An Overlap of Systemic Lupus Erythematosus, Dermato
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A Rare Case Report of Polyangiitis Overlap Syndrome: Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis Michele V
Quan et al. BMC Pulmonary Medicine (2018) 18:181 https://doi.org/10.1186/s12890-018-0733-2 CASE REPORT Open Access A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis Michele V. Quan1* , Stephen K. Frankel2, Mehrnaz Maleki-Fischbach3 and Laren D. Tan1* Abstract Background: Granulomatosis with polyangiitis (GPA) is a systemic ANCA-associated vasculitis characterized by necrotizing granulomatous inflammation and a predilection for the upper and lower respiratory tract. Eosinophilic granulomatosis with polyangiitis (EGPA) is also a systemic ANCA-associated vasculitis, but EGPA is characterized by eosinophilic as well as granulomatous inflammation and is more commonly associated with asthma and eosinophilia. Polyangiitis overlap syndrome is defined as systemic vasculitis that does not fit precisely into a single category of classical vasculitis classification and/or overlaps with more than one category. Several polyangiitis overlap syndromes have been identified, however, there are very few case reports of an overlap syndrome involving both GPA and EGPA in the medical literature. Case presentation: We conducted a PUBMED literature review using key words ‘granulomatosis with polyangiitis,’ ‘Wegener’s,’‘GPA,’‘eosinophilic granulomatosis with polyangiitis,’‘Churg-Strauss,’‘EGPA,’‘overlap syndrome,’‘Wegener’s with eosinophilia,’ and ‘GPA with eosinophilia’ in English only journals from 1986 to 2017. Relevant case reports and review articles of overlap syndromes of GPA and EGPA were identified. We aim to report a unique case of GPA and EGPA overlap syndrome and review the cases that have been previously described. Between 1986 and 2017, we identified 15 cases that represent an overlap syndrome with compelling features of both GPA and EGPA. -
Gether with Anti -Synthetase, Ro52 and Jo-1-Double Positive: High Rate of Malignancies, Poorer E.G
Journal of Neuromuscular Diseases 5 (2018) 109–129 109 DOI 10.3233/JND-180308 IOS Press Review Current Classification and Management of Inflammatory Myopathies Jens Schmidt∗ Department of Neurology, Muscle Immunobiology Group, Neuromuscular Center, University Medical Center G¨ottingen, G¨ottingen, Germany Abstract. Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyogra- phy (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis. Keywords: Skeletal muscle, muscle inflammation, myositis, immunosuppression, neuroinflammation, autoimmunity INTRODUCTION requires testing of auto-antibodies, histological eval- uation of a skeletal muscle biopsy and further tests Inflammatory myopathies (synonym: idiopathic including muscle MRI and EMG. Novel diagnostic inflammatory myopathy, IIM) –in short: myositis– criteria have recently been established, but an update are rare conditions that can affect multiple organs will be required (see below for details). -
Percutaneous Conchotome Muscle Biopsy. a Useful Diagnostic and Assessment Tool CHRISTINA DORPH, INGER NENNESMO, and INGRID E
Percutaneous Conchotome Muscle Biopsy. A Useful Diagnostic and Assessment Tool CHRISTINA DORPH, INGER NENNESMO, and INGRID E. LUNDBERG ABSTRACT. Objective. To evaluate the diagnostic yield, performance simplicity, and safety of the percutaneous conchotome muscle biopsy technique for clinical and research purposes in an outpatient rheuma- tology clinic. Methods. Biopsies taken by rheumatologists in an outpatient clinic during 1996 and 1997 were eval- uated for histopathological and clinical diagnoses. Results. A total of 149 biopsies were performed on 122 patients. Physicians learned the method easily. Samples were of adequate size and quality to allow for diagnostics. In total 106 biopsies were taken due to different diagnostic suspicions: 24 polymyositis (PM) or dermatomyositis (DM); 43 PM, DM, or vasculitis in addition to another rheumatic condition; 19 systemic vasculitis; and 20 myalgias. Criteria for definite or probable PM/DM were fulfilled in 21 patients, 18 with positive biopsies. Thirteen patients received vasculitis as clinical diagnosis, 3 with positive biopsies. No patient with myalgia had a biopsy with inflammatory changes. Fifteen of 43 rebiopsies performed to assess disease activity had signs of active inflammation. In 48% there were changes in immuno- suppressive therapy after biopsy results. Four complications occurred; one was a serious subfascial hematoma. Conclusion. The percutaneous conchotome muscle biopsy technique gives a good size sample that allows for diagnostic evaluation and has a high yield in patients with myositis. It is a simple proce- dure, easy to learn and to perform, with a low complication rate and minimum discomfort for the patient. The method can preferably be used as a diagnostic tool and to perform repeated biopsies to assess the effect of a given therapy for both clinical and research purposes. -
A Case of Undifferentiated Connective Tissue Disease
Journal of College of Medical Sciences-Nepal, 2013, Vol-9, No-4, 59-62 Case Report A case of Undifferentiated connective tissue disease Chatterjee A,1 Chatterjee K,2 Sarkar N,3 1Assistant Professor, 2Senior Resident, Department of Pediatrics, Calcutta National Medical College 3 Registrar, Apollo Gleaneagles Hospital,Kolkata. ABSTRACT Undifferentiated connective tissue disease is an overlap syndrome in which the features of more than one disease is present but their complete diagnosis is lacking. We are presenting an 11year child with fever, arthritis, polyserositis, myalgia, nephritis, sclerodactyly with positive anti-dsDNA and anti-Smith antibody. She improved with prednisolone and cyclophosphamide. Key Words: Undifferentiated connective tissue disease, polyserositis, arthritis, myalgia, sclerodactyly. INTRODUCTION and investigation showed ESR-95mm/hour. After Connective tissue disease result from autoimmune 4 months of fever ,she developed pain, swelling processes that lead to inflammation of target organs. and movement restriction of the large joints, it was Rarely,children develop overlap syndromes, one symmetric with no small joint involvement. She is such is mixed connective tissue disease(MCTD) the third child of a non-consanguinous marriage, where features of two or more major rheumatic with no significant past illness and no family history disorders are seen-juvenile rheumatoid of musculoskeletal disease. arthritis(JRA), systemic lupus erythematosus She was admitted to a hospital at 8 months of fever (SLE), juvenile dermatomyositis(JDM) and with joint pain and swelling, weakness and pallor, systemic sclerosis. Children may also have cervical lymphadenopathy and abdominal undifferentiated connective tissue disease in which distention. Investigation showed Hb-8.3gm/ manifestations strongly suggest but do not meet dL,ESR-60mm/hour, malaria parasite, sputum for diagnostic criteria for a specific rheumatic disease. -
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). -
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. -
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. -
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. -
Sjögren's Syndrome and Sicca Symptoms in Patients with Systemic
al of Arth rn ri u ti o s J Horimoto et al., J Arthritis 2016, 5:1 Journal of Arthritis DOI: 10.4172/2167-7921.1000190 ISSN: 2167-7921 Research Article Open Access Sjögren’s Syndrome and Sicca Symptoms in Patients with Systemic Sclerosis Alex Magno Coelho Horimoto1*, Vinicius de Macedo Possamai2 and Izaias Pereira da Costa1 1Professor of Rheumatology, Medical University of Mato Grosso do Sul, Brazil 2Rheumatologist, Medical University of Mato Grosso do Sul, Brazil Abstract Introduction: Several autoimmune diseases can be accompanied by dysfunction of the salivary glands, regardless of the presence or absence of association with Sjögren’s syndrome (SS). A recent study by Maeshima et al. found salivary hyposecretion in 58.3% of patients with various connective tissue diseases, particularly systemic sclerosis (SSc). Objective: To determine the prevalence of SS and sicca symptoms in patients with SSc. Assess whether the presence of SS in patients with SSc causes worsening of the disease. Methods: 69 SSc patients periodically monitored in the rheumatology clinic at NHU / UFMS composed the study. All patients were questioned about sicca symptoms and clinical features. We evaluated the RF levels, ANA, anti-Ro / La. Results and discussion: 69 SSc patients were enrolled in the study, with average age of 51.2 years, women at 98.3% and white by 50%. Sicca symptoms were present in 48 patients (69.5%) with SSc; 43/69 patients (62.3%) with dry mouth and 46/69 patients (66,7%) with dry eye. Sicca symptoms observed predominantly in patients with diffuse disease (75%). The antinuclear antibody positivity was 95% and the rheumatoid factor (RF) was observed in 14 patients (23.3%). -
Antimitochondrial Antibodies and Primary Biliary Cholangitis in Patients with Polymyalgia Rheumatica/Giant Cell Arteritis
medicina Review Antimitochondrial Antibodies and Primary Biliary Cholangitis in Patients with Polymyalgia Rheumatica/Giant Cell Arteritis Ciro Manzo 1,* , Maria Maslinska 2, Alberto Castagna 3, Elvis Hysa 4, Alfonso Merante 5, Marcin Milchert 6, Tiziana Gravina 7, Betul Sargin 8, Maria Natale 9, Carmen Ruberto 10 and Giovanni Ruotolo 11 1 Azienda Sanitaria Locale Napoli 3 Sud, Internal and Geriatric Medicine Department, Rheumatologic Outpatient Clinic, Health District No. 59, Sant’Agnello, 80065 Naples, Italy 2 National Institute of Geriatrics, Rheumatology and Rehabilitation, 02-637 Warsaw, Poland; [email protected] 3 Azienda Sanitaria Provinciale Catanzaro, Primary Care Department, Casa Della Salute di Chiaravalle Centrale, Fragility Outpatient Clinic, 88100 Catanzaro, Italy; [email protected] 4 Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, San Martino Policlinic Hospital, 16121 Genoa, Italy; [email protected] 5 Geriatric Unit, Azienda Ospedaliera “Pugliese-Ciaccio”, 88100 Catanzaro, Italy; [email protected] 6 Department of Rheumatology, Internal Medicine, Geriatrics and Clinical Immunology of Pomeranian Medical University, 71-871 Szczecin, Poland; [email protected] 7 Hepatology Unit, Azienda Ospedaliera Universitaria “Mater Domini”, 88100 Catanzaro, Italy; [email protected] 8 Department of Physical Medicine and Rehabilitation, Division of Rheumatology, Medical Faculty of Adnan Menderes University, Aydin 09100, Turkey; [email protected] 9 Azienda -
Hepatic Manifestations of Autoimmune Rheumatic Diseases ANNALS of GASTROENTEROLOGY 2005, 18(3):309-324309
Hepatic manifestations of autoimmune rheumatic diseases ANNALS OF GASTROENTEROLOGY 2005, 18(3):309-324309 Review Hepatic manifestations of autoimmune rheumatic diseases Aspasia Soultati, S. Dourakis SUMMARY the association between primary autoimmune rheumatolog- ic disease and associated hepatic abnormalities and the Autoimmune rheumatic diseases including Systemic Lu- pharmaceutical interventions that are related to liver dam- pus Erythematosus, Rheumatoid Arthritis, Sjogrens syn- age are presented. drome, Myositis, Antiphospholipid Syndrome, Behcets syndrome, Scleroderma and Vasculitides have been associ- Key words: Connective Tissue Disease, Systemic Lupus Ery- ated with hepatic injury by virtue of multisystem immune thematosus, Rheumatoid Arthritis, Sjogrens syndrome, and inflammatory involvement. Liver involvement preva- Myositis, Giant-Cell Arteritis, Antiphospholipid Syndrome, lence, significance and specific hepatic pathology vary. Af- Behcets syndrome, Scleroderma, Vasculitis, Steatosis, Nod- ter careful exclusion of potentially hepatotoxic drugs or co- ular Regenerative Hyperplasia, portal hypertension, Autoim- incident viral hepatitis the question remains whether liver mune Hepatitis, Primary Biliary Cirrhosis, Primary Scleros- involvement emerges as a manifestation of generalized con- ing Cholangitis nective tissue disease or it reflects an underlying primary liver disease sharing an immunological mechanism. Com- 1. Introduction monly recognised features include mild elevation of liver A variety of autoimmune rheumatic diseases -
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.