2015年 · 第54卷第11期

Ankylosing spondylitis (AS) is a chronic autoimmune disease and inflammatory disease with inflammation of central axis and peripheral joints AS the main clinical manifestation. In recent years, the understanding of AS in the international rheumatology community has changed greatly. It is believed that AS, AS a chronic systemic inflammatory disease, not only invades the central axis and peripheral joints, but also is often accompanied by the involvement of extraarticular organs such AS eyes, skin, gastrointestinal tract, cardiovascular and other psychological symptoms such AS anxiety and depression. Moreover, the involvement of important organs such AS eyes and heart and psychological diseases will lead to the loss of work ability and higher disability rate, and also affect doctors' treatment decisions. At the 2015 European Anti-Rheumatic Alliance Congress, many scholars proposed that the extra-articular manifestations of AS should be attached great importance, and emphasized the view that AS is a systemic disease. When formulating treatment plans, it is necessary to join ophthalmology, dermatology, gastroenterology and even other specialists such AS psychology to improve the prognosis of patients. For more information, please read the monograph "Ankylosing Spondylitis: An Overlooked Systemic Disease" by Professor Kunpeng Li and Professor Feng Huang.

Ankylosing spondylitis (AS) is a chronic autoimmune disease and inflammatory disease with axial and peripheral joint inflammation AS the main clinical manifestation. X-ray sacroiliitis is one of the main diagnostic criteria, while tendon attachment point inflammation is the pathological characteristic of AS. AS a chronic systemic inflammatory disease, AS is often accompanied by the involvement of eyes, skin, gastrointestinal tract, cardiovascular organs and other psychological symptoms such AS anxiety and depression. Moreover, the involvement of eyes, heart and other important organs and psychological disorders will lead to the loss of work ability and higher disability rate, and will also affect doctors' treatment decisions^[1]。 At the 2015 European Anti-Rheumatic Alliance Congress, many scholars proposed that the extra-articular manifestations of AS should be attached great importance, and emphasized that AS is a systemic disease. When formulating treatment plans, it is necessary to join ophthalmology, dermatology, gastroenterology and even other specialists such AS psychology.

primary Sjogren's syndrome (pSS) is a kind of autoimmune disease involving mainly exocrine glands, which can involve all organs in the whole body. The incidence rate is 0.29% ~0.77%, and it is on the rise^[1]。 There are more and more researches on pSS in the world, but there are still few researches on pSS in China, which need to be further strengthened regardless of the breadth and depth.

In 1990, the American Institute of Medicine proposed a well-recognized definition of clinical practice guidelines: sets of guidance developed systematically to help physicians and patients make appropriate decisions and handling specific clinical problems^[1]。 In recent years, with the vigorous development of evidence-based medicine, there have been new developments in the definition and formulation of clinical practice guidelines. In 2011, the American Institute of Medicine revised the definition of clinical practice guidelines: optimal guidance from evidence generated through a systematic review and evaluation of the pros and cons of various alternative interventions^[2]。 This definition emphasizes the importance of evidence-based approaches, requiring the search for corresponding evidence and the development of clinical practice guidelines through systematic reviews, i.e. the development of clinical practice guidelines based on evidence.

Patients with aplastic anemia (AA) and myelodysplastic syndrome (MDS) can lead to iron overload due to dependence on blood transfusion and ineffective hematopoiesis of the disease itself. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is an important treatment for various benign and malignant hematological diseases, and its efficacy is affected by hematopoietic stem cell transplantation, infection and graft versus host disease (GVHD). Studies have shown that iron overload is associated with increased risk of hepatic venous occlusion (VOD), infection and GVHD after allo-HSCT, which affects the success rate of transplantation^[1,2]。 This study retrospectively analyzed the clinical data of 50 patients undergoing allo-HSCT with iron overload to explore the efficacy, safety and effect of iron chelator therapy on hematopoietic stem cell implantation, infection and GVHD.

The patient was a 70-year-old male. He was admitted to hospital in May 2010 due to "weight loss and fatigue, accompanied by abdominal mass for half a year". B-ultrasound in external hospital before admission showed splenomegaly; Blood routine showed WBC 41.6×10⁹/L, Hb 92 g/L, PLT 104×10⁹/L, untreated. Admission physical examination: body temperature 36.7℃, pulse 88 beats/min, breathing 21 beats/min, blood pressure 135/82 mmHg (1 mmHg =0.133 kPa); The superficial lymph nodes were not palpable and swollen, and no abnormal murmur was heard in both lungs and heart valves; The lower margin of the spleen reached the pelvis, and the right lateral margin reached 2 cm to the right of the anterior median line, and the abdominal mobile voicing was negative. Blood routine showed WBC 16.5×10⁹/L, RBC 2.54×10¹²/L, PLT 74×10⁹/L. Abdominal color ultrasound showed that the spleen was about 64 mm thick and 64 mm under the costs, with uneven echoes. Bone marrow puncture: the proliferation of nucleated cells is obviously active, the proportion of lymphocytes is increased (62.5%), and the cytoplasm of cells is easy to see hairy flocculate processes, which is considered as hairy cell leukemia (HCL). Flow cytometry (FCM) suggested B-cell proliferative disease; Flow immunophenotypic detection showed that the expression of CD₂₀HLA-DR, CD₁₀₃CD₃₈CD₁₃CD₂₅CD_11c, weakly expressing CD₅CD₂₂, suggestive of B-cell proliferative disease; Chromosome analysis showed: 46, XY [2], no abnormalities were observed. Comprehensive analysis diagnosed HCL and cladribine 5.67 mg (0.09 mg/kg) was administered on 16 June 2010 as a continuous 24 h instillation for 7 d. On the second day after the end of treatment, the patient developed fever with a maximum body temperature of 39.0℃, accompanied by chest tightness, cough, expectoration, and obvious wet rales and wheezing sounds in both lungs. Blood routine showed: WBC 3.9×10⁹/L, Hb 89 g/L, Neutrophils 1.1×10⁹/L, PLT 57×10⁹/L. Re-examination of chest CT showed inflammation of both lungs and bilateral pleural effusion. After that, all three lines of the patient's blood image decreased progressively, the agranulocytosis period exceeded 3 weeks, reaching 3 degrees of bone marrow suppression, and combined with cardiac insufficiency. He was given anti-infective treatment with ceftazidime, imipenem and voriconazole successively, supplemented by diuretic drugs to reduce cardiac load, nutritional myocardial drugs to improve cardiac function, body temperature was controlled, and the symptoms improved after discharge. After 4 years of intermittent follow-up, there was no obvious discomfort, no lymph nodes and spleen enlargement, and the blood routine was normal, suggesting that he was in hematological remission.

Patient, female, 65 years old. The main cause was "intermittent skin ecchymosis throughout the body for more than 2 years, aggravated with fatigue for more than 1 month". In the past 2 years, the patient had intermittent skin "ecchymosis" without obvious triggers, and there was no other special discomfort. The ecchymosis could be relieved by itself, and no diagnosis and treatment were given. In the past 1 month, skin ecchymosis was significantly aggravated, accompanied by fatigue, no fever, no night sweats, no obvious weight loss, no swelling and pain of bones and joints, no abdominal distension and abdominal pain, anorexia, and urination and defecation as usual. Examination at local hospital showed: WBC 71.56×10⁹/L, neutrophils 0.92, RBC 1.85×10¹²/L, Hb 76 g/L, PLT 41×10⁹/L; Bone marrow puncture considering the chronic phase of chronic myeloid leukemia. He was transferred to our hospital on March 20, 2014. Prior history of hypertension for 1 year. Admission physical examination: general condition OK, no fever, anemia appearance, heart, lung, abdomen and nervous system physical examination no positive signs. Laboratory Test: WBC 84.82×10⁹/L, neutrophils 0.903, monocytes 0.019, lymphocytes 0.055, basophils 0.003, Hb 74 g/L, PLT 62×10⁹/L. fecal occult blood (-); Urinary routine occult blood (+), protein (-), leukocytes 119.4/μ L, 21.5/high power field. Liver and kidney function, C-reactive protein, procalcitonin, immunoglobulin, autoimmune disease serum immune antibody and tumor markers were all normal. CT: A few small inflammatory nodules in the lateral segment of the right middle lobe, bilateral pleural thickening, and a small amount of bilateral pleural effusion; Spleen enlargement, local protrusion of the posterior upper part of the right gluteus maximus muscle, inflammatory changes; Blurred gap of subcutaneous fat in left buttock.

The patient was an 18-year-old female. He was admitted to the hospital on 17 July 2012 with a complaint of "10 months of menopause". The patient had no obvious trigger of menopause in October 2011, and no other obvious discomfort and abnormalities. Menarche was 11 years old, the cycle was 28 days, and the period was 3 days. The period was regular and the amount was acceptable. Last menstruation September 20, 2011. Past history, personal history, and family history are not special. Admission physical examination : BMI 18.7 kg/m²The muscles are stronger; The voice is deep; Breast stage B5; Thick black hair is seen next to the double areola and the midline under the umbilicus; Pubic hair P5 stage, in inverted triangle shape; The clitoris is hypertrophic, approximately 1.5 cm long, and hairy around the anus. The right adnexal area touches a mass about 5 cm in diameter. Follicle stimulating hormone (FSH) 4.2 IU/L, luteinizing hormone (LH) 10.1 IU/L, testosterone 22.2 nmol/L (female normal 0-2.6 nmol/L), estradiol 169.1 pmol/L, progesterone 3.7 nmol/L; 17 α hydroxyprogesterone 11.6 nmol/L; Medium-dose dexamethasone inhibition test: 17 α hydroxyprogesterone (after administration) 6.8 nmol/L; Blood cortisol (10:00) 259.8 nmol/L (normal 185-624 nmol/L); Adrenocorticotropic hormone (ACTH) (10:00) 10.0 pmol/L (normal 1.6-13.9 pmol/L); No obvious abnormalities were found on adrenal CT. Pelvic CT showed a 4.51 cm ×5.53 cm mixed density occupation in the right adnexal area of the uterus. After gynecological contrast-enhanced ultrasound examination, the cyst wall showed complete thick wall perfusion after injection of contrast medium, with a wall thickness of 0.3 cm, surrounding the middle and high echoic foci, and rapidly synchronized perfusion in the middle and high echoic foci, but the internal contrast medium resolved significantly earlier than the cyst wall and the surrounding normal ovarian tissues.

June 2015Rheumatology(Oxford) published a study on IgG led by Professor Zhang Wen of Peking Union Medical College and Peking Union Medical College Hospital, Chinese Academy of Medical Sciences₄Related disease (IgG₄-RD): "IgG₄Related Disease Clinical Characteristics: A Prospective Cohort Study of 118 Chinese Patients "[Lin W, Lu S, Chen H, et al. Clinical characteristics of immunoglobulin G4-related disease: a prospective study of 118 Chinese patients [J]. Rheumatology (Oxford), 2015], which summarized the IgG₄-Clinical characteristics, laboratory tests and therapeutic response of affected organs in patients with RD.

May 2015Rheumatology(Oxford) published online the clinical study: "Diagnostic value of salivary gland ultrasound in Sjogren's syndrome: comparison of two scoring systems" by the Department of Rheumatology and Immunology and the Department of Ultrasound Diagnosis, Peking University People's Hospital [Xia Z, Shanshan Z, Jing H, et al. Ultrasonographic evaluation of major salivary glands in primary Sjogren's syndrome: comparison of two scoring systems. Rheumatology (Oxford), 2015]. This study compared the value of two scoring systems, salivary gland ultrasound 0-16 and 0-48, in the diagnosis of primary Sjogren's syndrome (pSS) using ultrasound technique, and analyzed the correlation between the two scoring systems and the clinical indexes of pSS.

Fever is one of the common clinical symptoms, and it is a systemic reaction of human body to pathogenic factors. It is generally considered that fever is when the oral temperature is higher than 37.3℃, the anal temperature is higher than 37.6℃, or the body temperature fluctuates more than 1.2℃ in one day. If the fever lasts for more than 3 weeks and the body temperature exceeds 38.3℃ (anal temperature) many times, the disease group that cannot be definitively diagnosed after at least 1 week of careful examination is called fever unknown origin (FUO), which is customarily called fever to be examined^[1]。

Autoinflammatory disease (AID) refers to a group of diseases in which the encoded protein is altered due to gene mutation, and the innate immune system is dysregulated, thus causing a systemic immune response. AID is more common than the early onset after birth, and has a certain geographical and ethnic distribution. It is more common in the Middle East, Europe and the United States, and it is also reported in China and Japan. The disease spectrum is wide and the clinical manifestations are diverse, mainly with fever, rash, joint pain, arthritis and ocular lesions as prominent symptoms, which can involve multiple organs and systems in the whole body, and are often accompanied by immune abnormalities and metabolic disorders. According to its pathogenesis, AID is divided into monogenic genetic diseases (dominant or recessive) and multifactorial polygenic diseases. Now, monogenic AID is mainly introduced, and the classification is shown inTable 1^[1,2,3,4]。

Polymyositis (PM) /dermatomyositis (DM) is a kind of systemic inflammatory disease that mainly invades skeletal muscle. interstitial lung disease (ILD) is a common complication, which can be divided into two subcategories according to the onset of the disease: acute/subacute interstitial pneumonia (A/SIP) and chronic interstitial pneumonia (CIP). A/SIP progresses rapidly and has a poor prognosis. The literature reports that the survival rate within half a year is less than 50%^[1]。 The pathogenesis, autoantibodies, treatment and prognosis of PM/DM combined with ILD are described.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is an effective treatment for malignant hematological diseases such as acute and chronic leukemia. Graft versus host disease (GVHD) is one of the major complications after allo-HSCT therapy, and it is also an important factor leading to transplantation failure. When patients undergo allo-HSCT, preconditioned tissue damage produces inflammatory factors that activate the host's antigen presenting cells (APCs). APC presents host antigen and further activates immunoactive T lymphocytes in the graft, causing them to proliferate, differentiate, and produce effector factors (including cytotoxic T lymphocytes, NK cells, TNF α, IL-1, etc.), causing tissue damage of the host target organ and forming GVHD. GVHD is regulated by many factors, among which indoleamine 2,3-dioxygenase (IDO) is a key factor in the regulation of GVHD.

[Website] http:onlinelibrary.wiley.com/journal/10.1002/ (ISSN) 2326-5205

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Recently, the results of a randomized, double-blind, multicenter study of knee osteoarthritis treatment showed that the pain intensity score of patients in the intra-articular sustained-release triamcinolone acetonide (FX006) 40 mg dose group was significantly better than the current standard of care of immediate-release triamcinolone acetonide group (J Bone Joint Surg Am, 2015, 97: 877-888).