新生兒科 風濕免疫 黃副考古 DAY1

1.SLE 病人的Iniatial event是甚麼?

   先複習SLE診斷口訣: MD SOAP BRAIN
M:蝴蝶斑
D:盤狀紅斑
S:漿膜炎 O:口腔潰瘍(不會痛，所以要主動檢查病人) A:抗核抗體 P:光敏感
B:血液疾病(貧血/百血球過低<4000/淋巴球過低<1500/血小板過低<100000)
R:腎臟疾病:蛋白尿
A:關節炎(紅腫熱痛)
I:免疫問題:anti-DNA/anti-SmN
N:神經疾病

From Torondo 2012


大多數一開始的表現是:發燒Fever / 疲累Fatigue / 關節痛Arthritis / 蝴蝶斑 Malar rash

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林口長庚11月教育櫥窗解題

Solver: Weitinglin

   A 58-year-old female patient visited to your clinic. Her chief complaint was polyarthralgia over 

her bilateral wrists and PIPs joints for more than 3 months. The associated symptoms included 

severe morning stiffness more than one hour. No fever or other discomfort was found. She had 

lymphoma history 4 years ago. But after chemotherapy, lymphoma is complete remission. Her 

physical examination revealed swelling and tenderness over her bilateral wrists, left 2nd, 3rd 

         PIPs and right 4th PIP joints (Figure 1). Lab showed ESR 108mm/Hr, CRP 76.76 mg/L, RF    84.60 

IU/ml, ANA 1:80 (anti-nuclear membrane Ab). Disease activity score 28(DAS28): 6,28. Hand 

X-ray showed as figure 2 

1. Since high disease activity was found after standard DMARDs, NSAID and steroid therapy. You need to consider biologic agents for this patient. What kinds of biologic agents for rheumatoid arthritis are available in Taiwan? In your opinion, what is your best biologic agent for this patient?

-目前健保有給付之六種治療類風濕性關節炎的生物製劑  Etanercept(恩博)/Adalimumab(復邁)/Golimumab(欣普尼)/Abatacept(恩瑞舒) =>用於傳統化學抗風濕病藥物治療無效後的患者  Rituximab(莫須瘤)/Tocilizumab(安挺樂) =>用於前三種抗腫瘤患死因子之生物製劑無效時使用 Ans:Etanercept/Adalimumab/Golimumab/Abatacept/Rituximab/Tocilizumab are available in Taiwan now. -依照目前台灣健保局的規定，必須先使用DMARDs充分治療後失敗才能使用生物製劑，其對於充分治療的定義為:   i.DMARDs藥物治療時間須符合下列條件之一： (i)必須至少6個月以上，而其中至少2個月必須達到 (附表十四) 所示標準目標劑量 (standard target dose)。 (ii)DMARDs藥物合併使用prednisolone 15 mg/day治療，須至少3個月以上，而其中至少2個月DMARDs藥物必須達到 (附表十四) 所示標準目標劑量 (standard target dose)。(100/12/1) ii.若病患因DMARDs藥物毒性無法忍受，以致無法達到上項要求時，DMARDs劑量仍需達 (附表十四) 所示治療劑量 (therapeutic doses) 連續2個月以上。 -因這名病患有lymphoma histtory，雖然已經remission，但考慮到可能flare up，目前研究顯示RA病人其獲得淋巴增生性疾病機率是一般人的兩倍，尤其是在類風濕性疾病嚴重性較高的病人身上，而治療類風濕性疾病的藥物像是azathioprine, MTX, 以及anti-Tumor necrosis factor的第一線生物製劑都會提高風險。考慮到這因素可能推薦這病患使用第二線生物製劑:Rituximab/Abatacept/Tocilizumab，而Retuximab本身可用在治療non-Hodgkin lymphoma的治療(不知道這病人的lymphoma是哪一個type)，或許Retuximab是目前最推薦給這名病患使用的。 Ans: the recommendation bioagent for this patient is          Retuximab

2. What is the risk management plan before starting the biologic agents treatment according to Taiwan rheumatology association recommendation?

使用生物製劑後最重要的問題是會提升病人的感染機率，根據台灣風濕病醫學會提出的風險管理建議，主要是爭對結核感染症及B型肝炎。   B型肝炎的風險管理: 使用藥物前進行 -肝功能評估與:AST、ALT、total bilirubin、Prothrombin time、腹部超音波及血清alpha-fetoprotein檢查 -HBV感染篩檢:HBsAg、Anti-HBs Ab、Anti-HBc、Ab-IgG，假如HBsAg為+的話，進一步檢查，HBe Ag、HBe Ab、HBV viral loads   HBsAg(+)且併有症狀或肝功能檢查異常者 HBs-Ag(+)但肝功能檢查正常者              不建議使用生物製劑 使用生物製劑前1-2周口服抗病毒藥物，entevavir/tenofovir為首選 HBs-Ag(-)但HBc Ab-IgG(+) HBs-Ag(-)、HBc Ab-IgG(-)但Anti-HBs            Ab(+) 可能為HBV潛伏感染，須於使用生物製劑前，檢測HBV病毒量，無檢測到則可使用生物製劑 有免疫，可開始使用生物製劑 HBs-Ag(-)、HBs-Ab(-)、HBc   Ab-IgG(-) 沒有免疫力，先接踵B肝疫苗，再進行生物製劑治療 結合感染風險管理 活動性肺結核患者是不能接受生物製劑治療，故必須在事前篩檢是否有活動性結核以及潛伏結合感染。 -篩檢活動性結核: 1.病史(咳嗽、痰、發燒超過三週、接觸史) 2.肺部X光檢查 3.檢驗三套痰液耐酸桿菌塗片與痰液TB培養 -潛伏結核感染 1.QuantiFERON試驗 假如有POSTIVE，進行治療，INAH 300mg QD for 9 months/rifampin 10mg/kg/day for 4 months 以上為使用生物製劑前的風險管理根據台灣風濕免疫協會

3. The hand X-ray revealed obvious peri-articular osteoporosis. You arranged Dual-energy X-ray absorptiometry(DXA) for her which demonstrated as figure 3 . Can you explain to this patient about her 10 years osteoporotic fracture risk? ( patient basic data: weight 55kg, height 154cm, No alcohol or smoking history, no previous fracture history or parents fracture history, DXA machine: GE-lunar)

臨床上骨折的風險主要由兩個因子去評估: 1.BMD骨質密度(可由DXA測得) 2.臨床風險因子:包括年紀/骨折病史/類固醇治療/家族父母骨折史/低體重(<58kg)/吸菸/喝酒/類風溼性關節炎/營養不良 而在DXA測量結果中可以看到T-score，T-score是以20歲白人女性的平均骨密度作為標準0，去比較而得。 T-score 0~-1 Mild osteopenia -1~-2.5 Osteopenia,須開始預防治療 -2.5~ Osteoporosis，須開始治療 可以看到病人整體而言，T-score=-2.4，幾乎是borderline，需要積極預防或是治療。 而關於病人骨折的風險其臨床危險因子應該有年紀、低體重，其他都不算高。 目前用來估算病人十年的骨折率可以利用WHO fracture Risk Assessment Tool來估算。 根據WHO的骨折風險評估工具計算結果其T-score為-2.9，對其上的表大約可以估算這病人未來十年的骨折機率是30%。

4. According to Taiwanese guidelines for prevention and treatment of osteoporosis, does this patient need to receive osteoporosis treatment? If needed, what kind of treatment do you provide to this patient?

此病人已接受類固醇治療三個月(每日使用Prednisolone超過5mg)且為骨質疏鬆症患者，依WHO Fracture risk tool評估，十年內骨折機率約30%，為高風險骨折病人，根據台灣骨質疏鬆症學會的指引，需要接受骨質疏鬆的藥物治療。   除了1.跌倒預防2.加強衛教3.定期追蹤外，4增加飲食中鈣質與維生素D攝取，5保持體重，6避免菸酒，7規律運動，在使用類固醇的高風險骨折患者，推薦使用雙磷酸鹽類的Alendronate或Zoledronic acid，或是造骨細胞刺激藥物Teriparatide，但一次只能使用一種藥物。

    

參考資料:

1.      黃文男, 類風溼性關節炎之生物製劑治療,台灣免疫風濕疾病關懷協會第五期會刊

2.      李彩蓮, 生物製劑與健保給付的拔河, 第1813期, 藥師週刊

3.      全民健康保險藥品給付規定102年

4.      John H Stone, Tumor necrosis factor-alpha inhibitors: Risk of malignancy, Oct 2013, UpToDate

5.      Peter H Schur, Malignancy and rheumatic disorders, Oct 2013, UpToDate

6.      洪維廷, 使用生物製劑患者之風險管理, 台灣免疫風濕疾病 關懷協會 會刊 第五期

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林口長庚10月教育櫥窗解題

Solver:WEITINGLIN

Case I

General data

Age:53 Gender: Female

Chief Complaint

Epigastric pain for 1 week with blackish formed stool

Presence illness

53-year-old female suffered from epigastric pain for 1 weeks with tarry stool.She went to LMD, taking the examination of EGD showed only superficial gastritis. However, the patient had blackish stool again and came to our hospital.   After admission, normocytic anemia was noted. EGD revealed tiny erosions at the duodenal bulb, and then colonoscopy exhibited lots of blood clots retained at cecum and no blood clots could be seen at the terminal ileum. Because of no obvious bleeder, double-balloon endoscopy was done via the oral route, and one ill-defined mucosal nodular lesion was noted in the jejunum.   The patient has no systemic disease and she has the history of GI bleeding with unknown origin 9 year ago.

1.  What is your tentative diagnosis?

My tentative diagnosis is Inflammatory bowel disease(IBD),especially may be Crohn’s disease.

The 53-female-old patient in menopause age has the presentation of the tarry stool and abdominal pain. According to the description, it seems not a active GI bleeding situation without the unstable vital sign and specific physical finding. The normocytic anemia showed the fact of blood loss. Furthermore. the EGD and colonoscopy roughly rule out some of the upper GI problem such as Peptic ulcer, Varices , Erosive esophagitis/ulcer, Mallory-Weiss tear, Vascular lesions, Diverticular hemorrhage, Angiodysplasia.the finding by EGD and double-balloon show the two lesions at duodenal bulb and the ill-defined mucosal nodular lesions. The image show nodular thickening of mucosa which may look like the appearance of cobblestone.   Under the impression of the Inflammatory bowel disease. The pattern of the disease more like Cronh’s disease, which non-bloody stool, lesions mainly were found in the intestine, uncontinous(one in the duodenal bulb, the other in the jejunum),the endoscopy finding of the cobblestone appearance nodular thickening lesions.   The biopsy of the lesions and serology examination (pANCA，ASCA) can be worked out. The cross-section CT and MRI can evaluate the luminal narrowing or extra-luminal complications(abscess and fistula).   The possibility of TB infection and Tumor still should be ruled out by Chest X ray, Tuberculin test and pathology report.   Also need to take the complete history about drug usage(NSAIDS,Aspirin,alcohol),contact history。

2.  Could other exams be done for survey obscure GI bleeding except EGD, colonoscopy, and double-balloon enteroscopy?
 If other exams could be done before double-balloon enteroscopy?

1.There are also survey such as Small bowel series ,Radionuclide scanning and Angiography can be done to evaluate the obscure GI bleeding -Small bowel series >ingestion of diluted Barium to obtain the information of the small bowel image such  Stricture >not good at detecting ulcer - enteroclysis >injection Barium, methylcellulose and air in the proximal small bowel to obtain image >patient feel uncomfortable -Radionuclide scanning(Tc 99m RBC bleeding scan ) >high sensitive and noninvasive >can not localize very specific -Angiography >unless the patient have severe bleeding >can do intervention

2.Before the double-balloon endoscopy, if we want to evaluation the small intestine. The small bowel Barium series and Tc-99m RBC bleeding scan. Both examinations are well to survey the condition of the small bowel. Another alternative way is to have wireless video capsule endoscopy. It is very sensitive to detect the blood loss of the intestine

Refernece: 1.長庚大學醫學六內科上課講義
         2. MGH pocket medicine 4^th ,3-3,3-10
         3.The Washington manual of Medical therapeutics 33^rd,P577-583
         4.UpToDate,”Differential diagnosis of abdominal pain in adults”
                   “Evaluation of obscure gastrointestinal bleeding”
        

CASE II

General Data

Age:16 Gender: female

Chief complaint

Intermittent abdominal dull pain in LUQ > 1 month

Present illness

This 16-year-old female suffered from intermittent abdominal dull pain in LUQ for more than 1 month. There were no nausea, vomiting, diarrhea, constipation, bloody stool, fever, and skin rash, and no relationship to food intake or posture change was noted. Because abdominal pain persisted, she was admitted for further survey.   After admission, microcytic anemia (Hb 6.3 g/dL; MCV 66.3 fL) were noted. EGD showed some small (0.2-0.3 cm) polyps in the stomach and duodenum, and colonoscopy revealed no abnormal finding. Small bowel series showed some small bowel polyps, and double-balloon enteroscopy disclosed a big (3-4 cm) wide based polyp at proximal jejunum (about 50 cm away from the bulb) and piecemeal resection was done.   The patient has history of (1) gastric polyp, status post polypectomy, and (2) intussusception, status post resection.

1.  What is your tentative diagnosis?

My tentative diagnosis in this case is Peutz-Jeghers syndrome

This is a 16-year-old female with multiple polyps in her gastrointestinal tract. The patient’s chief complaint is the symptom of the intermittent LUQ abdominal pain. There were no nausea, vomiting. It may not like the disease associated with hepato-biliary system. There were no diarrhea and fever, it may not like microbiology infection. There is no constipation, it may no be obstruction or bowel motion problem. There is no bloody stool, it may not like GI bleeding. There is no skin rash, and no relationship to food intake or posture change. It may not like pancreas disease.   It’s very rare that the presentation of the multiple polyps in gastrointestinal tract in this young age. It may be the clinical manifestation in Familial adenomatous polyposis,MYH-associated polyposis , Peutz-Jeghers syndrome, Juvenile polyposis. Because the colonoscopy revealed no abnormal finding, the disease will look more like Peutz-Jeghers syndrome. And the patient has the history of intussusception, which have 43% possibility to be happened in the patient of Peutz-Jeghers syndrome. The patient also have microcytic anemia. It may be connected to the disease. Because the polyps in the intestinal tract may affect the normal iron absorption which may lead to iron deficiency anemia.   The biopsy of the polyps , physical examination and the family history will be very important to do the differential diagnosis in this patient. Also need to have the evaluation of the complete blood count and iron studies.   If needed, can do STK11 gene testing to check whether or not mutate.

2.  If something important could be acquired in the history and physical examination?

What the risk should we take care in the patient’s future life?

The family history of this patient need to be taken carefully. Because this disease is autosomal dominant disease, there must be also the same disease existed in her family. If the patient’s parents or relative have such relation disease, it need to know that the onset, clinical manifestation of the relation disease.   If the patient have the Peutz-Jeghers syndrome, the physical examination may have the finding of the pigmented spots in lip and perioaral region, hand.

The patient with Peutz-Jeghers syndrome is associated with an increased risk of gastrointestinal and nongastrointestinal malignancies.   The risk of gastrointestinal cancer in this patient was between 38-66 percent.The most common sites were Colorectal(38%), Stomach(29%), Small bowel(13%) ,Pancreas(11-36%)。   In female, the patient have an increased incidence of gynecological cancer(13-18%)。The most common sites are breast(32-54%), ovary(21%), and cervix(10%).   There are some surveillance we can do to follow up this patient. First, Regular endoscopy examination should be done. The endoscopic polypectomy should be performed , if the sizr of the polyps > 1cm。The video capsule endoscopy can do every three years.   Monthly breast self-examination are advised.And between the ages of 25-50 years can follow up with annual breast NRIs.   Besides the follow up for the patient, there are advised to do first degree relatives screening with an history, physical examination, evaluation for melanotic spots.

Refernece:

  1.UpToDate,”Overview of Peutz-Jeghers syndrome”
“Clinical manifestations and diagnosis of familial adenomatous polyposis “Peutz-Jeghers syndrome and juvenile polyposis: Screening and management of      

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