Mao-Yuan Chen National Taiwan University Hospital

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Mao-Yuan Chen National Taiwan University Hospital

  1. 1. 中華民國免疫學會暨風濕病醫學會 臨床病例討論會 時間:民國 91 年 3 月 30 日(星期六)下午 2:00-4:00 地點:台大醫學院一樓第一0三講堂 主持人:臺大醫院過敏免疫風濕科 余家利 主任 演講者及題目 1. Hypothyroidism Mimicking Polymyositis in A Patient with Systemic Lupus Erythematosus 陳茂源 醫師 2. Pilocarpine in The Treatment of Sjogren’s Syndrome 謝松洲 醫師 3. A Young Man with Intermittent High Fever for Half Year, Diffuse Lymphadenopathy and Peripheral Eosinophilia 吳建陞 醫師 主辦單位:臺大醫院過敏免疫風濕科
  2. 2. 協辦單位:台灣諾華股份有限公司 2
  3. 3. Hypothyroidism Mimicking Polymyositis in A Patient with Systemic Lupus Erythematosus Mao-Yuan Chen National Taiwan University Hospital The 67-year-old female patient was noted to have thrombocytopenia and leukopenia in 1984. Systemic lupus erythematosus was diagnosed based on positive antinuclear antibodies (ANA) and low serum complement level. She was treated at hematological department with prednisolone 1mg/kg/day. The 3 platelet count stabilized at around 100k/mm and the dosage of steroid was tapered gradually to 5-10 mg every other day. She was referred to endocrinologist and rheumatologist in 1997 because of lethargy and myalgia. Hypothyroidism was disclosed by high TSH (42.4 uIU/ml) and low T3,T4 serum level ( 39.7 ng/dL and 1.19 ug% respectively). The thyroid autoantibodies were all positive, including anti-thyroglobulin, anti-thyroid peroxidase (anti-microsomal antibodies) and thyroid receptor antibodies. The ANA was 1280x with positive anti-Ro antibodies. Low C3 (59 mg/dL) and C4 (15 mg/dL) were also found. The rheumatoid factor was positive (RAHA 320x). Borderline low serum cortisol level was noted. No lupus activity was found except mild symptoms of sicca syndrome. She was treated with L- thyroxine 100 ug/day and prednisolone 5mg/day with relief of symptoms. In 3
  4. 4. May 1999, follow-up rheumatological profile showed decreased ANA and RA titer (1:80 each), normal C3 and C4 level (97 and 21 respectively) and negative anti-DNA and ENA. The creatine kinase (CK) and hemogram were normal. But for unknown reason, L-thyroxine was no longer prescribed to her since late 2000. She began to suffer from numbness and aches in lower limbs especially in pelvic girdle muscle since August 2001. Aggravation of muscle pain was noted on walking. She was admitted in September because of persistent muscle pain and progressive proximal weakness. Blood chemistry revealed high creatin kinase level (1052 U/L) and the possibility of polymyositis was considered. At the same time, endocrinologist noticed that the patient had low-pitch hoarse voice and he discovered the missing prescription of thyroxine. The free T4 was 0.16 and TSH was >75 U/mL. After the reinstitution of thyroxine, her symptoms improved gradually. 4
  5. 5. Pilocarpine in The Treatment of Sjogren’s Syndrome Song-Chou Hsieh National Taiwan University Hospital Sjogren’s syndrome (SS) is characterized by symptoms of dryness of the eyes and clinical findings of keratoconjunctivitis. Patients with SS also have the dryness of the mouth associated with focal lymphoid infiltrates of the salivary glands. Sjogren’s syndrome may exit as a primary condition or be associated with other autoimmune disease (secondary SS), such as rheumatoid arthritis, systemic lupus erythematosus, myositis and scleroderma. Since Sjogren’s syndrome is an autoimmune disease, one of the initial approaches to treatment was to use various immunosuppressive drugs to treat the dry mouth and dry eyes and try to prevent complications. Except for alpha-interferon still in clinical trials in USA and Japan, all these other immunosuppressive treatments have really been ineffective. They are used to treat the arthritis, lung disease and other complications of SS but not the dry eye and dry mouth. The greatest innovation in this filed during the last ten years has been the idea to use a secretagogue to treat this disease. In Sjogren’s syndrome, there is a deficient secreatory response of lacrimal and salivary glands that leads to the symptoms of dryness. This decrease in 5
  6. 6. salivary and lacrimal flow is likely to be multifactorial, but it involves both a decrease in the number of secretary units and a dysfunction of the residual secretory units. Despite destroyed secretory units by perforin/granzyme A mechanism or by Fas/Fas ligand mechanisms, only about half of the acinar or ductal structures are destroyed in SS patients with long-standing symptoms of severe dryness. It is likely that multiple factors contribute to the diminished function of residual secretory units. Investigations suggest that factors related to lymphocytic infiltration and chronic inflammatory response prevent function and/or communication of residual of the neural and the residual acinar/ductal elements. Taken together, these results support that decreased secrtory function in Sjogren’s syndrome is not the result of destruction of neural innervation in the entire glandular lobule or the absence of muscarinic receptors for neuropeptides on the residual acini/ducts. In the meanwhile, excess of muscarinic receptors may provide a mechanism for amplifying the cholinergic nerve signal, allowing excess acetylcholine from the presynaptic nerve endings to spill over onto the additional receptor sites. These excess receptors may be the therapeutic targets for analogs of acetylcholine. Two muscarinic agonists (pilocarpine and cevimeline) have recently been approved for the treatment of symptoms of xerostomia in Sjogren’s syndrome. 6
  7. 7. These agents stimulate the M1 and M3 receptors present on salivary glands, leading to increased secretory function. A Young Man with Intermittent High Fever for Half Year, Diffuse Lymphadenopathy and Peripheral Eosinophilia Chien-Sheng Wu National Taiwan University Hospital The 19-year-old man has suffered from intermittent high fever with chills, mild cough and general malaise since late Aug, 2001. He had his graduate tour to southern Taiwan (墾丁、高雄) on July 11, 12, 13. Intermittent fever was noted since late Aug, 2001. Enlarging painful left inguinal lymph nodes with fever and chills developed in mid-Oct. Some non-itching fresh-color wheels were noted. He was admitted to MGMH on Oct 24. During admission, anemia with eosinophilia was noted. Lymph node biopsy revealed chronic lymphadenitis. The result of TB PCR was positive and anti-TB drug was given since Nov 11, 2001. Itching macular (about 3 mm) rash over four limbs and trunk appeared two week after anti-TB drug and drug allergy was suspected. But fever persisted and new lymphadenopathy developed despite anti-TB drug was in use. Chest CT in MKMH showed diffuse mediastinal and hilar lymphadenopathy as well as multifocal hypodense lesions in spleen. He was 7
  8. 8. admitted to this hospital on Jan 23, 2002. After admission, intermittent spiking fever (up to 40℃) with chills was noted. There was no more skin itching. NSAID was given. Lymphadenopathy became more diffuse, which involved bilateral inguinal areas, bilateral neck and submental area. Complete blood count showed Hb 8.8 g/dl, WBC 8250/μl with 22% Eosinophils and platelet 268K/μl. LDH was 952 U/L. Initial CRP was 2.35 mg/dl. C3 and C4 were 165 and 29.7 mg/dl, respectively. Other serology study such as ANA, rheumatoid factor was all within normal limit. Serum protein electrophoresis showed increased α1 and α2 globulins. IgA, IgG, IgM were 48.2, 1250 and 64.5 mg/dl. Lymph node biopsy was done. Prednisolone 30 mg per day was given due to persisted fever under NSAID use. Lymph node pathology revealed follicular hyperplasia, extensive eosinophils infiltration and vasculization, and serum total Ig E was 805.64 IU/ml, which was compatible with Kimura’s disease. Fever subsided under steroid and lymph nodes became soft. He was discharged for OPD follow-up. 8
  9. 9. 中山醫學大學附設醫院 (醫學中心) 誠 徵 風濕免疫科醫師 資 格:具風濕免疫專科證書,有教職優. 聯絡電話:(04)2472-7445 電子郵件:gjt@csmu.edu.tw 9

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