• Title/Summary/Keyword: Mixed connective tissue disease

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Pathological interpretation of connective tissue disease-associated lung diseases

  • Kwon, Kun Young
    • Journal of Yeungnam Medical Science
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    • v.36 no.1
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    • pp.8-15
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    • 2019
  • Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura. CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations. CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis. CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations. Histopathological findings of diverse morphological changes can be present in CTD-LDs airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.), interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia), pleural changes (acute fibrinous or chronic fibrous pleuritis), and vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.). CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs. Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs. A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

Osteomalacia and Hypophosphatemia Caused by Phosphaturic Mesenchymal Tumor Mixed Connective Tissue Variant (PMTMCT) -A Case Report- (골연화증과 저인산혈증을 유발한 인산뇨성 간엽성 종양 -증례 보고-)

  • Jeong, Jae-Yun;Kim, June-Hyuk;Lee, Sang-Hoon;Kim, Han-Soo
    • The Journal of the Korean bone and joint tumor society
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    • v.10 no.2
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    • pp.124-129
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    • 2004
  • The phosphaturic mesenchymal tumor mixed connective tissue variant (PMTMCT) is an extremely rare disease, and is frequently associated with oncogenic osteomalacia showing an paraneoplastic syndrome, which is characterized by phosphaturia, hypophosphatemia, normocalcemia and decreased levels of 1,25-dihydroxyvitamin D3 associated with a tumor. We experienced a 45-year-old female who had a soft tissue tumor on her right buttock causing oncogenic osteomalacia, which was satisfactorily treated by surgical excision of the mass.

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A case of mixed connective tissue disease presenting initially with Raynaud's phenomenon (레이노드 증후군으로 초기 발현된 복합 교원성 질환 1예)

  • Kim, Soo Young;Choi, Young Seok;Kim, Young Ok;Woo, Young Jong
    • Clinical and Experimental Pediatrics
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    • v.51 no.8
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    • pp.886-891
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    • 2008
  • Mixed connective tissue disease (MCTD) is characterized by diverse symptoms including rheumatoid arthritis, scleroderma, systemic lupus erythematosus, and dermatomyositis, associated with high titers of antibodies to extractable nuclear antigen (ENA), especially anti-ribonucleoprotein (anti-RNP) antibody. Since the first report of 25 cases with MCTD in adults, there have been only a few cases of MCTD reported in children. Here, we report a rare childhood case of MCTD in a 7-year-old girl presenting initially with Raynaud's phenomenon, swollen hands, and ulceration of the right index finger tip followed by alopecia and arthritis during follow-up.

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 5. Connective Tissue Disease Associated Interstitial Lung Disease

  • Koo, So-My;Kim, Song Yee;Choi, Sun Mi;Lee, Hyun-Kyung;Korean Interstitial Lung Diseases Study Group
    • Tuberculosis and Respiratory Diseases
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    • v.82 no.4
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    • pp.285-297
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    • 2019
  • Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the cooccurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjogren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.

Familial gigantiform cementoma (가족성 거대 백악종)

  • Han Won-Jeong;Kim Eun-Kyung
    • Imaging Science in Dentistry
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    • v.36 no.3
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    • pp.157-162
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    • 2006
  • Familial gigantiform cementoma is a rare fibre-cemento-osseous disease of the jaws which appears to be transmitted as an autosomal dominant trait with variable expressivity of the phenotype. A 7-year-old girl visited DKUDH complaining of the painless facial deformity. Clinically, significant facio-lingual expansion was observed at the left maxilla, left mandibular body and symphysis portion. Malposition of lower anterior teeth was found. Panoramic radiograph and CT scan showed the extensive expansile mixed lesion at maxilla and mandible. Bone scan revealed hot spot at the maxilla and left side of mandible. Histologic examination revealed moderately dense fibrous connective tissue with scattered masses resembling cementum. The patient's mother had a history of the mandibular resection due to benign tumor. Her younger brother had buccal expansion of right mandible. We report our finding of a family that has exhibited clinical, radiographic and histologic findings consistent with the familial gigantiform cementoma.

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A Literatural study on the hemorrhoids and hemorrhoids complicated by anal fistula (痔瘡과 痔瘻에 對한 文獻的 考察)

  • No, Hyun-Chan;Rho, Sek-Seon
    • The Journal of Korean Medicine Ophthalmology and Otolaryngology and Dermatology
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    • v.10 no.1
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    • pp.284-305
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    • 1997
  • A Literatural study on the etiological factors, classification, prescription of hemorrhoids and hemorrhoids complicated by anal fistula following results were obtained. 1. The cause of hemorrhoids are long time sit, long time gate, overfatigue, overeating, imbalance of stool( constipation or diarrhea), pregnant fertility(overfatigue after childbirth, insufficiency of middle warmer energy), uncontrol sexual excess, pathgenic factors of wetness, heat, wind, dry, genetic cause, excess of anxiety, pile up of heat poison, weakness of entrails and viscera. The cause of hemorrhoid complicated by anal fistula are attack of external wind, heatness, dry, fire, wetness(pathgenic factors), inapporiate treatment and chronic disease, greasy diet, excess of anxiety, constipation, uncontrol sexual excess, obstacle of circulation of vital energy and blood on anal site. 2. Classification of hemorrhoids are female hemorrhoids, male hemorrhoids, pulse hemorrhoids, intestines hemorrhoids, vital energy hemorrhoids, wine hemorrhoids, blood hemonhoids, flowing hemorrhoids. Classification with other method are external hemorrhoids, internal hemorrhoids, mixed hemorrhoids, excrescence hemorrhoids, nipple homorrhoids. External hemorrhoids is classified of varicosis of hemorrhoidal vein, connective tissue form, thrombus form. Classification of hemorrhoid complicated by anal fistula are simple lower hemorrhoid, lower mixed hemorrhoid, deep hemorrhoid, outer of one hole hemorrhoid, a horseshoe hemorrhoids. Once more classificated of four are space of sphincter muscle form, penetration sphincter muscle form, upper of sphincter muscle form, outer of sphincter muscle form. 3. Therapy method of hermorrhoid and hemorrhoid complicated by anal fistula are internal method, fumigation method method, ointment, method of close with medicine, necrotizing method, hot medicated compress( gxternal method), injection, insertion, bind, (operation) and acupuncture therapy (the others method) 4. Herb medicine for many used of internal method are Scutellaria baikalensis George(黃芩), Coptis japonia Makino(黃連), Rehmania giutinosa Liboschitz ex Fischer & Meyer(生地黃), Poncirus trifoliata Refinesque(枳殼), Sanguisorba officinalis Linne(地楡), Sophora japonica L.(槐花), Cnidium officinale Makino (川芎), Astragalus membranaceus Bunge(황기), Angelica gigas Nakai (當歸). 5. Herb medicine for many used of fumigation are Schlechtendalia Chinesis J. Bell (五倍子), Artemisia Vulgaris L. var indica Maxim(艾葉), Poncirus trifoliata Refinesque (枳殼), Nepeta japonica Maximowicy(荊芥), And herb medicine for many used of ointment are Calomelas(輕粉), Alum(白礬), Boswellia carterii Birdwood(乳香), Os Draconis Fossilia Ossis Mastodi(龍骨).

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Herpes Simplex Virus Pneumonia in Immunocopmromised Host (면역저하 환자에서 발생한 단순포진바이러스 폐렴)

  • Kim, Jin-Ku;Lee, Choong-Hyun;Kang, Kyeong-Woo;Seo, Gee-Young;Chung, Man-Pyo;Kim, Ho-Joong;Kwon, O-Jung;Rhee, Chong-H.;Han, Joung-Ho
    • Tuberculosis and Respiratory Diseases
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    • v.46 no.1
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    • pp.82-88
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    • 1999
  • Herpes simplex virus pneumonia in immunocompromised host is difficult to diagnose with non-invasive method, and has high mortality rate. Because early diagnosis and early treatment can significantly decrease the mortality rate, the enthusiastic efforts for the early diagnosis should be done. A 41-year-old woman who took prednisolone due to mixed connective tissue disease developed gradually increasing dyspnea with radiological features of interstitial lung disease. Initially, we treated her with empirical antibiotics, but failed to improve her dyspnea. So we performed bronchoalveolar lavage and open lung biopsy. Open lung biopsy specimen showed herpes simplex virus pneumonia. Herpes simplex virus was also isolated from bronchoalveolar lavage fluid. There was both clinical and radiological improvement after treatment with acyclovir for 14 days.

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Awareness of the Causes of Drug-Induced Liver Injury: A Case of Hepatotoxicity Resulting from Antipsychotics (사례로 본 한방임상에서 양약으로 인한 약인성간손상에 대한 인식 필요성)

  • Chang-gue Son
    • The Journal of Internal Korean Medicine
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    • v.44 no.4
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    • pp.751-756
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    • 2023
  • Objective: This study attempts to increase awareness of hepatotoxicity caused by antipsychotic drugs and to provide updated information on drug-induced liver injury (DILI) to physicians in Korean medicine (KM) clinics. Methods: This study presents a detailed case of a female patient diagnosed with DILI attributed to antipsychotic drugs, highlighting the improvement observed through laboratory findings. Results: A 56-year-old female patient with underlying disorders, including mixed connective tissue disease and depression, was under medical care. One day, she reported experiencing intense fatigue and distressing sensations, prompting the author to order blood tests. The levels of AST and ALT were significantly elevated by more than 2.5-fold, indicating hepatocellular DILI. The RUCAM score for antipsychotic drugs was 9, as no other medications, including herbal medicine, were being taken. Upon discontinuation of the antipsychotic drugs, the patient's laboratory findings returned to normal levels within 2 weeks, accompanied by a recovery of subjective symptoms. Conclusion: This study presents a noteworthy case of hepatotoxicity caused by antipsychotic drugs, serving as an illustrative example that highlights the crucial need for awareness among doctors of KM in clinical settings.