RESUMO
Crohn's disease (CD) is an inflammatory disease of the gastrointestinal system. It is suspected to be caused by a combination of genetic and environmental factors and changes in the patient's intestinal microbiome. As opposed to colitis ulcerosa, which usually only affects the large intestine, CD may affect various parts of the gastrointestinal tract. Many patients show oral lesions related to CD, which sometimes can precede the first gastrointestinal symptoms. Specific oral lesions include granulomatous cheilitis, cobblestone-like mucosal tags and linear ulcerations. Non-specific lesions occur in the form of angular cheilitis and opportunistic candidiasis, aphthous ulcerations, reduced salivation, dental caries and many more. We present two cases of patients suffering from Crohn's disease with different oral manifestations and discuss the dentist's role in early detection of specific oral lesions and the importance of the follow-up care of affected patients.
Assuntos
Doença de Crohn , Humanos , Doença de Crohn/complicações , Doença de Crohn/diagnóstico , Adulto , Masculino , Feminino , Doenças da Boca/etiologia , Doenças da Boca/diagnósticoRESUMO
Paradoxical tuberculosis reaction is defined as the aggravation of lesions present at diagnosis or the development of new lesions under anti-tuberculosis treatment, after exclusion of other alternate causes. It affects 5 to 30% of tuberculosis patients, with a variable prevalence depending on the site of infection and the clinical background. The diagnosis of paradoxical reaction is one of elimination, and requires having ruled out therapeutic failure, notably linked to poor compliance and/or to the presence of mycobacterial antibiotic resistance. The severity of paradoxical tuberculosis reaction lies in its neurological impairment. Despite its clinical importance, the mechanisms involved remain poorly understood and its management is not consensual. Corticosteroids are the cornerstone in the medical management. The role of anti-TNF agents, currently proposed in cases of corticodependence or corticoresistance, remains to be properly defined.
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Antituberculosos , Tuberculose , Humanos , Antituberculosos/uso terapêutico , Antituberculosos/efeitos adversos , Tuberculose/diagnóstico , Tuberculose/epidemiologia , Tuberculose/tratamento farmacológico , Tuberculose/complicaçõesRESUMO
Chronic Granulomatosis Disease (CGD) is an inherited immune deficiency due to a mutation in the genes coding for the subunits of the NADPH oxidase enzyme that affects the oxidative capacity of phagocytic cells. It is characterized by increased susceptibility to bacterial and fungal infections, particularly Aspergillus, as well as complications associated with hyperinflammation and granulomatous tissue infiltration. There exist two types of frequently encountered pulmonary manifestations: (1) due to their being initially pauci-symptomatic, possibly life-threatening infectious complications are often discovered at a late stage. Though their incidence has decreased through systematic anti-bacterial and anti-fungal prophylaxis, they remain a major cause of morbidity and mortality; (2) inflammatory complications consist in persistent granulomatous mass or interstitial pneumoniae, eventually requiring immunosuppressive treatment. Pulmonary complications recurring since infancy generate parenchymal and bronchial sequelae that impact functional prognosis. Hematopoietic stem cell allograft is a curative treatment; it is arguably life-sustaining and may limit the morbidity of the disease. As a result of improved pediatric management, life expectancy has increased dramatically. That said, new challenges have appeared with regard to adults: difficulties of compliance, increased inflammatory manifestations, acquired resistance to anti-infectious therapies. These different developments underscore the importance of the transition period and the need for multidisciplinary management.
Assuntos
Doença Granulomatosa Crônica , Adulto , Humanos , Criança , Doença Granulomatosa Crônica/complicações , Doença Granulomatosa Crônica/diagnóstico , Doença Granulomatosa Crônica/terapia , NADPH Oxidases/genética , NADPH Oxidases/uso terapêutico , Bactérias , Pulmão , MutaçãoRESUMO
INTRODUCTION AND OBJECTIVES: Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic autoantibody (ANCA)-associated systemic vasculitis and is characterized by inflammation of blood vessels. The aim of the present study was to assess cardiac valvular changes in patients with GPA in a cohort of 105 patients followed for a mean of six years. METHODS: We followed 105 patients (mean age 50.4 years, 67 female) for a mean of 6.2±1.3 years. Echocardiography and laboratory tests were performed in all patients. RESULTS: At baseline, 43% of patients were diagnosed with aortic regurgitation (AR), which was the most common valvular lesion. Moreover, it was the only valvular involvement that significantly increased during observation (p=0.01). In a multivariate model, only D-dimer level was a predictor of AR in this group of patients (OR 8.0 (95% CI: 1.7-38.2, p=0.01). CONCLUSIONS: Involvement of the heart valves is a common finding in patients with GPA, but significant valvular disease is a rare complication. The most common valvular disease in this group of patients is AR. Aortic valves are also the most prone to degeneration in the course of the vasculitis.
Assuntos
Granulomatose com Poliangiite , Doenças das Valvas Cardíacas , Humanos , Feminino , Pessoa de Meia-Idade , Granulomatose com Poliangiite/complicações , Catéteres , Valvas Cardíacas , Inflamação , Doenças das Valvas Cardíacas/diagnóstico por imagem , Doenças das Valvas Cardíacas/etiologiaRESUMO
INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of necrotizing vasculitis affecting small vessels and typically characterized by severe glucocorticoid (GC)-dependent eosinophilic asthma. While mepolizumab, which is indicated at a dose of 100mg/4weeks in severe eosinophilic asthma, has been shown to be an effective treatment for EGPA-related asthma at a dose of 300mg/4weeks, it was only recently approved at this dose. METHODS: This retrospective, single-center, observational study was conducted to investigate over a 5-year period (2014-2019) the effect of mepolizumab 100mg/4weeks at 12months in patients with EGPA and glucocorticoid-dependant severe asthma. Response to treatment was defined as reduction in daily dose of oral corticosteroids to at most 5mg/day or reduction in annual exacerbation by at least 50%. RESULTS: Thirty patients were included, of whom twenty-three were treated (two were not fully evaluable). Among the 21 evaluable treated patients, 13 (62%) had responded at 12months. At baseline, non-responders had lower FEV1 levels and lower blood eosinophil levels than responders. CONCLUSIONS: Mepolizumab at a "severe asthma" dose (100mg/4weeks) is effective in treatment of GC-dependent severe asthma in most patients with EGPA.
Assuntos
Asma , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Humanos , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Granulomatose com Poliangiite/tratamento farmacológico , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/tratamento farmacológico , Glucocorticoides/uso terapêutico , Estudos Retrospectivos , Asma/complicações , Asma/diagnóstico , Asma/tratamento farmacológicoRESUMO
Systemic diseases (connective disease, granulomatosis) may be associated with peripheral neuropathies. The diagnosis can be complex when the neuropathy is the presenting manifestation of the disease, requiring close collaboration between neurologists and internists. Conversely, when the systemic disease is already known, the main question remaining is its imputability in the neuropathy. Regardless of the situation, the positive diagnosis of neuropathy is based on a systematic and rigorous electro-clinical investigation, specifying the topography, the evolution and the mechanism of the nerve damage. Certain imaging examinations, such as nerve and/or plexus MRI, or other more invasive examinations (skin biopsy, neuromuscular biopsy) enable to specify the topography and the mechanism of the injury. The imputability of the neuropathy in the course of a known systemic disease is based mainly on its electro-clinical pattern, on which the alternatives diagnoses depend. In the case of an inaugural neuropathy, a set of arguments orients the diagnosis, including the underlying terrain (young subject), possible associated systemic manifestations (inflammatory arthralgias, polyadenopathy), results of first-line laboratory tests (lymphopenia, hyper-gammaglobulinemia, hypocomplementemia), autoantibodies (antinuclear, anti-native DNA, anti-SSA/B) and sometimes invasive examinations (neuromuscular biopsy).
Assuntos
Doenças do Tecido Conjuntivo , Doenças do Sistema Nervoso Periférico , Humanos , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/etiologia , Autoanticorpos , Anticorpos Antinucleares , Doenças do Tecido Conjuntivo/complicaçõesRESUMO
A Histiocitose de células de Langerhans (HCL) é uma doença caracterizada pela proliferação anormal dessas células dentríticas em um tecido, que podem acometer uma única estrutura ou órgão ou ainda ocorrer simultaneamente em mais de uma região. Na cavidade oral geralmente a HCL pode se apresentar como uma hipertrofia gengival e ulceração da mucosa, provocando dor, edema, mobilidade, perda dentária, e destruição do tecido ósseo da maxila e da mandíbula. O objetivo desse trabalho foi de reportar a evolução clínica de um caso de HCL com lesões maxilo-mandibulares em uma paciente adulta. A paciente de 27 anos, feoderma, sexo feminino, foi admitida na Clínica de Estomatologia e Cirurgia Bucomaxilofacial do Hospital Metropolitano Odilon Behrens (HMOB), onde foi diagnosticada com HCL. Houveram cinco manifestações da doença desde 2012 até 2022, sendo todas restritas ao tecido ósseo e se estendendo aos tecidos moles mucogengivais, acometendo duas vezes a mandíbula, duas vezes a maxila, e uma vez na região occiptal do crânio, este último caso tratado pela neurologia. A paciente foi submetida ao tratamento cirúrgico e quimioterápico. O tratamento cirúrgico consistiu na exérese das manifestações da HCL em mandíbula e maxila, sendo que na mandíbula E, foi realizada a ressecção segmentar com margem de segurança após fratura patológica da mandíbula. No momento a paciente encontra-se em acompanhamento pelas clínicas de Cirurgia BMF e Médica Oncológica, realizando novo regime quimioterápico, devido a constantes recidivas. A HCL pode apresentar aspectos clínicos e radiográficos comuns a outras patologias que são encontrados com frequência na clínica odontológica, portanto, é de extrema importância que o cirurgião dentista saiba reconhecer as características clínicas e imaginológicas que possam sugerir o diagnóstico de HCL para melhor conduzir o caso afim de se obter o diagnóstico precoce dessa patologia.
Langerhans cell histiocytosis (LCH) is a disease characterized by the abnormal proliferation of these dendritic cells in a tissue, which can affect a single structure or organ or even occur simultaneously in more than one region. In the oral cavity, LCH can usually present as gingival hypertrophy and mucosal ulceration, causing pain, swelling, mobility, tooth loss, and destruction of bone tissue in the maxilla and mandible. The objective of this work was to report the clinical evolution of a case of LCH with maxillomandibular lesions in an adult patient. The patient of 27 years old, female, feoderm, was admitted to the Clinic of Stomatology and Oral and Maxillofacial Surgery at the Metropolitan Hospital Odilon Behrens (MHOB), where she was diagnosed with LCH. There were five manifestations of the disease from 2012 to 2022, all of which were restricted to bone tissue and extended to mucogingival soft tissues, affecting twice the mandible, twice the maxilla, and once in the occipital region of the skull, the latter case treated by neurology. The patient underwent surgical and chemotherapy treatment. The surgical treatment consisted of the excision of the manifestations of LCH in the mandible and maxilla, and in left mandible, segmental resection was performed after a pathological fracture of the mandible. At the moment, the patient is being followed up by the BMF Surgery and Medical Oncology clinics, undergoing a new chemotherapy regimen, due to constant recurrences. LCH can present clinical and radiographic aspects common to other pathologies that are frequently found in the dental practice, therefore, It is extremely important that the dental surgeon knows how to recognize the clinical and imaging characteristics that may suggest the diagnosis of LCH in order to better manage the case in order to obtain an early diagnosis of this pathology.
Assuntos
Relatos de Casos , Histiocitose de Células de Langerhans , Doenças Raras , Traumatismos MaxilofaciaisRESUMO
Introdução: no cenário da pandemia de COVID-19, muitos sintomas respiratórios têm sido atribuídos a tal doença, deixando de lado a ampliação de possíveis diagnósticos diferenciais, principalmente em casos atípicos que podem simular outras patologias, como a Granulomatose com Poliangeíte (GPA) e outras vasculites. Objetivo: ressaltar a importância do diagnóstico diferencial em sintomáticos respiratórios e compreender o espectro de manifestações das vasculites como a GPA. Descrição do caso: homem, 61 anos, branco, hipertenso, diabético, ex-tabagista que iniciou quadro de tosse e dispneia com exame de imagem sugestivo de pneumonia, sendo levantada hipótese de COVID-19. Entretanto, com rt-PCR para SARS-Cov-2 negativo e surgimento de novos sintomas como emagrecimento, febre e astenia associados a piora rápida de função renal, outras hipóteses foram levantadas, dentre elas o diagnóstico final de Granulomatose com Poliangeíte, confirmada por biópsia renal eanticorpos positivos, sendo tratada com metilprednisolona e ciclofosfamida, com paciente tendo desfechos favoráveis. Discussão: a GPA é uma vasculite ANCAassociada que acomete artérias de pequeno calibre e possui apresentação variada, desde sintomas de vias aéreas superiores até quadros graves de síndrome pulmãorim com risco de óbito. Conclusão: a GPA, apesar de rara, deve entrar no diagnóstico diferencial de quadros respiratórios de apresentação atípica ou associads a disfunção renal e outras manifestações sistêmicas, por se tratar de quadro grave e com boa resposta quando a terapia adequada é iniciada de maneira precoce.
Assuntos
Humanos , Masculino , FemininoRESUMO
AIM OF THE STUDY: Our study aimed to identify the characteristics of cardiac involvement in eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: We conducted a retrospective analytic study including EGPA cases diagnosed between 2000 and 2019 in an internal medicine department. Diagnosis was made according to the 1990 American College of Rheumatology criteria and the 2012 Chapel Hill Concensus. RESULTS: Eleven EGPA cases were included, 64% of patients were female. Median age at diagnosis was 52 years [42-58]. Heart damage revealed EGPA in 55% of cases with a significant predominance of women (p=0.015). The main cardiac manifestations were myocarditis, ischemic cardiomyopathy due to small vessel vasculitis, cardiac tamponade and intracardiac thrombus. Cardiac magnetic resonance imaging (MRI) mainly showed subendocardial hyposignal in early infusion and late enhancement in the same areas, nodular by locations, associated with impaired left ventricle function and micro-infarctions by distal vasculitis. Cardiac damage was associated to ANCA negativity in 83.3% of cases. The median Birmingham Vasculitis Activity Score version3 (BVAS v3) was 16 [10-17]. Under conventional treatment, no relapses had occurred. The median vasculitis damage index (VDI) was 2 [1-2.3] and the mortality rate was zero after a mean follow-up of 43 months. CONCLUSION: Cardiomyopathy is a frequent revealing mode of EGPA. A late onset asthma and hypereosinophilia should guide the diagnosis. As ANCA research often turns out to be negative, histological evidence is recommended in this context. The contribution of cardiac MRI in the diagnosis of EGPA remains to be defined.
Assuntos
Tamponamento Cardíaco , Síndrome de Churg-Strauss , Granulomatose com Poliangiite , Anticorpos Anticitoplasma de Neutrófilos , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/diagnóstico , Feminino , Granulomatose com Poliangiite/complicações , Granulomatose com Poliangiite/diagnóstico , Humanos , Masculino , Estudos RetrospectivosRESUMO
Introdução: A granulomatose de Wegener é uma doença autoimune rara que ocorre através de mediadores imunológicos. Sua etiologia permanece desconhecida. No entanto, sabe-se que é caracterizada principalmente pela inflamação dos vasos sanguíneos que acometem preferencialmente as vias aéreas superiores, inferiores e os rins. A doença apresenta altas taxas de morbidade e mortalidade quando não tratada, seu principal tratamento é através do uso de corticoides e imunossupressores. Objetivo: realizar um relato de caso de Granulomatose de Wegener tratando a comunicação orosinusal através de prótese bucomaxilofacial e uma discussão baseada na literatura recente. Relato de caso: paciente do gênero masculino, 40 anos de idade, leucoderma, encaminhado ao serviço de Bucomaxilofacial do Hospital Universitário da Universidade Federal de Santa Catarina (HU-UFSC) queixando-se de comunicação oronasal em região de palato. Foi tratado com prótese BMF obturadora, o que devolveu ao paciente a capacidade de fonação, deglutição e convívio social. Conclusão: o tratamento com a prótese BMF reestabelece o velamento velofaríngeo, corrige hipernasalidade, melhora a deglutição e dá conforto psicológico ao paciente com estabilidade protética funcional... (AU)
Introduction: Wegeners' Granulomatulosis is a rare autoimmune disease that acts through immunologic mediators. It's etymology remains unknown. However, it is known that it's mainly characterized by the inflammation of blood vessels that affect, by preference, upper and lower airways, as well as kidneys. The disease presents high rates of morbidity and mortality when not treated, and it's main treatments are corticoids and immunosuppressants. Objective: Perform a Wegener's Granulomatulosis case report treating orosinusal communication through the use of a bucomaxilofacial prosthesis and a discussion based on current literature. Case report: Male patient, 40 years of age, leucoderma, brought to the Bucomaxilofacial department of Federal University of Santa Catarina (HU UFSC) with complaints regarding oronasal communication in the palate region. The patient was treated with a BMF prosthetics, which has allowed the patient to resume normal social activities, as well as returning phonation and deglutition. Conclusion: Treatment with a BMF prosthetics reestablishes velopharyngeal veiling, assists in the correction of nasal voice, improves deglutition and offers the patient psychological comfort with functional prosthetic stability... (AU)
Introducción: la granulomatosis de Wegener es una enfermedad autoinmune rara que se presenta a través de mediadores inmunológicos. Su etiologia permanece desconocida. Sin embargo, se sabe que se caracteriza principalmente por la inflamación de los vasos sanguíneos que afectan preferentemente las vías respiratorias superiores e inferiores y los riñones. La enfermedad tiene altas tasas de morbilidad y mortalidad cuando no se trata, su principal tratamiento es mediante el uso de corticosteroides e inmunosupresores. Objetivo: realizar un reporte de caso de Granulomatosis de Wegener en el tratamiento de la comunicación orosinusal mediante prótesis maxilofacial y una discusión basada en la literatura reciente. Caso clínico: paciente de sexo masculino, 40 años, leucoderma, remitido al Servicio Maxilofacial del Hospital Universitario de la Universidad Federal de Santa Catarina (HU-UFSC) por comunicación oronasal en región paladar. Fue tratado con una prótesis obturatriz de BMF, que le devolvió al paciente la capacidad de hablar, tragar y socializar. Conclusión: el tratamiento con la prótesis BMF restablece el velo velofaríngeo, corrige la hipernasalidad, mejora la deglución y proporciona confort psicológico al paciente con estabilidad protésica funcional... (AU)
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Humanos , Masculino , Adulto , Doenças Autoimunes , Granulomatose com Poliangiite , Prótese Maxilofacial , Palato , Fonação , Vasos Sanguíneos , Mortalidade , Corticosteroides , Deglutição , ImunossupressoresRESUMO
Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystem disorder characterized by asthma, peripheral blood eosinophilia, and signs of vasculitis. Glucocorticoids are considered the cornerstone of treatment, but most patients remain steroid-dependent and carry a significant burden of adverse effects. We report a case of a patient with steroid-dependent EGPA successfully treated with mepolizumab. A 36-year-old man presented with persistent rhinitis, dyspnea, wheezing, and dry cough poorly controlled with inhaled therapy. Eosinophilia in peripheral blood and bronchoalveolar lavage fluid was seen. Histological findings from nasal mucosa revealed eosinophilic microabscesses and vasculitis without granulomas compatible with EGPA diagnosis. After daily oral prednisolone (PSL) was started, symptoms and eosinophilia improved, but adverse effects emerged. Attempts at tapering off PSL resulted in worsening of symptoms. He started mepolizumab 300 mg monthly, with clinical improvement and sustained disease remission, which allowed reducing the need for PSL. We present a very disabling steroiddependent EGPA. Mepolizumab was able to taper off PSL while maintaining symptomatic control.
Granulomatose eosinofílica com poliangiite (EGPA) é uma doença multissistêmica caracterizada por asma, eosinofilia no sangue periférico e sinais de vasculite. Os corticoides são considerados a base do tratamento, no entanto, a maioria dos pacientes permanece dependente deste tratamento com os seus efeitos adversos associados. Relatamos o caso de um paciente com granulomatose eosinofílica dependente de esteroides com poliangiite (EGPA) tratado com sucesso com mepolizumabe. Um homem de 36 anos apresentou rinite persistente, dispneia, sibilos e tosse seca mal controlada com terapia inalada. Observou-se eosinofilia no sangue periférico e no lavado broncoalveolar. Os achados histológicos da mucosa nasal revelaram microabscessos eosinofílicos e vasculite sem granulomas compatíveis com o diagnóstico de EGPA. Após o início da prednisolona oral diária (PSL), os sintomas e a eosinofilia melhoraram, mas surgiram efeitos adversos. As tentativas de redução gradual da PSL resultaram no agravamento dos sintomas. Iniciou mepolizumabe 300 mg mensalmente, com melhora clínica e remissão sustentada da doença, o que permitiu reduzir a necessidade de PSL. Apresentamos um EGPA dependente de esteroides muito incapacitante. O mepolizumab foi capaz de diminuir o PSL mantendo o controle sintomático sustentado.
Assuntos
Humanos , Masculino , Adulto , Prednisolona , Granulomatose com Poliangiite , Corticosteroides , Anticorpos Monoclonais Humanizados , Sinais e Sintomas , Terapêutica , Sons Respiratórios , Rinite , Interleucina-5 , Tosse , Diagnóstico , Dispneia , EosinofiliaRESUMO
INTRODUCTION: Cat scratch disease caused by Bartonella henselae with bone involvement is a rare presentation. CASE REPORT: We report a case of disseminated bartonellosis with multifocal osteomyelitis and multiple visceral involvement in an immunocompetent adult. Diagnostic confirmation was obtained by PCR on lymphadenopathy. In addition to our observation, 31 cases of bartonellosis with bone involvement were reported in the literature. Diagnosis is based on a combination of history, serology and PCR performed on tissue. The antibiotic treatment allows recovery in all cases. CONCLUSION: Cat scratch disease in its systemic form with bone involvement is a rare and difficult diagnosis for the clinician and an invasive approach is often required to obtain the diagnosis.
Assuntos
Bartonella henselae , Doença da Arranhadura de Gato , Osteomielite , Antibacterianos/uso terapêutico , Doença da Arranhadura de Gato/complicações , Doença da Arranhadura de Gato/diagnóstico , Humanos , Osteomielite/diagnóstico , Reação em Cadeia da PolimeraseRESUMO
Silicosis and sarcoidosis are two very distinct entities in the literature. All the additional non-invasive examinations, including the chest CT scan, often do not differentiate them. The history, including occupational exposure to identified silica particles, is a discriminating factor. However, due to the pathogenic power of silica, it would be possible to have the simultaneous development of these two pathologies in the same patient. To illustrate this situation, here is the case of a 62-year-old patient, who presented initially with a picture of dyspnea and productive cough. The chest CT showed micronodular peribronchovascular infiltrates and mediastinal lymphadenopathy. The other additional examinations did not find anything specific. In the diagnostic process, the patient had multiple endoscopic samples which did not make it possible to be conclusive on one or the other of these pathologies. He therefore underwent a surgical lung biopsy which revealed histological lesions compatible with the two pathologies. Recent studies suggest that inhaled particles, especially silica, could be responsible for the pattern of sarcoidosis. However, it is difficult to say whether, in this case, silica was responsible for the development of sarcoidosis.
Assuntos
Sarcoidose , Silicose , Humanos , Pulmão/diagnóstico por imagem , Masculino , Pessoa de Meia-Idade , Sarcoidose/diagnóstico , Dióxido de Silício/toxicidade , Silicose/diagnóstico , Silicose/etiologia , Tomografia Computadorizada por Raios XRESUMO
A Rare Cause of Hypercalcemia Abstract. Sarcoidosis is a rare cause of hypercalcemia, which is in most cases caused by primary hyperparathyroidism or a malignant tumor. Other rare causes include vitamin D intoxication, other granulomatous diseases (such as tuberculosis, or fungal infection) or mutiple myeloma. Because of the broad differential diagnosis, a systematic diagnostic approach is crucial to establish the correct diagnosis. Therapeutic measures include volume replacement and subsequent administration of bisphosphonates. If hypercalcemia is caused by sarcoidosis, therapy with systemic corticosteroids is indicated.
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Hipercalcemia , Neoplasias , Sarcoidose , Diagnóstico Diferencial , Difosfonatos , Humanos , Hipercalcemia/diagnóstico , Hipercalcemia/etiologia , Hipercalcemia/terapia , Sarcoidose/complicações , Sarcoidose/diagnósticoRESUMO
Common variable immunodeficiency disorders (CVID) are the most common symptomatic primary antibody deficiency in adults with an estimated prevalence of 1/25,000. The most frequent clinical manifestations are upper respiratory tract infections (including pneumonia, bronchitis, and sinusitis) predominantly with Streptococcus pneumoniae or H. influenzae. However, CVID are complicated in 20 to 30 % of cases of non-infectious manifestations which have been well characterized in recent years. Several complications can be observed including autoimmune, lymphoproliferative, granulomatous or cancerous manifestations involving one or more organs. These complications, mostly antibody-mediated cytopenias, are correlated with a decrease in the number of circulating switched memory B cells. Replacement therapy with polyvalent gammaglobulins has greatly improved the prognosis of these patients but it remains poor in the presence of digestive complications (especially in the case of chronic enteropathy and/or porto-sinusoidal vascular disease), pulmonary complications (bronchiectasis and/or granulomatous lymphocytic interstitial lung disease) and when progression to lymphoma. Much progress is still to be made, in particular on the therapeutic management of non-infectious complications which should benefit in the future from targeted treatments based on knowledge of genetics and immunology.