DOCSLIB.ORG

Behçet's Disease Associated with Malignancies. Report of Two Cases

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Behçet's Disease Associated with Malignancies. Report of Two Cases Behçet’s disease associated with malignancies. Report of two cases and review of the literature V.G. Kaklamani1, A. Tzonou2, P.G. Kaklamanis3 1Feinberg School of Medicine, Northwest- ABSTRACT Introduction ern University, Chicago, USA; 2Depart- Objective. To investigate the incidence Behçet’s disease (BD) is a chronic, re- ment of Hygiene and Epidemiology, Med- of malignancies in a cohort of Behçet’s lapsing multi-system disorder. T h e ical School, Athens University, Greece; disease patients and review the world principal manifestations are: oral apht- 3Department of Rheumatology, Athens Medical Center, Athens, Greece. literature. hous ulcers, genital ulcers, skin lesions, Methods. Our database of 128 patients eye, joint, neurological and vascular Vir ginia G. Kaklamani, MD, DSc, As s i s t a n t Professor in Haematology/Oncology; was searched and the age standardized manifestations (1-3). Rare clinical find- Anastasia Tzonou, Associate Professor, rate (ASR) for cancer was calculated. ings include: cardiac, pulmonary and De p a r tment of Hygiene and Epidemiology; F u rt h e r m o re, we performed a MED - renal disorders (1-3), as well as, epidi- Phaedon G. Kaklamanis, MD, Emeritus LINE search from 1970 through 2003, dymoorchitis (4). The epidemiology of Professor of Internal Medicine. as well as, a search in the proceedings BD in most parts of the world has re- Please address correspondence to: of international conferences for cases cently been reviewed (5-7). The patho- Phaedon Kaklamanis, MD, 61 Ipsilantou of malignancies associated with Beh - genesis of the disease has not been elu- St., Athens 11521, Greece. çet’s disease. cidated (1,2,8), although genetic fac- E-mail: Virginia @hol.gr Results. Two of our 128 patients with tors, environmental agents and immune Received on September 30, 2004; accepted B e h ç e t ’s disease were found to have aberrations have been implicated (1, 2, in revised form on April 1, 2005. solid tumors. One male had lung can - 9). Immune abnormalities, particularly Clin Exp Rheumatol 2005; 23 (Suppl. 38): cer and the other female had kidney increased production of some antibod- S35-S41. cancer. The ASR for cancer cases in ies have been reported in patients with © Copyright CLINICALAND EXPERIMEN- our population was investigated and it BD (9). However, Raynaud’s phenom- TAL RHEUMATOLOGY 2005. was found to be 1,600 per 100,000 in enon, polyserositis, Sjögren’s syndro- Key words: Behçet’s disease, 10 years. The ASR for cancer cases in me, neutrophil, antiphospholipid and malignancies. Greece according to WHO is 272.51 antinuclear antibodies, which charac- per 100,000 per year and there f o re terize an autoimmune disease, have not 2,725 per 100,000 in 10 years. been associated with BD (10,11). Sev- In the world literature 112 cases of ma - eral studies have shown that arterial and lignancies associated with Behçet’s di - venous involvement of all size vessels sease were found: Sixty five cases were are characteristic findings of the dis- of male patients and 46 of female with ease (12-16). Given the above vas- 1 case of unknown gender. The solid culitic manifestations, BD is classified malignancies associated with Behçet’s among the wide spectrum of systemic disease included cases of bladder, vasculitides (14). b reast, uterus, thyroid and stomach In autoimmune and vasculitic disorders cancer, whereas haematological malig - the risk of malignancies has been ex- nancies included leukemia, myelodys - tensively studied. In patients with rheu- plastic syndrome, lymphoma, multiple matoid arthritis (RA), both solid and myeloma, Hodgkin’s disease and lym - haematopoietic malignancies have been phosarcoma. The treatment administer - reported (17,18). In Western Europe ed in these patients with their disease is and in the United States the risk is re- also reported. lated primarily to the development of Conclusion. The age standardized rate haematopoietic neoplasms, whereas in of cancer in our population was lower Japanese patients oropharyngeal malig- than that of the general population in nancies are more commonly seen (19). G reece, although the difference was The discrepancy may have to do with not statistically significant. However, environmental and dietary differences there is discrepancy in the world litera - between the different regions. Patients ture and the possibility of development with systemic lupus erythematosus of malignancies in Behçet’s disease pa - (SLE) have increased risk of develop- tients should not be ignored. ing both solid tumors and haematologic S-35 Behçet's disease and malignancies / V.G. Kaklamani et al. malignancies (19-23). In patients with genital ulcers appeared. These lesions of search headings of «Behçet’s dis- progressive systemic sclerosis (sclero- were recurrent. Pathergy test was posi- ease», «Behçet’s syndrome», «Adaman- derma) increased risk of malignancy tive and the diagnosis of BD was estab- tiades-Behçet’s disease», «cancer» and was found (24), particularly bronchoal- lished. He was treated with colchicine «malignancy». Cases from proceedings veolar carcinoma (23). The incidence and prednisolone 30 mg/d which was of international conferences in BD of malignancy in dermatomyositis and tapered slowly to 7.5 mg/d. Since then were also included in the analysis. polymyositis (DM,PM) has been the every 3-4 months most of the clinical subject of multiple publications (25- manifestations would recur and the Statistical section 29). Among 618 patients with DM and dose of prednisolone would be increas- We wanted to determine whether the PM, 198 cases of cancer were reported ed. The time of cancer diagnosis he incidence of malignancy in our BD pop- (26), pointing toward a predisposition presented with small oral aphthae, one ulation was similar to that of the gener- to the development of malignant disor- genital ulcer and arthritis of the right al population. For this we had to stan- ders in such patients. Wegener’s granu- knee. In March 1999 he presented with dardize our population since the age di- lomatosis (30), Henoch-Schönlein pur- neurological symptoms and a brain stribution of our population was not si- pura (31) and other vasculitides (32) MRI showed a solid mass measuring milar to that of the general population. have also been associated with increas- 3.5 cm at the lower left parietal area. We calculated the age standardized rate ed risk of malignancies. In BD patients This was a metastatic tumor originating (ASR) for cancer cases in our popula- more than hundred cases associated from his lung and a biopsy of the lung tion which was 1,600 per 100,000 in 10 with malignancies have been publish- tumor showed to be a microcytic type years. We used the following formula ed. However, the direct relationship adenocarcinoma with large cells. The for calculating ASR: ASR = S cases with BD has not been proved. patient was treated with carboplatin, (age group)/population (age group) x The development of malignancies in paclitaxel and radiotherapy. Several 100,000 world standard population autoimmune diseases and vasculitides months later he died. The patient devel- (age group)/world standard population. could be related to abnormalities in im- oped lung cancer 10 years after the According to the WHO the ASR for mune regulation, T cell deficiency, or diagnosis of BD. He had a 40 pack/year cancer cases in Greece is 272.51 per the overproliferation of B cells, which smoking history. 100,000 per year and therefore 2.725 may trigger a malignant transformation per 100,000 in 10 years (36). After per- of cells (33). The association of malig- Case 2 forming a c 2 test to assess the signifi- nancies with BD may represent a para- A 60-year-old housewife at the age of cance, we found that there was no sta- neoplastic syndrome (34) or may be re- 17 developed oral and genital ulcers. tistically significant difference between lated to the above mentioned distur- They were followed by arthritis of the the two populations. Furthermore, we bances (33). Furthermore, medications lower extremities. At the age of 20 calculated the annual incidence of ma- that BD patients are chronically on those symptoms recurred several times lignancy in our population of 128 pa- may also predispose to the develop- and anterial uveitis was diagnosed. tients with BD and found it to be 156 in ment of neoplastic diseases. Five years later erythema nodosum and 100,000. Our primary aim in this report is to recurrences of oral and genital ulcers share our own experience on the asso- developed. Pathergy test was negative Review of the literature ciation of malignant neoplasms and BD and she had HLA B5 (51). The dia- A variety of solid tumors have been re- by searching our database of 128 pa- gnosis of BD was established and be- ported in the world literature including tients with a median follow up of 10 gan treatment with prednisolone 30 two of our own. The diagnosis of BD years. Furthermore, we will review the mg/d and chlorambucil 6-10 mg/d for 3 was established according to the crite- cases of malignancies associated with years. Two years later hypopyon iritis ria reported (96). In a few cases the dis- BD present in the scientific literature. was diagnosed and received predni- ease onset was defined as the time solone 20 mg/d and azathioprine 100- when the patient developed the first Material and methods 200 mg/d for 8 years. At the time of symptom, while in others when the dia- Our BD patient cohort has been pros- diagnosis of cancer she suffered from gnostic criteria were fulfilled. pectively collected since 1990, during chronic uveitis, recurrent erythema no- which time we have enrolled 128 pa- dosum and oral aphthae. At the age of 1.
Load more

Recommended publications
  • Does Previous Corticosteroid Treatment Affect the Inflammatory Infiltrate Found in Polymyositis Muscle Biopsies? M.M
    Does previous corticosteroid treatment affect the inflammatory infiltrate found in polymyositis muscle biopsies? M.M. Pinhata1, J.J. Nascimento1, S.K.N. Marie2, S.K. Shinjo1 1Division of Rheumatology, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil; 2Laboratory of Molecular and Cellular Biology, Department of Neurology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. Abstract Objective The aim of the study was to evaluate the effect of the prior use of corticosteroids (CS) on the presence of inflammatory infiltrates (InI) in muscle biopsies of polymyositis (PM). Methods We retrospectively evaluated 60 muscle biopsy samples that had been obtained at the time of the diagnosis of PM. The patients were divided into three groups according to the degree of the InI present in the muscle biopsies: (a) minimal InI present only in an interstitial area of the muscle biopsy (endomysium, perimysium) or in a perivascular area; (B) moderate InI in one or two areas of the interstitium or of the perivascular area; and (C) moderate InI throughout the interstitium or intense inflammation in at least one area of the interstitium or of the perivascular area. Results The three groups were comparable regarding the demographic, clinical and laboratory features (p>0.05). Approximately half of the patients in each group were using CS at the time of the muscle biopsy. The median (interquartile) duration of CS use [4 (0-38), 4 (0–60) and 5 (0–60) days: groups A, B and C, respectively] and the median cumulative CS dose used [70 (0–1200), 300 (0–1470) and 300 (0–1800)mg] were similar between the groups (p>0.05).
    [Show full text]
  • Malignant Granular Cell Tumour with Generalized Metastases And
    310 Letters to the Editor ovoid-shaped Giardia cysts. A one-day oral treatment with REFERENCES ornidazole (Tiberal® , Roche) at dosage of 1,500 mg, then 1. Smith LA. Still around and still dangerous: Giardia lamblia and repeated after 2 weeks, was given. The cutaneous lesions Entamoeba histolytica. Clin Lab Sci 1997; 10: 279–286. gradually improved after the rst dose of the drug. After one 2. Ridley MJ, Ridley DS. Serum antibodies and jejunal histology in month, however, new papules on elbows appeared and a giardiasis associated with malabsorption. J Clin Pathol 1976; 29: 30–34. coproparasitological control revealed the persistence of the 3. Luja`n HD, Mowatt MR, Byrd LG, Nash TE. Cholesterol starva- parasitic infection. A new cycle of ornidazole (1,500 mg/day tion induces diVerentiation of the intestinal parasite Giardia for 3 days) was prescribed. One month later both cutaneous lamblia. Proc Natl Acad Sci USA 1996; 93: 7628–7633. lesions and Giardia cysts in stools were still present. An 4. Geller M, Geller M, Flaherty DK, Black P, Madruga M. Serum alternative treatment with oral paromomycin (Humatin® , levels in giardiasis. Clin Allergy 1978; 8: 69–71. Parke-Davis), 500 mg q.i.d for 5 days was prescribed. The 5. Nash TE, Herrington DA, Losonsky GA, Levine MM. parasitological follow-up at 1, 3 and 6 months was negative Experimental infections with Giardia lamblia. J Infect Dis 1987; and cutaneous signs and symptoms completely resolved. 156: 974–984. 6. Di Prisco MC, Hagel I, Lynch NR, Jimenez JC, Rojas R, Gil M, et al.
    [Show full text]
  • Management of Systemic Lupus Erythemathous with Polymyositis Overlap Syndrome
    ILLUSTRASION CASE MEDICINA 2019, Volume 50, Number 3: 543-549 P-ISSN.2540-8313, E-ISSN.2540-8321 Management of systemic lupus erythemathous with Illustrasion case polymyositis overlap syndrome Doi: http://dx.doi.org/10.15562/medicina.v50i3.575 Suryo Gading,* Ketut Dewi Kumara Wati, Komang Ayu Witarini, Hendra Santoso, I Gusti Ngurah Suwarba CrossMark Volume No.: 50 ABSTRACT There has been an increase in SLE cases among children in Sanglah of Rheumatology. Neurologic examination and electromyography General Hospital. In the rare case, there is a possibility SLE occurs were significant for the decrease in motoric power on the right lower Issue: 3 not as a single entity but overlap with another connective tissue limb, gastrocnemius atrophy, steppage gait, and reduction of the disease. Polymyositis is a disease with a primary symptom of muscle sensory sensation of right L4-S1 dermatome. Hence, the diagnose weakness associated with muscle pain and swollen. Polymyositis very of SLE and polymyositis was concluded. This is a case of SLE overlap rarely becomes overlapping syndrome with SLE, occurring in 4-6% syndrome with polymyositis. The patient was treated with prednisone First page No.: 543 of SLE patients. The aim of this study is to describe clinical findings 2 mg/kg/day for 2 weeks, and also given ibuprofen 10 mg/kg/dose for and management of SLE and Polymyositis. This case is a 12-year-old pain relief, continued with azathioprine plan for one year. The patient girl presented with arthralgia and myalgia since one month before showed an excellent result with the disappearance of symptoms and P-ISSN.2540-8313 admission, accompanied by a 1-month episode of relapsing fever, normal laboratory examination.
    [Show full text]
  • Inclusion Body Myositis: a Case with Associated Collagen Vascular Disease Responding to Treatment
    J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.48.3.270 on 1 March 1985. Downloaded from Journal ofNeurology, Neurosurgery, and Psychiatry 1985;48:270-273 Short report Inclusion body myositis: a case with associated collagen vascular disease responding to treatment RJM LANE, JJ FULTHORPE, P HUDGSON UK From the Regional Neurological Centre, Newcastle General Hospital, Newcastle-upon-Tyne, elec- SUMMARY Patients with inclusion body myositis demonstrate characteristic histological and muscle and are generally considered refractory to treatment. tronmicroscopical abnormalities in autoimmune A patient with inclusion body myositis is described with evidence of associated disease, who responded to steroids. muscles. He felt that his legs were quite normal. He denied guest. Protected by copyright. The diagnosis of inclusion body myositis depends symptoms. There was no relevant family or of the characteristic any sensory ultimately on the demonstration drug history. dis- intracytoplasmic and intranuclear filamentous inclu- On examination, he had a prominent bluish/purple sions, and cytoplasmic vacuoles originally described colouration of the knuckles, thickening of the skin on the by Chou in 1968.' However, reviews of reported dorsum of the hands and a slight heliotrope facial rash. The features which facial muscles were slightly wasted and he had marked cases have also emphasised clinical sternomastoids, deltoids, appear to distinguish inclusion body myositis from weakness and wasting of the Prominent among spinatti, biceps and triceps, with relative preservation of other forms of polymyositis.2-7 distal muscles. All upper limb reflexes were grossly these are the lack of associated skin changes or other bulk, power and to diminished or absent.
    [Show full text]
  • Autoimmunity Mixed Connective Tissue Disease (CTD)
    Autoimmunity Mixed Connective Tissue Disease (mixed CTD) and Undifferentiated Connective Tissue Disease (UCTD) Autoimmunity and Connective Tissue Disease (CTD) The immune system normally produces antibodies which attack bugs (viruses, bacteria and fungi). Sometimes, for reasons we don’t fully understand, the immune system goes into ‘overdrive’ and produces antibodies which attack the body’s own tissues, causing inflammation. This is called autoimmunity and may cause an autoimmune disease. A common example of this is underactive thyroid where antibodies are produced which attack the thyroid gland. The connective tissues are the structural portions of our body that essentially hold the cells of the body together. These tissues form a framework or matrix for the body. Connective Tissue Disease (CTD) Connective tissue disease is an autoimmune disease where the body produces antibodies against its own connective tissue, causing inflammation. The ‘classic’ connective tissue diseases include: Lupus Rheumatoid arthritis Scleroderma (or systemic sclerosis) Polymyositis and Source: Rheumatology Reference No: 6252-1 Issue date: 26/9/19 Review date: 26/9/22 Page 1 of 4 Dermatomyositis Each of these diseases has a typical presentation with clinical findings that doctors can recognise during an examination. Each also has certain blood test abnormalities and abnormal antibody patterns. However, each of these diseases can start with very mild symptoms before developing the classic features that help in the diagnosis. Undifferentiated Connective Tissue Disease (UCTD) Almost one in four people seen in rheumatology clinics develop an autoimmune disease which doesn't fit neatly into a category, so they are not given a definite disease label. When these conditions have not developed the classic features of a particular disease, doctors will often refer to the condition as "undifferentiated connective tissue disease" or UCTD for short.
    [Show full text]
  • Scleroderma, Myositis and Related Syndromes [4] Giordano J, Khung S, Duhamel A, Hossein-Foucher C, Bellèvre D, Lam- Blin N, Et Al
    Scientific Abstracts 1229 Ann Rheum Dis: first published as 10.1136/annrheumdis-2021-eular.75 on 19 May 2021. Downloaded from Scleroderma, myositis and related syndromes [4] Giordano J, Khung S, Duhamel A, Hossein-Foucher C, Bellèvre D, Lam- blin N, et al. Lung perfusion characteristics in pulmonary arterial hyper- tension and peripheral forms of chronic thromboembolic pulmonary AB0401 CAN DUAL-ENERGY CT LUNG PERFUSION hypertension: Dual-energy CT experience in 31 patients. Eur Radiol. 2017 DETECT ABNORMALITIES AT THE LEVEL OF LUNG Apr;27(4):1631–9. CIRCULATION IN SYSTEMIC SCLEROSIS (SSC)? Disclosure of Interests: None declared PRELIMINARY EXPERIENCE IN 101 PATIENTS DOI: 10.1136/annrheumdis-2021-eular.69 V. Koether1,2, A. Dupont3, J. Labreuche4, P. Felloni3, T. Perez3, P. Degroote5, E. Hachulla1,2,6, J. Remy3, M. Remy-Jardin3, D. Launay1,2,6. 1Lille, CHU Lille, AB0402 SELF-ASSESSMENT OF SCLERODERMA SKIN Service de Médecine Interne et Immunologie Clinique, Centre de référence THICKNESS: DEVELOPMENT AND VALIDATION OF des maladies autoimmunes systémiques rares du Nord et Nord-Ouest de THE PASTUL QUESTIONNAIRE 2 France (CeRAINO), Lille, France; Lille, Université de Lille, U1286 - INFINITE 1,2 1 1 1 J. Spierings , V. Ong , C. Denton . Royal Free and University College - Institute for Translational Research in Inflammation, Lille, France; 3Lille, Medical School, University College London, Division of Medicine, Department From the Department of Thoracic Imaging, Hôpital Calmette, Lille, France; 4 of Inflammation, Centre for Rheumatology and Connective
    [Show full text]
  • Diagnosis and Treatment of Dermatomyositis-Systemic Lupus
    Diagnosis and Treatment of Dermatomyositis-Systemic Lupus Erythematosus Overlap Syndrome Preston Williams1; Benjamin McKinney, MD2 1Texas A&M College of Medicine; 2Baylor University Medical Center Family Medicine Residency Introduction Case Description Discussion Dermatomyositis is an autoimmune condition classically A punch biopsy of her rash showed atrophic epithelium with This case of overlap syndrome between dermatomyositis and characterized by symmetric proximal muscle weakness, dyskeratotic keratinocytes, vacuolar interface changes, superficial systemic lupus erythematosus presents a rare but important inflammatory muscle changes, and dermatologic abnormalities.1 perivascular and lichenoid inflammation, and pigment challenge to the primary care physician. Our patient presented Several studies have shown that the inflammatory myopathies, incontinence consistent with systemic lupus erythematosus initially with arthralgias and fatigue, symptoms more such as dermatomyositis, commonly overlap with other (SLE). The patient was started on prednisone 40 mg daily for a 2- characteristic of systemic lupus erythematosus. However, these connective tissue disorders, significantly complicating the week taper to 10 mg and hydroxychloroquine 200 mg daily with symptoms were followed by a facial rash that involved the diagnosis.2 The reported incidence of overlap syndrome ranges marked improvement in symptoms. Further lab work-up was nasolabial folds and periorbital regions more in line with from 11% to 40% in patients diagnosed with significant
    [Show full text]
  • Myositis 101
    MYOSITIS 101 Your guide to understanding myositis Patients who are informed, who seek out other patients, and who develop helpful ways of communicating with their doctors have better outcomes. Because the disease is so rare, TMA seeks to provide as much information as possible to myositis patients so they can understand the challenges of their disease as well as the options for treating it. The opinions expressed in this publication are not necessarily those of The Myositis Association. We do not endorse any product or treatment we report. We ask that you always check any treatment with your physician. Copyright 2012 by TMA, Inc. TABLE OF CONTENTS contents Myositis basics ...........................................................1 Diagnosis ....................................................................5 Blood tests .............................................................. 11 Common questions ................................................. 15 Treatment ................................................................ 19 Disease management.............................................. 25 Be an informed patient ............................................ 29 Glossary of terms .................................................... 33 1 MYOSITIS BASICS “Myositis” means general inflammation or swelling of the muscle. There are many causes: infection, muscle injury from medications, inherited diseases, disorders of electrolyte levels, and thyroid disease. Exercise can cause temporary muscle inflammation that improves after rest. myositis
    [Show full text]
  • Studies with Human Leukocyte Lysosomes. Evidence for Antilysosome Antibodies in Lupus Erythematosus and for the Presence of Lysosomal Antigen in Inflammatory Diseases
    Studies with human leukocyte lysosomes. Evidence for antilysosome antibodies in lupus erythematosus and for the presence of lysosomal antigen in inflammatory diseases. D A Bell, … , J H Vaughan, J P Leddy J Clin Invest. 1975;55(2):256-268. https://doi.org/10.1172/JCI107929. Research Article Human lysosomes were isolated from normal peripheral blood leukoyctes and characterized by electron microscopy, enzyme analysis, and assays for DNA and RNA. Stored sera from 37 unselected patients with systemic lupus erythematosus (SLE), including active and inactive, treated and untreated cases, were tested in complement fixation (CF) reactions with these lysosome preparations. 23 SLE sera exhibited positive CR reactions, as did sera from two patients with "lupoid" hepatitis. The seven SLE sera with strongest CF reactivity also demonstrated gel precipitin reactions with lysosomes. Neither CF nor precipitin reactions with lysosomes were observed with normal sera or with sera of patients with drug-induced lupus syndrome, rheumatoid arthritis (RA), polymyositis, or autoimmune hemolytic anemia. By several criteria the antilysosome CF and precipitin reactions of SLE sera cound not be attributed to antibody to DNA, RNA, or other intracellular organelles. The lysosomal component reactive with SLE sera in CF assays was sedimentable at high speed and is presumably membrane associated. The CF activity of two representative SLE sera was associated with IgG globulins by Sephadex filtration. A search for lysosomal antigen in SLE and related disorders was also made. By employing rabbit antiserum to human lysosomes in immunodiffusion, a soluble lysosomal component, apparently distinct from the sedimentable (membrane-associated) antigen described above, was identified in serum, synovial fluid, or pleural fluid from patients with […] Find the latest version: https://jci.me/107929/pdf Studies with Human Leukocyte Lysosomes EVIDENCE FOR ANTILYSOSOME ANTIBODIES IN LUPUS ERYTHEMATOSUS AND FOR THE PRESENCE OF LYSOSOMAL ANTIGEN IN INFLAMMATORY DISEASES DAVID A.
    [Show full text]
  • Polymyositis, Not Polymyalgia Rheumatica
    Annals ofthe Rhewnatic Diseases 1991; 50: 321-322 321 CASE REPORTS Ann Rheum Dis: first published as 10.1136/ard.50.5.321 on 1 May 1991. Downloaded from Polymyositis, not polymyalgia rheumatica N D Hopkinson, D J Shawe, J M Gumpel Abstract prednisolone 30 mg/day; two weeks later there The distinction between polymyalgia rheu- was improvement and the ESR had fallen to 14 matica and polymyositis is important for mm/h. Six months later she again had proximal treatment and prognosis. Four elderly patients muscle pain but also weakness and she reported were diagnosed and treated for polymyalgia: numerous chest infections, all responding raised creatine kinase led to muscle biopsy poorly to antibiotics. Hospital referral and and a diagnosis of polymyositis. subsequent admission confirmed a broncho- pneumonia and marked proximal muscle wast- ing and weakness. Investigations showed a Polymyositis and polymyalgia rheumatica may haemoglobin concentration of 119 g/l and the both present with a similar distribution of white blood cell count was raised at 14 9x 109/l proximal muscle pain but are not normally with a neutrophil leucocytosis. The ESR was confused. In polymyositis tenderness or weak- raised at 100 mm/h and creatine kinase was ness of muscles may be marked and there may raised at 600 IU/1. Needle biopsy confirmed a be other helpful diagnostic features-for moderate myositis. example, dysphagia, whereas muscle pain or stiffness without weakness are the more prominent symptoms in polymyalgia rheu- CASE NO 3 matica. In older patients the differentiation may A 77 year old woman presented to her general not be easy, especially when muscle weakness is practitioner with pain and weakness of the not marked.
    [Show full text]
  • Drug-Induced Lupus Erythematosus Incidence, Management and Prevention
    Drug Saf 2011; 34 (5): 357-374 REVIEW ARTICLE 0114-5916/11/0005-0357/$49.95/0 ª 2011 Adis Data Information BV. All rights reserved. Drug-Induced Lupus Erythematosus Incidence, Management and Prevention Christopher Chang1 and M. Eric Gershwin2 1 Division of Allergy, Asthma and Immunology, Nemours/A.I. Dupont Children’s Hospital, Thomas Jefferson University, Wilmington, Delaware, USA 2 Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis, Davis, California, USA Contents Abstract. 357 1. The History and Epidemiology of Drug-Induced Lupus . 358 2. Drug-Induced Subacute Cutaneous Lupus Erythematosus and Chronic Cutaneous Lupus Erythematosus. 360 3. Clinical Presentation and Laboratory Abnormalities. 361 3.1 Traditional Drug-Induced Lupus. 361 3.1.1 High-Risk Drugs . 361 3.1.2 Moderate-Risk Drugs . 361 3.1.3 Low-Risk Drugs . 362 3.2 Biological Modulators and Drug-Induced Lupus. 363 3.2.1 Tumor Necrosis Factor Inhibitors . 363 3.2.2 Cytokines . 365 4. Diagnosis . 366 5. Pathophysiology. 368 6. Prevention and Treatment . 370 7. Discussion . 371 Abstract The generation of autoantibodies and autoimmune diseases such as sys- temic lupus erythematosus has been associated with the use of certain drugs in humans. Early reports suggested that procainamide and hydralazine were associated with the highest risk of developing lupus, quinidine with a mod- erate risk and all other drugs were considered low or very low risk. More recently, drug-induced lupus has been associated with the use of the newer biological modulators such as tumour necrosis factor (TNF)-a inhibitors and interferons. The clinical features and laboratory findings of TNFa inhibitor- induced lupus are different from that of traditional drug-induced lupus or idiopathic lupus, and standardized criteria for the diagnosis of drug-induced lupus have not been established.
    [Show full text]
  • Liver Damage in Patients with Polymyositis and Dermatomyositis
    Original Contribution Kitasato Med J 2016; 46: 40-46 Liver damage in patients with polymyositis and dermatomyositis Tatsuhiko Wada, Gakurou Abe, Takeo Kudou, Eisuke Ogawa, Tatsuo Nagai, Sumiaki Tanaka, Shunsei Hirohata Department of Rheumatology and Infectious Diseases, Kitasato University School of Medicine Objective: To clarify the prevalence of liver damage associated with polymyositis (PM) and dermatomyositis (DM) Methods: Forty-two patients with PM/DM were exhaustively studied. Six patients showed liver diseases of known etiology, including drug-induced hepatitis, fatty liver, metastatic liver tumor, primary biliary cirrhosis and autoimmune hepatitis. As a control, 8 patients with miscellaneous diseases who showed elevation of the creatine kinase (CK) due to extreme exercise were studied. Serum levels of aspirate aminotransferase (AST), alanine aminotransferase (ALT) and CK, as well as the ratios of AST/CK, ALT/CK and AST/ALT were evaluated. Results: ALT levels were significantly elevated in PM/DM compared with control group. The ratios of AST/CK and ALT/CK were significantly elevated in PM/DM compared with the control group. AST/ALT ratios in PM/DM were significantly lower than those in control group (P < 0.0001). Twenty- six of 36 patients (72.2%) with PM/DM showed AST/ALT ratios below the minimum value of AST/ ALT ratios in the control patients. Additionally, 10 of 26 patients (38.5%) showed hepatocellular liver damage. There were no significant differences in the levels of AST, ALT or CK, or in the ratios of AST/CK, ALT/CK or AST/ALT between PM and DM. Conclusions: The results indicate that a disproportionate elevation of ALT takes places in patients with PM/DM.
    [Show full text]