RESUMO
OBJECTIVE: Studies evaluating pregnancy outcomes in systemic sclerosis (SSc) are limited. SSc is associated with maternal complications and adverse neonatal outcomes. This study investigated the impact of disease stage (stable vs active) on the maternal and neonatal outcomes of pregnancies of patients followed at an Israeli medical centre. METHOD: The charts of 354 SSc female patients followed during 2003-2020 were reviewed. Data on clinical and laboratory features, number of pregnancies close to SSc diagnosis, and maternal and neonatal outcomes were analysed. Patients were divided into a stable disease and an active/early disease group. RESULTS: The active/early disease group included 26 patients [19 diffuse SSc (dSSc)], with 38 pregnancies. Median disease duration was 1 year, except for four patients who were first diagnosed during pregnancy. SSc was exacerbated in all patients during pregnancy or shortly after delivery [skin, lung, and heart involvement in dSSc; severe vasculopathy in limited SSc patients]. Six patients had hypertensive disorders of pregnancy; four pregnancies ended with foetal death. Thirty-three children were born, 60% with intrauterine growth retardation (IUGR)/low birthweight (LBW). The stable disease group included 19 patients, including seven with previously active disease, now stabilized (five dSSc), and 32 pregnancies. All pregnancies were planned and monitored closely. Disease remained stable in all patients. Four patients had hypertensive disorders of pregnancy; 12/29 newborns had LBW (41%). CONCLUSION: Active maternal disease during pregnancy poses an increased risk of SSc aggravation. The maternal and neonatal outcomes in planned pregnancy during stable disease are favourable. IUGR/LBW is common among neonates, even in stable disease.
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Hipertensão Induzida pela Gravidez , Escleroderma Sistêmico , Gravidez , Criança , Humanos , Recém-Nascido , Feminino , Serviços de Planejamento Familiar , Resultado da Gravidez/epidemiologia , Retardo do Crescimento Fetal/epidemiologia , Retardo do Crescimento Fetal/etiologia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/epidemiologiaRESUMO
AIMS: Pneumatosis cystoides intestinalis (PCI) is a rare life-threatening gastrointestinal complication in the course of connective tissue disease (CTD). PCI is characterised by the appearance of intramural clusters of gas in the small and large bowel wall on X-ray or computed tomography and often is accompanied by free air in the peritoneal cavity. METHODS: We present three cases of PCI in patients with scleroderma-related conditions. A review of the English language literature published on MEDLINE from 1973 to 2008 was conducted using the terms: 'systemic sclerosis', 'connective tissue disease' and 'pneumatosis cystoides intestinalis'. This review focused on clinical features, diagnostic and treatment strategies of PCI in the context of CTD. RESULTS: Symptoms of PCI are non-specific: abdominal pain, vomiting, constipation, bloating and weight loss. Coexistence of PCI with other manifestations of CTD, such as intestinal pseudo-obstruction and/or bacterial overgrowth, complicates the clinical diagnosis. Treatment approach to PCI is mostly conservative: intestinal 'rest', parenteral nutrition, antibiotics, fluids and electrolyte supplementation, and inhaled oxygen. Surgical intervention should be performed only in cases of bowel perforation, ischaemia or necrosis. Patients with PCI have high mortality rates due to PCI itself but also to the severity and variety of basic CTD complications. CONCLUSION: Recognition of PCI, particularly in the context of underlying CTD, is necessary for proper therapeutic application. In patients with underlying CTD and symptoms of abdominal emergency, recruitment of multidisciplinary teams, including rheumatologist, gastroenterologist, imaging specialist and surgeons familiar with intestinal complications of CTD-related conditions, is warranted.
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Pneumatose Cistoide Intestinal/etiologia , Escleroderma Sistêmico/complicações , Abdome Agudo/etiologia , Corticosteroides/efeitos adversos , Corticosteroides/uso terapêutico , Adulto , Idoso , Doenças do Tecido Conjuntivo/complicações , Diagnóstico Diferencial , Diatrizoato de Meglumina/uso terapêutico , Evolução Fatal , Feminino , Humanos , Hipoalbuminemia/etiologia , Imunoglobulinas Intravenosas/uso terapêutico , Obstrução Intestinal/diagnóstico , Pseudo-Obstrução Intestinal/etiologia , Pessoa de Meia-Idade , Octreotida/uso terapêutico , Omeprazol/uso terapêutico , Pneumatose Cistoide Intestinal/diagnóstico , Pneumoperitônio/etiologia , Polimiosite/complicações , Escleroderma Sistêmico/terapia , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Redução de PesoRESUMO
OBJECTIVE: To assess the effect of rituximab on the efficacy and safety of influenza virus vaccine in patients with rheumatoid arthritis (RA). METHODS: The study group comprised patients with RA treated with conventional disease-modifying drugs with or without rituximab. Split-virion inactivated vaccine containing 15 microg haemagglutinin/dose of B/Shanghai/361/02 (SHAN), A/New Caledonian/20/99 (NC) (H1N1) and A/California/7/04 (CAL) (H3N2) was used. Disease activity was assessed by the number of tender and swollen joints, duration of morning stiffness and evaluation of pain on the day of vaccination and 4 weeks later. CD19-positive cell levels were assessed in rituximab-treated patients. Haemagglutination inhibition (HI) antibodies were tested and response was defined as a greater than fourfold rise 4 weeks after vaccination or seroconversion in patients with a non-protective baseline level of antibodies (<1/40). Geometric mean titres (GMT) were calculated in all subjects. RESULTS: The participants were divided into three groups: RA (n = 29, aged 64 (12) years), rituximab-treated RA (n = 14, aged 53 (15) years) and healthy controls (n = 21, aged 58 (15) years). All baseline protective levels of HI antibodies and GMT were similar. Four weeks after vaccination, there was a significant increase in GMT for NC and CAL antigens in all subjects, but not for the SHAN antigen in the rituximab group. In rituximab-treated patients, the percentage of responders was low for all three antigens tested, achieving statistical significance for the CAL antigen. Measures of disease activity remained unchanged. CONCLUSION: Influenza virus vaccine generated a humoral response in all study patients with RA and controls. Although the response was significantly lower among rituximab-treated patients, treatment with rituximab does not preclude administration of vaccination against influenza.
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Anticorpos Monoclonais/farmacologia , Antirreumáticos/farmacologia , Artrite Reumatoide/imunologia , Vacinas contra Influenza/imunologia , Influenza Humana/prevenção & controle , Adulto , Idoso , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais Murinos , Anticorpos Antivirais/biossíntese , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Feminino , Testes de Inibição da Hemaglutinação/métodos , Humanos , Vírus da Influenza A/imunologia , Vírus da Influenza B/imunologia , Masculino , Pessoa de Meia-Idade , Rituximab , Índice de Gravidade de Doença , VacinaçãoRESUMO
Polyarteritis nodosa (PAN) of the calf muscles is a rare form of vasculitis. We present two cases of PAN limited to the calf and a review of the literature, based on a MEDLINE (PubMed) search of the English literature from 1980 to 2005, using the key words "vasculitis restricted to limbs", "polyarteritis nodosa", and "intravenous immunoglobulin". PAN limited to the calf muscles is a condition presenting with severe shin pain and walking difficulties. In contrast to classic PAN, there is no skin, joint, visceral or nerve system involvement in this form of the disease. The main clinical signs are tenderness and swelling of the calf. Inflammatory markers, such as erythrocyte sedimentation rate and C-reactive protein, are usually elevated, and a perinuclear pattern of anti-neutrophil cytoplasm antibodies can be found. Electromyography of the calf is not contributory. Magnetic resonance imaging may be useful in recognizing the limb-restricted vasculopathy and selecting the muscle biopsy site, which is obligatory for diagnosis. Corticosteroids (CS) are the main treatment regimen, but CS-resistant cases have been reported. The patients presented here failed to respond to CS but were successfully treated with intravenous immunoglobulin therapy (IVIG). In the absence of vital organ involvement, the addition of cytotoxic drugs is controversial. IVIG seems to be an efficient alternative therapy in PAN limited to the calf muscles especially for patients with limitations to conventional cytotoxic treatment.
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Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Perna (Membro)/irrigação sanguínea , Músculo Esquelético/patologia , Poliarterite Nodosa/terapia , Adulto , Anticorpos Anticitoplasma de Neutrófilos/sangue , Quimioterapia Combinada , Feminino , Glucocorticoides/uso terapêutico , Humanos , Masculino , Músculo Esquelético/irrigação sanguínea , Poliarterite Nodosa/sangue , Poliarterite Nodosa/patologia , Indução de Remissão , Resultado do TratamentoRESUMO
Ultrasonography (US) was shown as an effective imaging modality in evaluating the shoulder. The shoulder joint is probably the most accessible joint for sonography in adults. However, inflammatory changes of the shoulder have received too little attention in US studies. Anterior access for US assessment of glenohumeral joint (GHJ) has not been investigated. Another problem of patients with acute synovitis of glenohumeral joint is the difficulty to perform a 90 degrees abduction for the axillary US because of severe pain and active and passive limitation. We offer the anterior access for assessment of glenohumeral joint synovitis (GHS). Sonographic evaluation (Sonosite-Titan) was carried out in 25 patients with acute GHS and 15 healthy controls. The diagnosis of GHS was made after the patients underwent physical examination and the laboratory evidence was obtained. We used the anterior position of transducer applied laterally to coracoid processus along the anterior joint cavity. The problem of anterior joint cavity investigation in neutral position is a poor presentation of the joint and the application of the biceps tendon. The problem is simply resolved after supination of the hand and external rotation of the shoulder. We measured and compared upper, middle, and lower width of the anterior GHJ cavity. Echogenicity of joint cavity was assessed by comparison with adjacent tissues. Homogeneity and regularity of GHJ cavity was designated in both groups as well. We measured labrum-infraspinatus distance on posterior view for assessment of GHJ synovitis. All cases of GHJ synovitis were confirmed by a US Doppler study. US investigation of healthy controls enabled to find normal values of the width of the anterior GHJ cavity that was less than 7.4 mm. The synovitis group showed GHJ cavity expansion: 8.3+/-2.4 (p=0.001) and 10.5+/-3.1 (p<0.001) for the middle and the lower anterior part of the GHJ respectively. The upper part width was not different in synovitis and control groups. Anterior joint cavity extension to 7.4 mm and upper in its lower part was high sensitive (96%) and specific (86%) US sign of synovitis with the test power above 0.9. The posterior labrum-infraspinatus extension had high specificity for synovitis (100%), but only seven of 25 patients (28%) had increased (>2 mm) the value of the labrum-infraspinatus dimension, which was previously proposed as the US sign of synovitis. Echogenicity of the anterior joint cavity in healthy controls was moderately high (far more echogenic than deltoid muscle). Echogenicity of synovitis declined, and mild effusions were found to be common. Those were not to be seen on US of GHJ in neutral position and were revealed only in supination and external rotation of the shoulder. Intra-articular tissue of healthy controls was relatively echo-homogenic compared with nonhomogenic one of the synovitis group. Bone irregularity was found in patients with long-standing GHJ synovitis reflecting erosive process. A certain position of the shoulder and good knowledge of the normal anterior joint cavity parameters enabled us to diagnose synovitis by anterior shoulder sonography, with the patients experiencing minimal pain during movements.
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Articulação do Ombro/diagnóstico por imagem , Sinovite/diagnóstico por imagem , Estudos de Casos e Controles , Feminino , Humanos , Masculino , Ultrassonografia/métodosRESUMO
We report a rare case of essential mixed cryoglobulinemia type II with membrano-proliferative glomerulonephritis (MPGN) type I in which HCV was not found. Long-term history of palindromic rheumatism, skin leukocytoclastic vasculitis attacks and micro-normocytic anemia preceded the appearance of cryoglobulinemia. Cryoprecipitate consisted of monoclonal IgMk-RF and polyclonal IgG (essential mixed type II). The newly appreciated cryoglobulinemia was associated with Coombs positive hemolytic anemia. The MPGN in this case had a benign course and responded to complex simple therapies including prevention of exposure to cold, low antigen content diet, treatment of provoking factors such as UTI, and maximal dose of ACE inhibitor. Responsiveness of skin vasculitis to colchicine therapy was restored after a two-month colchicine withdrawal period and therefore corticosteroid and immunosuppressive therapy was postponed.
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Anemia Hemolítica/patologia , Crioglobulinemia/patologia , Glomerulonefrite Membranoproliferativa/patologia , Vasculite Leucocitoclástica Cutânea/patologia , Idoso , Anemia Hemolítica/complicações , Anemia Hemolítica/terapia , Inibidores da Enzima Conversora de Angiotensina/uso terapêutico , Antígenos/análise , Antirreumáticos/uso terapêutico , Colchicina/uso terapêutico , Terapia Combinada , Teste de Coombs , Crioglobulinemia/complicações , Quimioterapia Combinada , Feminino , Alimentos Formulados/análise , Glomerulonefrite Membranoproliferativa/complicações , Glomerulonefrite Membranoproliferativa/terapia , Humanos , Hidroxicloroquina/uso terapêutico , Ramipril/uso terapêutico , Resultado do Tratamento , Vasculite Leucocitoclástica Cutânea/etiologia , Vasculite Leucocitoclástica Cutânea/terapiaRESUMO
We present a 28-year-old woman with mixed connective tissue disease (MCTD) complicated by a recalcitrant longstanding leg ulcer, which responded to complex therapy with local polydine, systemic ciprofloxacin, iloprost, enoxaparin and aspirin. Cyclophosphamide pulse therapy and corticosteroids controlled the systemic inflammation but failed to heal the leg ulcer. We considered a rationale of complex therapy for the leg ulcer on a basis of pathogenesis and complications of MCTD.
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Ciclofosfamida/administração & dosagem , Úlcera da Perna/tratamento farmacológico , Doença Mista do Tecido Conjuntivo/tratamento farmacológico , Pulsoterapia , Adulto , Ciclofosfamida/uso terapêutico , Quimioterapia Combinada , Feminino , Humanos , Úlcera da Perna/etiologia , Úlcera da Perna/patologia , Doença Mista do Tecido Conjuntivo/complicaçõesRESUMO
OBJECTIVE: To examine whether the release of superoxide anions from neutrophils of healthy donors was affected when incubated with plasma from infliximab-treated rheumatoid arthritis (RA) patients. METHODS: Fifteen consecutive seropositive RA patients were treated with 3mg/kg infliximab on weeks 0, 2, 6, and 14. Disease activity was assessed by DAS28 score and by IL-6 level. Neutrophils from healthy donors were incubated with plasma drawn before each infliximab treatment. PMA-stimulated superoxide release was measured by the ferricytochrome C reduction method. RESULTS: 53% of the patients had a favorable clinical response. IL-6 levels showed a significant decline at week two, with a gradual increase thereafter. Treatment with infliximab did not change the superoxide production. However, when the group was divided retrospectively to responders (DeltaDAS28 > -1.2) and non-responders (DeltaDAS28 < -1.2), two different patterns were seen, although the pre-treatment levels were similar: Among the responders IL-6 remained low at its 2 weeks level till week 14, while in the non responders IL-6 increased 3 times (P < 0.03) from week 2 to 14. The responders showed mild, but continuous, reduction of superoxide release, while in the non-responders it increased significantly from week 2 on. CONCLUSION: The reduction in IL-6 in RA sera following anti-TNFalpha therapy has little influence on the capacity of these sera to stimulate healthy neutrophils to produce superoxide, suggesting the existence of non-TNFalpha non-IL-6 dependent neutrophil-stimulating mediators in RA sera. The increasing level of IL-6 among the non-responders after initial dramatic decline might represent an escape phenomenon, possibly caused by alternative mediator(s). Clinically, this IL-6 "escape" might be used as a tool for early identification of responders from non-responders.
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Anticorpos Monoclonais/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Neutrófilos/metabolismo , Idoso , Anticorpos Monoclonais/imunologia , Antirreumáticos/imunologia , Artrite Reumatoide/imunologia , Células Cultivadas , Feminino , Humanos , Infliximab , Interleucina-6/sangue , Masculino , Pessoa de Meia-Idade , Neutrófilos/efeitos dos fármacos , Neutrófilos/imunologia , Índice de Gravidade de Doença , Superóxidos/metabolismo , Acetato de Tetradecanoilforbol/farmacologiaRESUMO
The etiology of arthritis episodes in normouricemic patients with gout is still unclear. We propose that the fluctuation in synovial urate level, as well as pH, ion strength, albumin, and globulin values relative to serum levels, could be involved in crystal formation. To assess serum-synovial gradient (SSG), the sera and synovial fluid (SF) of six normouricemic patients (men, age 48-79) with a history of gout (American College of Rheumatology criteria) and acute knee effusion were screened for uric acid, pH, osmolality (Osm), P/Ca, albumin, globulin, and SSG. Monosodium urate monohydrate (MSUM) crystals were determined by polarized light (PL). Infectious arthritis was ruled out via Gram staining and synovial fluid culture. Negative X-ray and PL microscopy results excluded chondrocalcinosis. Five patients (1-5) had inflammatory knee effusion (WBC >2,000/mm(3)), and one (patient 6) had noninflammatory knee effusion (600 WBC/mm(3)). MSUM crystals were found in the WBC of patient 1 only. He had tophaceous gout with normal serum uric acid levels and showed significant negative Osm and P and positive Ca SSG. Two crystal negative patients had severe negative pH SSG with alkaline synovial fluid, significant P/Ca SSG, and high positive globulin SSG, while one of them had supersaturated SF uric acid content. The other patients displayed an increased Osm and P/Ca SSG. All SSG values were five to ten times higher than the coefficient of variance for used methods. Noninflammatory SF of patient 6 does not appear to be related to active gout. The data on SSG for MSUM, pH, Osm, Alb/Glob, and P/Ca in normouricemic patients with gout history and acute knee effusion was not homogeneous. We propose that acid-base and ionic-protein gradient may lead to instability of subsaturated urate solution, thereby predisposing to MSUM deposits within synovial membrane and inducing inflammation.
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Gota/complicações , Hidrartrose/complicações , Articulação do Joelho , Prontuários Médicos , Líquido Sinovial/metabolismo , Ácido Úrico/sangue , Ácido Úrico/metabolismo , Doença Aguda , Idoso , Cristalização , Gota/sangue , Humanos , Leucócitos/metabolismo , Masculino , Pessoa de Meia-Idade , Valores de Referência , Ácido Úrico/químicaRESUMO
A young woman with transient osteoporosis of the hip (TOH) during pregnancy, severe pain, physical disability and marked generalized osteoporosis was treated with intravenous biphosphonates with prompt beneficial effects on both clinical symptoms and bone density. The features of this rare and sometimes underdiagnosed condition are reviewed. Intravenous biphosphonates seem to be an effective therapy for TOH.
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Analgésicos não Narcóticos/uso terapêutico , Antimetabólitos/uso terapêutico , Ácido Clodrônico/uso terapêutico , Articulação do Quadril , Osteoporose/tratamento farmacológico , Complicações na Gravidez/tratamento farmacológico , Absorciometria de Fóton , Adulto , Analgésicos não Narcóticos/administração & dosagem , Artrografia , Densidade Óssea/efeitos dos fármacos , Ácido Clodrônico/administração & dosagem , Feminino , Articulação do Quadril/diagnóstico por imagem , Articulação do Quadril/patologia , Humanos , Injeções Intravenosas , Articulação do Joelho/diagnóstico por imagem , Articulação do Joelho/patologia , Osteoporose/etiologia , Osteoporose/patologia , Gravidez , Complicações na Gravidez/patologia , Resultado do TratamentoRESUMO
A 37-year-old man with ischaemic finger necrosis and recent-onset Raynaud's phenomenon associated with cocaine abuse is reported. Initial therapy with systemic vasodilators, low-molecular-weight heparin and aspirin failed. Resolution of the ischaemia and ulcer healing was rapidly achieved with intravenous infusions of the prostacyclin analogue iloprost. The mechanism of vascular ischaemic injury and the development of secondary Raynaud's phenomenon due to cocaine use is discussed.
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Transtornos Relacionados ao Uso de Cocaína/complicações , Dedos/irrigação sanguínea , Isquemia/induzido quimicamente , Doença de Raynaud/induzido quimicamente , Adulto , Seguimentos , Humanos , Iloprosta/administração & dosagem , Infusões Intravenosas , Isquemia/tratamento farmacológico , Isquemia/patologia , Masculino , Necrose , Doença de Raynaud/tratamento farmacológico , Medição de RiscoRESUMO
OBJECTIVES: To review the literature on the immunomodulatory and anti-inflammatory properties of cotrimoxazole (CTX)-a combination of sulfamethoxazole and trimethoprim, to summarize the use of this medication in the treatment of autoimmune diseases, to stimulate and renew the interest of both physicians and researchers in this possible therapy for rheumatoid arthritis (RA), and to inspire further investigation in this field. METHODS: A MEDLINE search of the literature from 1966 until 2000 was performed, and information about the pharmacology of CTX and its use in the therapy of rheumatic diseases was critically reviewed. RESULTS: RA treatment is associated with numerous problems such as lack of efficacy, frequent side effects, and high cost. Analysis of the relevant literature revealed that experience with CTX in the treatment of RA is limited. However, the results of several nonrandomized and evidently forgotten clinical trials and laboratory investigations suggested that CTX might serve as an effective and inexpensive therapy for RA. Several lines of evidence suggested that CTX has nonspecific anti-inflammatory and immunomodulatory properties. Although nausea and vomiting were common reasons for CTX withdrawal, they were noted in only some studies, and no major organ toxicity was observed. CONCLUSIONS: Because of its therapeutic qualities, low cost, and relative nontoxicity, CTX seems to warrant a role in the treatment of RA.
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Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Anti-Inflamatórios/uso terapêutico , Antirreumáticos/farmacologia , Artrite Reumatoide/fisiopatologia , Ensaios Clínicos como Assunto , Quimioterapia Combinada , Sistema Imunitário/efeitos dos fármacos , Sistema Imunitário/fisiopatologia , MEDLINE , Resultado do Tratamento , Combinação Trimetoprima e Sulfametoxazol/farmacologiaRESUMO
This paper describes four patients with inflammatory joint diseases treated with methotrexate who developed lymphoma either of unusual type or with bizarre clinical features. The pathogenesis of this unusual condition is discussed and the difficulty in differentiating it from the features of the initial disease is emphasised.
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Artrite/complicações , Artrite/tratamento farmacológico , Transtornos Linfoproliferativos/etiologia , Metotrexato/uso terapêutico , Idoso , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Ticlopidine is a widely used drug for prevention of stroke and other serious vascular events with a multitude of possible side effects. An increasing number of drugs are being recognized as the triggering agents of drug-induced lupus. We describe three patients in whom the etiologic connection between ticlopidine and lupus was supported by the appearance of lupus-like features (fever, rash, arthritis, renal involvement, positive antinuclear and antihistone antibodies), shortly after drug initiation, and their gradual resolution after its discontinuation. If suggested by clinical or/and laboratory findings (fever of unknown cause, musculoskeletal involvement, hematologic abnormalities), the possibility of ticlopidine-induced lupus should be taken into consideration and appropriate investigations should be performed. Patients should resolve slowly but completely after withdrawal of ticlopidine.
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A patient with Crohn's disease developed proximal muscle weakness, increased serum creatine phosphokinase activity and electromyographic abnormalities. A muscle biopsy was non-diagnostic. Although rare, myositis should be included in the differential diagnosis of muscle weakness in patients with inflammatory bowel disease.