2017年 · 第56卷第09期

The prevalence of chronic diseases represented by rheumatic immune diseases is on the rise globally. In China, the prevalence of chronic diseases among people aged ≥15 years increased from 20.7% in 1993 to 33.1% in 2013, among which the prevalence of rheumatic immune diseases is second only to circulatory diseases and endocrine and metabolic diseases, ranking third. In recent years, with the unremitting efforts of rheumatologists in China, the standardized diagnosis and treatment of rheumatic immune diseases has been continuously improved. Relatively speaking, the management of disease diagnosis and treatment program implementation process and long-term prognostic interventions are not sufficiently paid attention to. Due to the lack of basic medical knowledge and life guidance and education for patients, patients' compliance is poor, the treatment plan is violated, the disease is ultimately failed to control, and even the disease relapses due to improper drug withdrawal. Set up rheumatic immune disease chronic disease management and patient education outpatient clinics, strengthen the "extended chronic disease management service" for rheumatic immune disease patients, and improve the prognosis of patients. Professors Yang Hang, Zhao Yi and Liu Yi were invited to write a monograph "Are you ready for chronic disease management of rheumatic immune diseases?".

In recent years, the prevalence of chronic diseases represented by rheumatic immune diseases has shown an obvious upward trend worldwide^[1]。 In China, the prevalence of chronic diseases among people aged ≥15 years increased from 20.7% in 1993 to 33.1% in 2013, among which the prevalence of rheumatic immune diseases ranked third after circulatory diseases and endocrine and metabolic diseases^[2]。 Rheumatic immune diseases not only bring great pain to patients, but also become a serious public health problem that affects social and economic development. The prevalence of rheumatic immune diseases is expected to increase further with the prolongation of the average life expectancy and the aging of the population^[3]。 In recent years, with the unremitting efforts of rheumatologists in China, the standardized diagnosis and treatment of rheumatic immune diseases has been continuously improved. Relatively speaking, the management of disease diagnosis and treatment program implementation process and long-term prognostic interventions are not sufficiently paid attention to. Due to the lack of education on patients' basic medical knowledge and life guidance, patients' compliance is poor, the treatment plan is violated, and the disease is ultimately failed to control, and even the disease relapses due to improper drug withdrawal. Since 2010, our department has taken the lead in setting up chronic disease management and patient education clinics for rheumatic immune diseases in view of this situation, and strengthened the "extended chronic disease management service" for patients with rheumatic immune diseases, which has significantly improved the long-term prognosis of patients.

Ankylosing spondylitis (AS) is one of the most common chronic inflammatory diseases in the field of rheumatism. Its main characteristics are chronic progressive low back pain and gradual spinal deformity and disability; In addition, it is often accompanied by the involvement of eyes, skin, gastrointestinal tract, cardiovascular and other extraarticular organs, as well as the resulting psychological symptoms such as anxiety and depression, which ultimately leads to the decline of patients' exercise ability and quality of life^[1]。 AS early AS 1950s, some scholars proposed the therapeutic effect of exercise on AS. Since then, exercise has been an important part of AS treatment recommendations for decades, and has been written into the recommendation guidelines for AS treatment by the International Association for the Evaluation of Spondyloarthritis (ASAS)^[2]。 In the past 10 years, with the emergence of TNF antagonists, the drug treatment of AS has undergone dramatic changes. TNF antagonists can not only rapidly improve the symptoms and signs of patients, but also significantly improve spinal function after long-term treatment. Nevertheless, the ASAS/European Alliance Against Rheumatism (EULAR) treatment recommendations updated in 2016 still emphasize that the best treatment strategy for AS needs to be combined with drug therapy and non-drug therapy^[3]It can be seen that the role of non-drug therapy in the treatment of AS is irreplaceable.

Gitelman syndrome (GS) is an autosomal recessive salt-loss renal tubular disease. It is now clear that the etiology of GS is the loss-of-function mutation of SLC12A3, a gene encoding the thiazide diuretic-sensitive sodium-chloride co-transporter (NCCT) protein located in the distal convoluted tubule of the kidney, resulting in structural and/or functional abnormalities of NCCT, which causes the reabsorption of sodium-chloride by the distal convoluted tubule of the kidney, resulting in a series of pathophysiological and clinical manifestations such as hypovolemia, activation of renin-angiotensin-aldosterone system (RAAS), hypokalemia and metabolic alkalosis^[1]。

Medicine is a profession that requires high professional standards, and has high requirements for the professional ability, integrity and self-discipline and ethics of practitioners, especially doctors. The quality cultivation of physicians directly determines the perception of the medical and health industry in the society.

The patient was a 25-year-old female. He was admitted to hospital on April 18, 2016 due to paroxysmal limb weakness with stiffness for more than 1 year. One year ago, the patient suffered from sudden limb weakness without obvious trigger, unable to stand and walk, unable to turn over, and then experienced numbness and stiffness of the limbs, accompanied by palpitations. He went to a local hospital to check for low blood potassium (details unknown), and his symptoms improved after potassium supplementation. Two months ago, the above symptoms appeared again after catching a cold, and he was examined for hypokalemia (details unknown) in the local hospital. After potassium supplementation, he was transferred to our hospital for further diagnosis and treatment. Past history and personal history are not special. Deny a history of similar disease in the family. Physical examination at admission: height 154 cm, weight 43 kg, blood pressure 108/66 mmHg (1 mmHg =0.133 kPa), heart rate 76 beats/min, no abnormalities in heart, lung and abdomen examinations, and no pathological reflexes were elicited. Laboratory tests: No abnormalities were found in routine hematuria stool. Serum potassium 2.84 mmol/L, sodium 136.40 mmol/L, chlorine 98.20 mmol/L, magnesium 0.560 mmol/L, TG 1.94 mmol/L, blood pH 7.47, standard bicarbonate 26.60 mmol/L, total carbon dioxide 21.00 mmol/L. Urinary calcium was 0.13 mmol/24 h, urine magnesium was 2.157 mmol/24 h, urine phosphorus was 2.79 mmol/24 h, serum potassium was 3.25 mmol/L and magnesium was 0.582 mmol/L in the same period. Cortisol circadian rhythm, adrenocorticotropic hormone normal. Basal renin-angiotensin-aldosterone system (RAAS) examination: angiotensin I (37 ℃) 49.94 μ g/L, angiotensin I (4 ℃) 6.87 μ g/L, aldosterone 149.05 ng/L, renin activity 31.87 μ g·L^－1• h^－1, aldosterone/renin activity 0.47. Chest X-ray, abdominal ultrasound, thyroid ultrasound, kidney and renal vascular ultrasound, adrenal ultrasound and adrenal thin-section CT showed no abnormalities. Electrocardiogram results showed: sinus tachycardia (105 beats/min). The mean 24-h ambulatory blood pressure was 105/71 mmHg. After admission, potassium supplementation was given, and blood potassium returned to normal.

Barth's syndrome (BS) is a rare autosomal recessive hereditary disease. It is a primary renal salt loss disease caused by water and salt reabsorption disorder in the thick segment of the ascending branch of the nephron haptic. Its main characteristics are hypokalemia, hypochlorine metabolic alkalosis, high renin activity and hyperaldosteronemia. BS is divided into 5 types according to the different genes where the pathogenic mutation is located, which areSLC12A1 gene (600839, type 1),KCNJ1 gene (600359, type 2),CLCNKBGene (602023, type 3),BSNDGene (606412,4aType),CLCNKBGenes andCLCNKAGene (602024) co-mutation (4bType) orCASRCaused by mutation in gene (601199, type 5). Among them, the type 2 pathogenic geneKCNJ1 is located on chromosome 11q24.3, but due to the variation of the cleavage site, five transcripts were produced, which can be encoded to form three products, the extrarenal medullary potassium channel (ROMK) 1, ROMK2 and ROMK3, which consists of 391, 372 and 389 amino acids respectively. These three products are expressed in the kidney. ROMK is distributed from the thick segment of the ascending branch of the haptic to the distal nephron. It is an inward rectifying potassium channel, which is mainly responsible for the secretion of potassium ions on the membrane side of the lumen. A case of type 2 BS is reported.

Case 1 male, 59 years old. He was admitted to the endoscopy center of our hospital in September 2016 due to "half a month of suffocation in the left upper abdomen". Half a month ago, the patient had left upper abdominal distension without obvious trigger, which was intermittent, and had nothing to do with eating and posture changes, without radiating pain. Physical examination showed no obvious positive signs. Abdominal color ultrasound showed chronic cholecystitis with gallbladder stones, and no obvious abnormalities were found in liver, pancreas, spleen, kidneys and portal vein. There were no abnormal findings in routine examinations such as blood routine, biochemistry and tumor markers. Gastroscopy showed that there were two bulges in the large curvature of the stomach, about 1.5 cm ×0.8 cm in size, with a purple-blue surface and slightly rough mucosa. Endoscopic ultrasonography showed that the mucosal layer was slightly thickened, and the submucosal layer was obviously thickened, showing multiple anechoic structures. Two large-scale ectopic gastric mucosa can be seen in the lower esophagus, one 35-38 cm away from the incisors and the other 39-42 cm away from the incisors. Endoscopic submucosal dissection (ESD) and purse suture of gastric body mass were performed in our department. Gross specimen: The cyst cavity can be seen in the section, containing viscous liquid, and the cyst wall thickness is 0.2~0.3 cm. Postoperative pathology showed that the gastric mucosa was thickened with polypoid hyperplasia, some of the gastric foveae were elongated in serrated shape, some of the glands were cystic dilated with epithelial hyperplasia, a few of them were sieve-like hyperplasia and mild atypical hyperplasia, and some of them were invaginated to the submucosa, and the shape was consistent with cystic polypoid gastritis/deep gastritis.

The patient was a female Uyghur, 54 years old. He was seen in May 2008 due to "general fatigue for half a year". Blood routine: WBC 9.4×10⁹/L, RBC 5.3×10¹²/L, Hb 132 g/L, PLT 1 048×10⁹/L; Liver and kidney function, electrolytes and globulin were normal; Bone marrow smear showed large number of megakaryocytes, good function, and large platelet aggregation;JAK2 geneV617FMutation positive; bcr-abl fusion gene negative; Chromosome: 46,XX[20]。 Diagnosis of essential thrombocythemia (essential thrombocythemia，ET), given platelet apheresis and interferon, hydroxyurea treatment, aspirin anti-platelet aggregation, the minimum platelet reduction to about 500×10⁹/L。 The blood routine was reviewed every week after discharge, and the platelet fluctuation was 500×10⁹/L～900×10⁹/LInterferon and hydroxyurea have been used for maintenance therapy. At the beginning of 2010, I stopped using interferon on my own. After falling in July 2010, I felt slight pain in the left hypochondrial area, and then gradually developed to bilateral femoral pain. In a routine blood test in another hospital, blood cells were reduced (with the most obvious thrombocytopenia), rib fracture, and globulin increased. I was transferred to our hospital for a clear diagnosis.

July 2017Kidney IntA single-center retrospective study "Analysis of clinical characteristics of patients with Takayasu arteritis with renal artery involvement" by Professor Tian Xinping and Professor Zeng Xiaofeng, Department of Rheumatology and Immunology, Peking Union Medical College Hospital was published online [Chen Z, Li J, Yang YJ, et al. The renal artery is involved in Chinese Takayasu arteritis patients. Kidney Int, 2017]. The clinical characteristics of TAK patients with renal artery involvement were explored for the first time.

The patient was a 43-year-old male, a farmer. Because of "swelling and pain of hand and foot joints for half a year and edema of both lower limbs for 1 month", he visited the internal medicine clinic of our hospital. Six months ago, the patient developed swelling and pain of bilateral wrist joints and proximal interphalangeal joints without trigger, accompanied by morning stiffness for 1~2 h, and occasionally took "analgesics", which could improve slightly. One month ago, I developed swelling and pain of both ankle joints, accompanied by concave edema of both lower limbs, without eyelid edema and increased foam in urine. Previous hypertension for more than 3 years, irregular administration of "amlodipine besylate", the highest blood pressure 160/110 mmHg (1 mmHg =0.133 kPa); 3~5 years ago, the physical examination found that blood sugar was elevated, and the diet was controlled appropriately without regular monitoring. Smoking, 1 pack/d, no alcohol. Family history: Both father and grandfather had diabetes. Physical examination: blood pressure 163/100 mmHg, BMI 27.5 kg/m²No obvious abnormalities were found in the heart and lungs. Bilateral wrist tenderness, slight swelling of bilateral proximal interphalangeal joints, concave edema of both ankles and lower limbs.

Systemic sclerosis (SSc) is an autoimmune disease characterized by skin sclerosis and involvement of multiple systems and organs. Autoimmune injury, vascular lesions and extensive fibrosis are the main pathogenesis^[1]。 The range of skin involvement of SSc is varied, which can only be local skin involvement such as distal limb and face, or extensive skin sclerosis in the whole body^[2]。 According to the skin involvement of patients, SSc was divided into four subtypes: localized cutaneous SSc, diffuse cutaneous SSc, no cutaneous sclerosis SSc, and overlapping syndrome^[3]。 Among them, the characteristics of non-dermatosclerotic SSc are that there are no typical dermatosclerotic manifestations of SSc, but there are lesions of heart, lung, gastrointestinal tract and other organs like SSc.

peripartum cardiomyopathy (PPCM) is an idiopathic cardiomyopathy associated with pregnancy. Its diagnostic criteria were developed by Demakis et al. in 1971^[1]It was first proposed that the etiology and pathogenesis are still not completely clear. In 2010, the European Society of Cardiology redefined it as: a clinical disease with left ventricular systolic dysfunction and heart failure as the main manifestations, which has no previous history of cardiovascular system, mainly occurs at the end of pregnancy or within a few months after delivery, and is an exclusive diagnosis, different from dilated cardiomyopathy^[2]。 Recently, there has also been a new idea that PPCM can be seen as a syndrome rather than a disease^[3]。 Although the incidence rate of this disease is low, the mortality rate is high, which seriously endangers the health of pregnant women and even the life of mothers and infants. The clinical symptoms of PPCM are not specific, and its related exertion symptoms are similar to the pathophysiological changes related to pregnancy, resulting in clinical diagnosis failure or missed diagnosis. Therefore, after discovering suspicious cases, diagnosing and carrying out active treatment as soon as possible, the patient's heart function may completely return to normal, thus reducing the mortality rate and improving the prognosis of patients. The etiology, diagnosis, treatment and prognosis of PPCM are summarized as follows.

Eosinophilia-related diseases are a heterogeneous group of diseases with complex etiology and pathogenesis and different treatments. So far, the nomenclature and classification of such diseases is still confusing. The research progress in the naming, classification, diagnosis and treatment of this kind of diseases is reviewed.

Patients with Barrett's esophagus (BE)/columnar lined esophagus (CLE) and adenocarcinoma are increasing, in whom 0.61% BE/CLE would develop to adenocarcinoma. The prognosis of esophageal cancer is related to the tumor stage at diagnosis. To standardize the screening, diagnosis and therapy of BE and adenocarcinoma in China, 31 digestive diseases and digestive endoscopy experts and digestive histologists drafted the consensus on the basis of clinical experience and references. The consensus defined BE as a complication of gastroesophageal reflux disease. The normal distal squamous epithelial lining is replaced by columnar epithelial. The squamous-columnar junction (SCJ) is above the gastroesophageal junction (GEJ) ≥1 cm and proved by endoscopy and histology. Adenocarcinoma developing in BE mucosa is called BE adenocarcinoma. The early BE adenocarcinoma is divided into 4 stages: M1, M2, M3 and M4, according to the depth of tumor infiltration without expanding beyond mucosa. Because 90% esophageal cancers are esophageal squamous cell carcinoma (ESCC) in China, this consensus emphasizes the significance of screening BE and adenocarcinoma in esophageal cancers. The diagnosis of BE should meet the following criteria: under endoscopy, the normal distal squamous epithelial lining is replaced by columnar epithelial (SCJ is above the GEJ ≥1cm), which is confirmed by histology. The lesion should be further assessed by electron staining endoscopy such as narrow band imaging (NBI), flexile spectral imaging color enhancement (FICE), i-scan, and endoscopic ultrasonography (EUS) to choose the optimal therapy. Endoscopic resection such as endoscopic submucosal dissection (ESD) and endoscopic mucosal resection (EMR) is preferred. Radiofrequency ablation (RFA), photodynamic therapy (PDT), cryotherapy, Argon plasma coagulation (APC) are alternative therapeutic regimens yet should be administrated cautiously. The standardized histologic result is very important, which can be used to assess the response effect, further treatment and follow-up schedule. It is recommended that the follow-up would better be done with high resolution endoscope. Patients without intestinal metaplasia in the four quadrants of BE and the length <3 cm is recommended to be excluded from the follow-up. BE with intestinal metaplasia<3 cm is recommended only follow-up for 3-5 years. BE and metaplasia≥3 cm is recommended to be observed every 2-3 years.

Gitelman syndrome (GS) is an autosomal recessive, salt-losing tubulopathy caused by inactivating mutations in the SLC12A3 gene that encodes the thiazide-sensitive sodium-chloride cotransporter (NCC). GS is characterized by hypokalemic metabolic alkalosis, hypomagnesemia and hypocalciuria. GS is one of the most common inherited renal tubulopathy with a prevalence estimated at about one to ten per 40 000 people. The prevalence of GS is even higher in Asia than other countries. The majority of GS patients present mild and nonspecific symptoms during adolescence or adulthood. Common clinical manifestations are associated with electrolyte abnormalities, such as muscle weakness, salt craving and tetany. However, the phenotype of GS is highly variable and links to the quality of life. Diagnosis of GS is based on the clinical symptoms, biochemical abnormalities (normal/low blood pressure, metabolic alkalosis, hypomagnesemia, hypocalciuria and increased activity of renin-angiotensin- aldosterone system) and genetic test. Genetic diagnosis of GS is recommended for all patients and the diagnosis is confirmed when biallelic inactivating SLC12A3 mutations are identified. The differential diagnosis includes renal tubular acidosis, primary hyperaldosteronism, Bartter syndrome, Liddle syndrome and other diseases that cause hypokalemia. Among them Bartter syndrome (especially type Ⅲ) is the most important genetic disorder to consider due to its similar manifestations with GS. All GS patients are encouraged to keep high-sodium diet. Magnesium and potassium supplements (oral or intravenous) are usually given to GS patients to improve clinical symptoms. Other medicines such as aldosterone receptor antagonists, angiotensin-converting-enzyme inhibitors (ACEIs), angiotensin Ⅱ receptor blockers (ARBs) and prostaglandin synthetase inhibitors (PGSIs) are alternative choices of treating hypokalemia, but the side-effects of these medication should be well considered. Management of GS includes health education, complication evaluation and regular follow-up. Annual evaluation by a nephrologist is recommended. Extra evaluation and treatment depend on special conditions, such as pregnancy, perioperative or growth period. Antenatal diagnosis for GS is technically feasible but not recommend due to the benign prognosis in the majority of patients. In general, this expert consensus statement aims to establish an initial framework for the better diagnosis, treatment and management of Chinese patients with GS.

July 13, 2017 is an ordinary day for people living in the capital, shrouded in hot heat. On the way to work, I received a message from Xu Le, director of the Department of Gastroenterology of Beijing Hospital: Comrade Pan Qiying, former director of the Department of Gastroenterology of our hospital, died in the early hours of this morning at the age of 89 due to ineffective treatment. According to the wishes of the deceased, the funeral will be simple, and no farewell ceremony will be held. Although I was mentally prepared for Professor Pan, who was old and sick for many years, to go to the west, when the bad news came, I was still in endless grief. Looking back, the exchanges of acquaintance for more than 30 years are vivid in my mind. His image of a knowledgeable, indifferent and smiling master lingers in my mind, evoking his endless memories.

Practical Internal Medicine was published in 1952 and has a history of 65 years (Figure 1)。 The first edition was edited by Lin Zhaoqi, a former first-class professor of internal medicine at Shanghai Medical College, and compiled by well-known experts such as first-class professors Qian De, Wu Shaoqing and Yang Guoliang. It was published by East China Medical Life Society (the predecessor of People's Medical Publishing House) at that time. This book was revised to the fifth edition before the "Cultural Revolution". After more than ten years, it stopped being republished, and there were no famous books for physicians to read. Later, in order to meet the needs of readers and benefit patients, it was revised and published for the 6th time with the approval of relevant departments. Since 1993, the publication of the book has finally been on track, and it is revised every four years. The 7th edition was edited by Professor Lin Zhaoqi and Professor Dai Ziying; The 8th and 9th editions were edited by Professor Dai Ziying; Later, the 10th ~12th editions were edited by Academician Chen Haozhu; The editor-in-chief of the 13th edition added Lin Guo as a professor; The 14th edition is still led by Academician Chen Haozhu, and Professor Wang Jiyao is added as editor-in-chief; The 15th edition was edited by Academician Chen Haozhu as the honorary editor-in-chief, and by Lin Guowei, Professor Wang Jiyao and Academician Ge Junbo. The editorial board of each edition is updated to add young experts and professors. There are 9 deputy editors in the 15th edition, who are distributed in various specialties of internal medicine, with 10 academic consultants, 30 senior editorial members and 64 editorial members. They have completed the replacement of old and new, and inherited the fine tradition founded by the ancestors who participated in the compilation of Practical Internal Medicine in Shanghai Medical College. More than half a century since the first edition was published, medical science has undergone earth-shaking changes, the disease spectrum of internal medicine has changed greatly, and people's understanding of disease entities has also changed greatly. In the 21st century, facing the rapid progress in the field of modern internal medicine and the deepening of the concept of evidence-based medicine, it is very necessary to keep pace with the times and update the contents in time. The book was revised twice in 1958 and 1972. After the 7th edition (1981), each edition was updated by more than one third. In the 10th edition (1997), the catalogue of the whole book was greatly adjusted, and the basic knowledge introduction of molecular biology, medical genetics, clinical immunology, clinical microbiology, clinical epidemiology (later changed to evidence-based medicine) and clinical pharmacology, which are closely related to the development of modern internal medicine, was added, and the catalogue was stabilized at the present 24 articles. It has formed its own system and characteristic disease classification of Practical Internal Medicine. Due to many revisions, the number of words has gradually increased, from about 500,000 words in the first edition to 4.1 million words in the seventh edition, which is divided into two volumes. Since the 13th edition (2009), it has stabilized at more than 6.8 million words. In terms of format design, after the 12th edition, it was revised to a large 16-page, making it more dignified.

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