-associated lymphoproliferative disorders in ALPS patients. Conclusion. In patients with ALPS, distinguishing chronic lymphadenopathy from emerging lymphoma is difficult, with few practical recommendations available. This case illustrates that, at least for some patients, monitoring for progressively increasing EBV viremia may be useful.
  • 2.

    【2hr】Differentiation between Celiac Disease, Nonceliac Gluten Sensitivity, and Their Overlapping with Crohn's Disease: A Case Series

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    机译 乳糜泻，Nonceliac麸质敏感性及其与克罗恩病重叠的区别：一个病例系列
    摘要：Celiac disease (CD) and nonceliac gluten sensitivity (NCGS) are two distinct conditions triggered by the ingestion of gliadin. Although symptoms of nonceliac gluten sensitivity may resemble those of celiac disease, due to the lack of objective diagnostic tests, NCGS is associated with overlapping symptomatologies of autoimmunities and Crohn's disease. Furthermore, a gluten-free diet is only recommended for those who meet the criteria for a diagnosis of CD. Unfortunately, that leaves many nonceliac gluten-sensitive people suffering unnecessarily from very serious symptoms that put them at risk for complications of autoimmune disorders that might be resolved with a gluten-free diet. Thus, a new paradigm is needed for aid in diagnosing and distinguishing among various gut-related diseases, including CD, NCGS (also known as silent celiac disease), and gut-related autoimmunities. Herein, we report three different cases: the first, an elderly patient with celiac disease which was diagnosed based on signs and symptoms of malabsorption and by a proper lab test; second, a case of NCGS which was initially misdiagnosed as lupus but was detected as NCGS by a proper lab test with its associated autoimmunities, including gluten ataxia and neuromyelitis optica; third, a patient with NCGS overlapping with Crohn's disease. The symptomatologies of all three patients improved significantly after 12 months of gluten-free diet plus other modalities.
  • 3.

    【2hr】A Precocious Cerebellar Ataxia and Frequent Fever Episodes in a 16-Month-Old Infant Revealing Ataxia-Telangiectasia Syndrome

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    机译 早熟的小脑性共济失调和16个月大婴儿揭示的共济失调-毛细血管扩张症的频繁发烧发作。
    摘要：Ataxia-telangiectasia (AT) is the most frequent progressive cerebellar ataxia in infancy and childhood. Immunodeficiency which includes both cellular and humoral arms has variable severity. Since the clinical presentation is extremely variable, a high clinical suspicion will allow an early diagnosis. Serum alpha-fetoprotein is elevated in 80–85% of patients and therefore could be used as a screening tool. Here, we present a case of a 5-year-old female infant who was admitted to our department at the age of 16 months because of gait disorders and febrile episodes that had begun at 5 months after the cessation of breastfeeding. Serum alfa-fetoprotein level was elevated. Other investigations showed leukocytopenia with lymphopenia, reduced IgG2 and IgA levels, and low titers of specific postimmunization antibodies against tetanus toxoid and Haemophilus B polysaccharide. Peripheral lymphocytes subsets showed reduction of T cells with a marked predominance of T cells with a memory phenotype and a corresponding reduction of naïve T cells; NK cells were very increased (41%) with normal activity. The characterization of the ATM gene mutations revealed 2 specific mutations (c.5692C > T/c.7630-2A > C) compatible with AT diagnosis. It was concluded that AT syndrome should be considered in children with precocious signs of cerebellar ataxia and recurrent fever episodes.
  • 4.

    【2hr】Variant of X-Linked Chronic Granulomatous Disease Revealed by a Severe Burkholderia cepacia Invasive Infection in an Infant

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    机译 X连锁慢性肉芽肿性疾病的一种变异，由婴儿的严重伯克霍尔德病菌洋葱侵袭性感染揭示
    摘要：Chronic granulomatous disease (CGD) is a primary immunodeficiency characterized by increased susceptibility to bacteria and fungi since early in life, caused by mutations in any of the five genes coding for protein subunits in NADPH oxidase. X-linked variant CGD can be missed during routine evaluation or present later in life due to hypomorphic mutations and a residual superoxide production. The case of a 10-month-old boy who died of pneumonia is reported. The isolation of Burkholderia cepacia from his lung, together with a marginally low nitroblue tetrazolium reduction assay (NBT), made us suspect and pursue the molecular diagnosis of CGD. A postmortem genetic analysis finally demonstrated CGD caused by a hypomorphic missense mutation with normal gp91phox expression. In a patient being investigated for unusually severe or recurrent infection, a high index of suspicion of immunodeficiency must be maintained.
  • 5.

    【2hr】Rare Cause of Seizures, Renal Failure, and Gangrene in an 83-Year-Old Diabetic Male

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    机译 一名83岁糖尿病男性的癫痫发作，肾功能衰竭和坏疽的罕见原因
    摘要：We report an 83-year-old diabetic male who presented with acute-onset renal failure, seizures, psychosis, pneumonia, and right foot gangrene. Investigations revealed thrombocytopenia, CSF lymphocytosis, ANA and dsDNA positivity, hypocomplementemia, and pneumonitis following which he was treated with pulse methylprednisolone. He was treated for Pseudomonas-related ventilator-associated pneumonia, candiduria, and E. coli-related bedsore infection prior to discharge. He was discharged at request and died 17 days later due to a respiratory infection.
  • 6.

    【2hr】Autoimmune Hepatitis with Anti Centromere Antibodies

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    机译 具有抗中心体抗体的自身免疫性肝炎
    摘要：We present the case report of a 49-year-old type 2 diabetes mellitus patient presenting with abdominal pain and black stool for 15 days. A proper workup of laboratory investigations helped us diagnose autoimmune hepatitis with anticentromere antibodies. The authors would like to highlight that screening AIH patients for anticentromere antibody is not mandatory but can be considered, especially in the presence of disease-related symptomatology for quicker, more accurate diagnosis and optimum management.
  • 7.

    【2hr】Chronic Granulomatous Disease Presenting as Aseptic Ascites in a 2-Year-Old Child

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    机译 慢性肉芽肿病为2岁儿童的无菌性腹水
    摘要：Chronic granulomatous disease (CGD) is a rare inherited immunodeficiency syndrome that results from abnormal nicotinamide adenine dinucleotide phosphate (NADPH) oxidase function. This defect leads to recurrent catalase-positive bacterial and fungal infections as well as associated granuloma formation. We review the case of a 2-year-old boy who presented with ascites and fever of an unknown origin as manifestations of CGD. Cultures were negative for infection throughout his course, and CGD was suspected after identification of granulomas on peritoneal biopsy. Genetic testing revealed a novel mutation in the CYBB gene underlying his condition. This paper highlights the importance of considering CGD in the differential diagnosis of fever of unknown origin and ascites in children.
  • 8.

    【2hr】Persistent Lymphadenopathy due to IgG4-Related Disease

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    机译 IgG4相关疾病引起的持续性淋巴结病
    摘要：A 28-year-old healthy female presented to her primary care physician with lymphadenopathy, fatigue, malaise, and night sweats. Symptoms persisted despite conservative treatment and eventually the patient underwent multiple lymph node resections and a bone marrow biopsy before a diagnosis of IgG4-related disease (IgG4-RD) was made. IgG4-RD is a relatively new disorder first histopathologically recognized within the last decade. As the disease can affect a single organ or multiple organs, symptoms can vary greatly among patients. With symptoms ranging from mild, such as lower extremity edema, to severe, such as spinal cord compression, IgG4-RD must be considered in appropriate patients. Diagnostic criteria have been proposed based on organ involvement, serum IgG4 levels, and histopathological criteria. Diagnosis can be difficult to make with many studies suggesting different values for diagnostic criteria, such as the level of tissue IgG4+/IgG+ cell ratio to delineate IgG4-RD. Treatment consists of high dose glucocorticoids as a first line therapy with some patients choosing instead to simply undergo observation. This case illustrates the difficulty in diagnosis and the need for increased awareness among medical professionals.
  • 9.

    【2hr】Intravenous Immunoglobulin and Mycophenolate Mofetil for Long-Standing Sensory Neuronopathy in Sjögren's Syndrome

    包量

    机译 静脉注射免疫球蛋白和霉酚酸酯治疗干燥综合征的长期感觉神经病
    摘要：Sensory neuronopathy is described in association with the Sjögren's syndrome (SS). We studied a 55-year-old woman with a 4-year history of progressive asymmetric numbness, distal tingling, and burning sensation in upper and lower limbs. In a few months, she developed ataxia with increased hypoanaesthesia. Electrodiagnostic tests revealed undetectable distal and proximal sensory nerve action potential in upper and lower limbs. Cervical spine magnetic resonance showed a signal hyperintensity of posterior columns. Previous treatment with high-dose glucocorticoids and azathioprine was ineffective. A combined treatment with intravenous immunoglobulin and mycophenolate mofetil was followed by a progressive and persistent improvement. This case documented the efficacy and the safety of the coadministration of intravenous immunoglobulin and mycophenolate mofetil in sensory neuronopathy associated with SS refractory to conventional immunosuppressive therapy.
  • 10.

    【2hr】A Girl with Autoimmune Cytopenias, Nonmalignant Lymphadenopathy, and Recurrent Infections

    包量

    机译 一名患有自身免疫性肾上腺癌，非恶性淋巴结肿大和反复感染的女孩
    摘要：We describe a girl, now 9 years of age, with chronic idiopathic thrombocytopenic purpura, persistent nonmalignant lymphadenopathy, splenomegaly, recurrent infections, and autoimmune hemolytic anemia. Her symptoms partly fit the definitions of both autoimmune lymphoproliferative syndrome (ALPS) and common variable immunodeficiency disorders (CVIDs). Genetic analysis showed no abnormalities in the ALPS-genes FAS, FASLG, and CASP10. The CVID-associated TACI gene showed a homozygous polymorphism (Pro251Leu), which is found also in healthy controls.
  • 11.

    【2hr】Effective Treatment of Rheumatoid Arthritis-Associated Interstitial Lung Disease by B-Cell Targeted Therapy with Rituximab

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    机译 利妥昔单抗B细胞靶向治疗有效治疗类风湿关节炎相关性间质性肺疾病
    摘要：Rheumatoid arthritis- (RA-) associated interstitial lung disease (RA-ILD) is the extra-articular complication with most adverse impact on the quality of life and survival in RA patients. However, treatment options are limited and controlled studies are lacking. Here, we present the case of a 66-year-old patient suffering from severe RA-ILD, which has been successfully treated with Rituximab (RTX). After failure of conventional DMARD therapy, our patient showed sustained improvement of clinical pulmonary parameters as well as joint inflammation following B-cell depletion with RTX. The six-minute-walk test improved from 380 meters to 536 meters and the forced vital capacity from 2.49 liters to 3.49. The disease activity score could be reduced from 7.7 to 2.8. Therefore, RTX might be considered as an alternative treatment for RA-ILD in patients not responding to conventional DMARD therapy.
  • 12.

    【2hr】Angora Wool Asthma in Textile Industry

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    机译 安哥拉羊毛哮喘在纺织工业中
    摘要：Up to now the exposures to hair and skin derivatives of animals have not yet been the subject of systematic studies. The observation of a clinical case has provided the opportunity for a review of the literature. The inpatient was a 49-year-old man, a carder in a textile factory, exposed to angora wool. He noticed the appearance of dyspnea during working hours. There was no eosinophilia in blood, and the results of pulmonary function tests were normal. The nonspecific bronchial provocation test with methacholine demonstrated an abnormal bronchial reactivity. The challenge test with angora wool was positive (decrease in FEV1 of more than 40%) as well as total IGE and specific IgE to rabbit epithelium (433 KU/l and 12.1 KUA/l, resp.). Several sources of allergens were found in the rabbit, and the main allergen was represented by proteins from epithelia, urine, and saliva. Most of these proteins belong to the family of lipocalin, they function as carriers for small hydrophobic molecules (vitamins and pheromones). If the diagnosis of occupational asthma caused by animal hair and skin derivatives may be relatively easy by means of the challenge test, defining etiology is complicated because of the lack of in vitro tests.
  • 13.

    【2hr】Chylous Ascites and Pleural Transudate: Rare Presentations in Systemic Lupus Erythematosus in Old Age

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    机译 乳状腹水和胸膜渗出液：老年系统性红斑狼疮的罕见表现。
    摘要：Systemic lupus erythematosus (SLE) is a systemic autoimmune disorder with involvement of multiple organs. Various forms of serositis, including pleural effusion, pericardial effusion, and ascites, may be found during the course of SLE. Peritoneal involvement by ascites is common in the initial presentation of SLE. However, chylous ascites is uncommon in SLE patients. Here, we describe a 93-year-old female with initial presentation of chylous ascites during SLE flares. Marked distention and an ovoid shape of the abdomen were observed. Shifting dullness and central tympanic sounds were found on percussion. Rales were heard in bilateral breathing sounds, multiple oral ulcers appeared in the oral cavity, and chest images showed bilateral pleural effusion. Abdominal sonography revealed moderate ascites and pleural effusion. Neither organisms nor malignant cells were revealed in the culture or cytology of ascites and pleural effusion. The diagnosis of SLE was arrived at by positive antinuclear antibody (ANA), discoid rash, oral ulcers, serositis (pleural effusion and ascites), and proteinuria. The patient received intravenous methylprednisolone 250 mg/day for three days. The pleural effusion resolved dramatically after steroid therapy and abdominal distention related to ascites formation subsided obviously.
  • 14.

    【2hr】Visceral Leishmaniasis or Systemic Lupus Erythematosus Flare?

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    机译 内脏利什曼病或系统性红斑狼疮耀斑？
    摘要：Systemic lupus erythematosus (SLE) is a multisystem disorder characterised by B-cell hyperactivity with production of multiple autoantibodies. Fever in SLE may be caused by disease exacerbation or by infection. We report a patient of SLE that was later complicated by fever, pancytopenia, and massive splenomegaly. Corticosteroid therapy for SLE might have masked the underlying infection at earlier stage. Despite negative results of rk-39 test and bone marrow biopsy, a very high suspicion for visceral leishmaniasis (VL) led us to go for direct agglutination test (DAT) and polymerase chain reaction (PCR) for leishmanial antigen that revealed positive results. Moreover, significant improvement in clinical and biochemical parameters was noted on starting the patient on antileishmanial therapy.
  • 15.

    【2hr】Symptomatic Secondary Selective IgM Immunodeficiency in Adult Man with Undiagnosed Celiac Disease

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    机译 有症状的继发性选择性IgM免疫功能低下的成年男子患有未确诊的腹腔疾病
    摘要：Selective IgM immunodeficiency (SIgMID) is a heterogeneous disorder with no known genetic background and may occur as a primary or a secondary condition. Celiac disease has been reported in association with several humeral immunodeficiencies, including isolated severe selective IgA deficiency, panhypogammaglobulinemia, and isolated combined IgA and IgM deficiency. There are only few reported cases of pediatric and adult patients with SIgMID and celiac disease. In this paper, we describe an adult patient with a symptomatic secondary SIgMID associated with undiagnosed celiac disease, with a resolution of clinical symptoms of immunodeficiency and serum IgM normalization following a gluten-free diet.
  • 16.

    【2hr】Acute Spontaneously Resolving Pulmonary Vasculitis: A Case Report

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    机译 急性自发解决肺血管炎：一例报告
    摘要：This is the first description that we are aware of describing the spontaneous resolution of an acute pulmonary vasculitis, possibly secondary to microscopic polyangiitis. Haemoptysis is a common symptom for patients presenting to primary and tertiary referral centres, and pulmonary vasculitis is one of a variety of aetiologies that should always be considered. The pulmonary vasculitides are difficult diagnostic and management problems. They are encumbered by a relative paucity of level 1 evidence addressing their diagnosis, classification, and treatment. This is therefore an important paper to publish because it adds to the global breadth of experience with this important clinical condition.
  • 17.

    【2hr】A Rare Case of Prototheca Algaemia in a Patient with Systemic Lupus Erythematosus and Recent Belimumab Infusion

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    机译 系统性红斑狼疮和近期贝利木单抗输注患者的原生物藻血症罕见病例
    摘要：Novel agents for the treatment of immune-mediated diseases such as systemic lupus erythematosus (SLE) have been increasingly used as an alternative to or in combination with conventional therapies. Belimumab, a human monoclonal antibody that inhibits B-cell activating factor (BAFF), has demonstrated efficacy in moderate-to-severe SLE with similar adverse effects when compared to other biologic agents and conventional SLE therapies. Here, we describe a woman with SLE and diabetes mellitus (DM) on immunosuppressive therapy for five years who was admitted to the hospital for pneumonia but had a complicated hospital course with multiple infections and, most notably, a nosocomial algaemia due to Prototheca wickerhamii, which was treated successfully with amphotericin B. She had recently received three belimumab infusions as an outpatient prior to admission to the hospital. To the best of our knowledge no cases of human protothecosis in patients receiving belimumab have been described in the English literature; however, unusual infections have to be considered in all patients undergoing immunosuppressive therapies who persist with fever despite conventional antimicrobials.
  • 18.

    【2hr】Multiple Thromboses in a Patient with Systemic Lupus Erythematosus after Splenectomy

    包量

    机译 脾切除术后系统性红斑狼疮患者的多处血栓形成
    • 作者：Deng-Ho Yang
    • 刊名：Case Reports in Immunology
    • 2012年第期
    摘要：Antiphospholipid syndrome is a disorder presenting with arterial or venous thrombus and a history of fetal loss. Early diagnosis and adequate treatment is important to prevent multiple organ failures. Here, we described a woman with a two-year history of systemic lupus erythematosus with severe nephrotic syndrome, manifested multiple thrombi over the portal vein and the inferior vena cava, combined with acute renal infarction. The patient underwent splenectomy 10 months ago. Initially, she received anticoagulant treatment and low-dose glucocorticoid, but multiple organ failure progressed. After emergency plasma exchange followed by glucocorticoid pulse therapy, the patient recovered.
  • 19.

    【2hr】Atypical Omenn Syndrome due to Adenosine Deaminase Deficiency

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    机译 腺苷脱氨酶缺乏症引起的非典型预兆综合征
    摘要：We present here a novel case of an atypical Omenn syndrome (OS) phenotype due to mutations in the ADA gene encoding adenosine deaminase. This case is noteworthy for a significant increase in circulating CD56brightCD16- cytokine-producing NK cells after treatment with steroids for skin rash.
  • 20.

    【2hr】The Efficacy of Mizoribine (Inosine Monophosphate Dehydrogenase Inhibitor) for ANCA-Associated Vasculitis with Hepatitis B Virus Carrier

    包量

    机译 咪唑啉碱（肌苷单磷酸脱氢酶抑制剂）对ANCA相关性血管炎伴乙型肝炎病毒携带者的功效
    摘要：A 42-year-old female who was an asymptomatic carrier of hepatitis B virus (HBV) was diagnosed with antineutrophil cytoplasm antibody- (ANCA-) associated vasculitis and was induced to remission with 30 mg/day prednisolone nine years ago. Four years ago, she suffered recurrence of ANCA-associated vasculitis and with 30 mg/day prednisolone was induced to remission. This time, laboratory data showed 3-fold increase in myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) levels. Administration of 30 mg/day prednisolone was started. Three days later, she was admitted to our hospital suffering from fatigue. After admission, urinalysis showed glomerular hematuria. Despite administration of 30 mg/day prednisolone, MPO-ANCA titer had been of high level, ranging from 42 to 83 EU for 2.5 months. Furthermore, the adverse effects of steroid were seen. We decided the tapering of prednisolone (25 mg/day) and the start of mizoribine (4-carbamoyl-1-β-D-ribofuranosyl imidazolium-5-olate) administration. After mizoribine treatment, MPO-ANCA titer was decreased without any mizoribine-related adverse effects. Six months later, MPO-ANCA titer was decreased to normal levels and she was induced to clinical remission without reactivation of HBV. We describe the effectiveness of mizoribine for the ANCA-associated vasculitis complicated with HBV-carrier.
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