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2.
Zhonghua Jie He He Hu Xi Za Zhi ; 42(9): 700-704, 2019 Sep 12.
Artigo em Chinês | MEDLINE | ID: mdl-31484245

RESUMO

Objective: To explore the effect of pirfenidone in fibrotic interstitial pneumonia with autoimmune features (IPAF) after treatment with corticosteroids and immunosuppressants. Methods: We conducted a retrospective analysis of 2 adult patients with IPAF in the Peking Union Medical College Hospital. As their fibrotic interstitial lung disease failed to improve with further treatment with corticosteroids and immunosuppressants, they were treated with pirfenidone based on corticosteroids and immunosuppressants. Their clinical, chest radiological data and prognosis were collected and relevant literatures were reviewed. Results: One patient was a 43 year old female, the other was a 53 year old male. IPAF was diagnosed with their classic clinical, serological and radiological features. They were partially responded to corticosteroids and immunosuppressants at the initial period. Pirfenidone was suggested for them as their lung fibrosis was not improved further with immunosuppressive therapy. After 4-5 months treatment with pirfenidone, based on corticosteroids and immunosuppressant administration, their clinical and radiological manifestations improved significantly. Conclusions: Pirfenidone might be a good add-on choice for fibrotic IPAF when the disease did not respond well to corticosteroids and immunosuppressants.


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Doenças Autoimunes/diagnóstico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Piridonas/uso terapêutico , Corticosteroides/uso terapêutico , Adulto , Feminino , Humanos , Fibrose Pulmonar Idiopática/diagnóstico , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
3.
Vnitr Lek ; 65(7-8): 520-523, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31487996

RESUMO

The autoimmune form of pancreatitis (AIP) is divided into the following two subtypes. The 1st subtype is characterized by the high presence of immunoglobulin G4 in the blood serum and tissue of some organs which are in the close connection to the so called IgG4 associated disorders. The typical diagnostic signs of AIP are the histomorphological changes. This type of AIP is more frequent than the 2nd type with which has however some common histomorphological signs and mainly the positive response to the administered steroids used in the initial treatment. Whilst the 1st type of AIP is typically connected to the disorders of various organs such are the biliary tract, salivary and lacrimal glands, retroperitoneal fibrosis, reins, prostate gland and the next ones, the 2nd type of AIP is significantly connected to the inflammatory bowel disease only. Inflammatory bowel diseases are rarely present in the 1st type of AIP on the contrary. In our case report we mention the first published observations performed in the Czech Republic when the 1st type of AIP is succeeded by the inflammatory bowel disease - Crohn´s disease.


Assuntos
Doenças Autoimunes , Doenças Inflamatórias Intestinais , Pancreatite , Doenças Autoimunes/complicações , Doenças Autoimunes/diagnóstico , República Tcheca , Humanos , Imunoglobulina G , Doenças Inflamatórias Intestinais/complicações , Doenças Inflamatórias Intestinais/diagnóstico , Pancreatite/complicações , Pancreatite/diagnóstico
4.
BMC Infect Dis ; 19(1): 763, 2019 Sep 02.
Artigo em Inglês | MEDLINE | ID: mdl-31477035

RESUMO

BACKGROUND: Actinomycetes can rarely cause intracranial infection and may cause a variety of complications. We describe a fatal case of intracranial and intra-orbital actinomycosis of odontogenic origin with a unique presentation and route of dissemination. Also, we provide a review of the current literature. CASE PRESENTATION: A 58-year-old man presented with diplopia and progressive pain behind his left eye. Six weeks earlier he had undergone a dental extraction, followed by clindamycin treatment for a presumed maxillary infection. The diplopia responded to steroids but recurred after cessation. The diplopia was thought to result from myositis of the left medial rectus muscle, possibly related to a defect in the lamina papyracea. During exploration there was no abnormal tissue for biopsy. The medial wall was reconstructed and the myositis responded again to steroids. Within weeks a myositis on the right side occurred, with CT evidence of muscle swelling. Several months later he presented with right hemiparesis and dysarthria. Despite treatment the patient deteriorated, developed extensive intracranial hemorrhage, and died. Autopsy showed bacterial aggregates suggestive of actinomycotic meningoencephalitis with septic thromboembolism. Retrospectively, imaging studies showed abnormalities in the left infratemporal fossa and skull base and bilateral cavernous sinus. CONCLUSIONS: In conclusion, intracranial actinomycosis is difficult to diagnose, with potentially fatal outcome. An accurate diagnosis can often only be established by means of histology and biopsy should be performed whenever feasible. This is the first report of actinomycotic orbital involvement of odontogenic origin, presenting initially as bilateral orbital myositis rather than as orbital abscess. Infection from the upper left jaw extended to the left infratemporal fossa, skull base and meninges and subsequently to the cavernous sinus and the orbits.


Assuntos
Actinomicose/diagnóstico , Doenças Autoimunes/diagnóstico , Infecções Bacterianas do Sistema Nervoso Central/diagnóstico , Doenças Maxilares/microbiologia , Miosite Orbital/diagnóstico , Infecções Bacterianas do Sistema Nervoso Central/microbiologia , Diagnóstico Diferencial , Diplopia/diagnóstico , Diplopia/microbiologia , Evolução Fatal , Humanos , Masculino , Doenças Maxilares/complicações , Doenças Maxilares/diagnóstico , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/microbiologia , Extração Dentária/efeitos adversos
5.
JAMA ; 322(7): 632-641, 2019 08 20.
Artigo em Inglês | MEDLINE | ID: mdl-31429897

RESUMO

Importance: Maternal hypothyroidism and hyperthyroidism are risk factors for preterm birth. Milder thyroid function test abnormalities and thyroid autoimmunity are more prevalent, but it remains controversial if these are associated with preterm birth. Objective: To study if maternal thyroid function test abnormalities and thyroid autoimmunity are risk factors for preterm birth. Data Sources and Study Selection: Studies were identified through a search of the Ovid MEDLINE, EMBASE, Web of Science, the Cochrane Central Register of Controlled Trials, and Google Scholar databases from inception to March 18, 2018, and by publishing open invitations in relevant journals. Data sets from published and unpublished prospective cohort studies with data on thyroid function tests (thyrotropin [often referred to as thyroid-stimulating hormone or TSH] and free thyroxine [FT4] concentrations) or thyroid peroxidase (TPO) antibody measurements and gestational age at birth were screened for eligibility by 2 independent reviewers. Studies in which participants received treatment based on abnormal thyroid function tests were excluded. Data Extraction and Synthesis: The primary authors provided individual participant data that were analyzed using mixed-effects models. Main Outcomes and Measures: The primary outcome was preterm birth (<37 weeks' gestational age). Results: From 2526 published reports, 35 cohorts were invited to participate. After the addition of 5 unpublished data sets, a total of 19 cohorts were included. The study population included 47 045 pregnant women (mean age, 29 years; median gestational age at blood sampling, 12.9 weeks), of whom 1234 (3.1%) had subclinical hypothyroidism (increased thyrotropin concentration with normal FT4 concentration), 904 (2.2%) had isolated hypothyroxinemia (decreased FT4 concentration with normal thyrotropin concentration), and 3043 (7.5%) were TPO antibody positive; 2357 (5.0%) had a preterm birth. The risk of preterm birth was higher for women with subclinical hypothyroidism than euthyroid women (6.1% vs 5.0%, respectively; absolute risk difference, 1.4% [95% CI, 0%-3.2%]; odds ratio [OR], 1.29 [95% CI, 1.01-1.64]). Among women with isolated hypothyroxinemia, the risk of preterm birth was 7.1% vs 5.0% in euthyroid women (absolute risk difference, 2.3% [95% CI, 0.6%-4.5%]; OR, 1.46 [95% CI, 1.12-1.90]). In continuous analyses, each 1-SD higher maternal thyrotropin concentration was associated with a higher risk of preterm birth (absolute risk difference, 0.2% [95% CI, 0%-0.4%] per 1 SD; OR, 1.04 [95% CI, 1.00-1.09] per 1 SD). Thyroid peroxidase antibody-positive women had a higher risk of preterm birth vs TPO antibody-negative women (6.6% vs 4.9%, respectively; absolute risk difference, 1.6% [95% CI, 0.7%-2.8%]; OR, 1.33 [95% CI, 1.15-1.56]). Conclusions and Relevance: Among pregnant women without overt thyroid disease, subclinical hypothyroidism, isolated hypothyroxinemia, and TPO antibody positivity were significantly associated with higher risk of preterm birth. These results provide insights toward optimizing clinical decision-making strategies that should consider the potential harms and benefits of screening programs and levothyroxine treatment during pregnancy.


Assuntos
Doenças Autoimunes/diagnóstico , Iodeto Peroxidase/imunologia , Complicações na Gravidez/diagnóstico , Nascimento Prematuro/etiologia , Doenças da Glândula Tireoide/diagnóstico , Testes de Função Tireóidea , Adulto , Autoanticorpos/sangue , Doenças Autoimunes/sangue , Doenças Autoimunes/complicações , Feminino , Idade Gestacional , Humanos , Hipotireoidismo/complicações , Hipotireoidismo/diagnóstico , Recém-Nascido , Gravidez , Complicações na Gravidez/sangue , Doenças da Glândula Tireoide/sangue , Doenças da Glândula Tireoide/complicações , Tireotropina/sangue , Tiroxina/sangue
6.
Autoimmun Rev ; 18(10): 102363, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31401342

RESUMO

The contribution of autoimmune phenomena to dysfunction of hypophysis or hypothalamus is far to be unraveled and also the specific pathways of hypophysitis are poorly understood until now, mostly for the pediatric population. Primary hypophysitis is rare in children and often regarded as an autoimmune disorder, following the evidence of lymphoplasmacytic infiltration in the pituitary gland, detection of anti-pituitary antibodies (APA) and anti-hypotalamus antibodies (AHA) by indirect immunofluorescence on cryostatic sections of human or primate hypophysis and hypothalamus, and coexistence with other autoimmune disorders. The rarity of this condition and the lack of ad hoc studies make hard any assessment of the real incidence of hypophysitis in pediatric patients, and also the role of APA and AHA has been poorly investigated in children with idiopathic hypopituitarism. Potential target autoantigens studied in autoimmune hypophysitis have been various pituitary-specific factors, chaperone proteins, alpha-enolase, secretogranins, chorionic somatomammotropin and intracellular transcription factors. Many clinical features both endocrine and neurologic or systemic can herald the onset of autoimmune hypophysitis. Antidiuretic hormone deficiency with central diabetes insipidus and growth retardation are the most significant presenting symptoms in children with hypophysitis, requiring a careful differential diagnosis with other causes of hypopituitarism, including tumors of the sellar region, differently from adults in whom adrenal insufficiency, hypogonadism, headache or diplopia might be the leading manifestations. Growth hormone deficiency is found in 3/4 of pediatric cases. Five histologic variants of primary hypophysitis have been described: lymphocytic, granulomatous, xanthomatous, IgG4-related and necrotizing: lymphocytic hypophysitis is the most frequent variant in the pediatric sceneries. Children with diagnosis of hypophysitis are also at risk of developing germinomas later in life, and require an extended follow-up in the long-term. Therapeutic options should be differentiated according to the rapidity of disease progression and modality of clinical onset, as acute pictures might require corticosteroids or immunosuppressant agents, while chronic forms may need a conservative management or appropriate hormone replacement therapies. This review updates and summarizes the most recent information related to the autoimmune involvement of hypophysis and hypothalamus in children, discusses the correlations between APA, AHA and disease activity, as well as the recommendations for treatment of primary hypophysitis from the pediatric perspective.


Assuntos
Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Doenças da Hipófise/diagnóstico , Doenças da Hipófise/terapia , Doenças Autoimunes/imunologia , Criança , Humanos , Doenças da Hipófise/imunologia , Prognóstico
7.
Arq Gastroenterol ; 56(2): 232-241, 2019 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-31460591

RESUMO

New data concerning the management of autoimmune liver diseases have emerged since the last single-topic meeting sponsored by the Brazilian Society of Hepatology to draw recommendations about the diagnosis and treatment of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), overlap syndromes of AIH, PBC and PSC and specific complications and topics concerning AIH and cholestatic liver diseases. This manuscript updates those previous recommendations according to the best evidence available in the literature up to now. The same panel of experts that took part in the first consensus document reviewed all recommendations, which were subsequently scrutinized by all members of the Brazilian Society of Hepatology using a web-based approach. The new recommendations are presented herein.


Assuntos
Doenças Autoimunes/diagnóstico , Doenças Autoimunes/terapia , Hepatopatias/diagnóstico , Hepatopatias/terapia , Colangite Esclerosante/diagnóstico , Colangite Esclerosante/terapia , Gerenciamento Clínico , Hepatite Autoimune/diagnóstico , Hepatite Autoimune/terapia , Humanos , Cirrose Hepática Biliar/diagnóstico , Cirrose Hepática Biliar/terapia , Sociedades Médicas
8.
Rev Med Chil ; 147(3): 334-341, 2019 Mar.
Artigo em Espanhol | MEDLINE | ID: mdl-31344171

RESUMO

Acquired hemophilia A (AHA) is a rare and life-threatening autoimmune hemorrhagic disorder where autoantibodies are developed against factor VIII. An early diagnosis is challenging and mandatory: an immediate hemostatic control is required to reduce morbidity and mortality. Laboratory features of AHA are: presence of autoantibodies against factor VIII, prolonged activated partial thromboplastin time (with normal prothrombin time and thrombin time) and decreased factor VIII levels. In some cases, the results of laboratory tests may be incorrect due to errors in analysis, blood extraction or manipulation of samples; also worth of consideration are limitations in the measurement range and low sensitivity of the tests. This review highlights the importance of adequate screening in patients with suspected AHA to make an adequate diagnosis and reduce overall fatal outcomes.


Assuntos
Hemofilia A/diagnóstico , Autoanticorpos/sangue , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/fisiopatologia , Testes de Coagulação Sanguínea , Diagnóstico Precoce , Fator VIII , Hemofilia A/fisiopatologia , Humanos , Tempo de Tromboplastina Parcial
9.
Pan Afr Med J ; 32: 181, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31312295

RESUMO

Introduction: The term anti-nuclear antibody (ANA) is used to define a large group of autoantibodies which specifically bind to nuclear elements. Although healthy individuals may also have ANA positivity, the measurement of ANA is generally used in the diagnosis of autoimmune disorders. However, various studies have shown that ANA testing may be overused, especially in pediatrics clinics. Our aim was to investigate the reasons for antinuclear antibody (ANA) testing in the general pediatrics and pediatric rheumatology clinics of our hospital and to determine whether ANA testing was ordered appropriately by evaluating chief complaints and the ultimate diagnoses of these cases. Methods: The medical records of pediatric patients in whom ANA testing was performed between January 2014 and June 2016 were retrospectively evaluated. Subjects were grouped according to the indication for ANA testing and ANA titers. Results: ANA tests were ordered in a total of 409 patients during the study period, with 113 positive ANA results. The ANA test was ordered mostly due to joint pain (50% of the study population). There was an increased likelihood of autoimmune rheumatic diseases (ARDs) with higher ANA titer. The positive predictive value of an ANA test was 16% for any connective tissue disease and 13% for lupus in the pediatric setting. Conclusion: in the current study, more than one-fourth of the subjects were found to have ANA positivity, while only 15% were ultimately diagnosed with ARDs. Our findings underline the importance of an increased awareness of correct indications for ANA testing.


Assuntos
Anticorpos Antinucleares/sangue , Doenças Autoimunes/diagnóstico , Doenças do Tecido Conjuntivo/diagnóstico , Doenças Reumáticas/diagnóstico , Adolescente , Instituições de Assistência Ambulatorial , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/imunologia , Criança , Pré-Escolar , Doenças do Tecido Conjuntivo/epidemiologia , Doenças do Tecido Conjuntivo/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Valor Preditivo dos Testes , Estudos Retrospectivos , Doenças Reumáticas/epidemiologia , Doenças Reumáticas/imunologia , Turquia/epidemiologia
11.
Rinsho Ketsueki ; 60(5): 392-397, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31168001

RESUMO

Distinguishing between IgG4-related disease (IgG4-RD) and hyper-interleukin (IL) -6 syndrome, such as immune mediated conditions, autoimmune diseases, and idiopathic multicentric Castleman disease (iMCD) is challenging. Here, we report the case of a 69-year-old man with cervical lymphadenopathy who was admitted to our hospital and histologically diagnosed with hyper-IL-6 syndrome mimicking IgG4-RD phenotypically. Laboratory data detected polyclonal hypergammaglobulinemia comprising IgG, including IgG4 (2,350 mg/dl). Computed tomography revealed presence of systemic lymphadenopathy, enlarged bilateral submandibular glands, and infiltrative shadow in the right lower lung. Magnetic resonance imaging revealed diffusely enlarged pancreas the size of a sausage and hypointense rim on T2, suggesting autoimmune pancreatitis as part of IgG4-RD. Biopsy of the cervical lymph node revealed proliferation of IL-6-positive mature plasma cells in the expanded interfollicular area with an elevated IgG4+/IgG+ cell ratio (approximately 70%). These histological findings were consistent with hyper-IL-6 syndrome rather than IgG4-RD; however, the serum IL-6 level was slightly elevated. Bone marrow aspiration detected both IgG4- and IL-6-positive mature plasma cells. Although this case cannot be diagnosed as IgG4-RD because it failed to meet its diagnostic criteria, administration of oral prednisolone (0.5 mg/kg) resulted in rapidly improved lymphadenopathy, enlarged pancreas, and serological findings. This report can be helpful for the diagnostic assessment of polyclonal hypergammaglobulinemia conditions.


Assuntos
Doenças Autoimunes/diagnóstico , Doença Relacionada a Imunoglobulina G4 , Interleucina-6/análise , Idoso , Diagnóstico Diferencial , Humanos , Masculino
14.
Nihon Shokakibyo Gakkai Zasshi ; 116(5): 443-451, 2019.
Artigo em Japonês | MEDLINE | ID: mdl-31080225

RESUMO

In the course of treatment for myasthenia gravis, enlargement of a cystic mass in the liver with peripheral bile duct dilation, diffuse pancreatic enlargement, and serum IgG4 level elevation was identified in a 65-year-old man. Following the diagnosis of autoimmune pancreatitis, a left hepatectomy was performed because of suspected malignancy of the cystic lesion. Analysis of the resected specimen revealed the cystic lesion to be a dilated bile duct. Intraductal papillary tumor comprising fibrovascular stalks covered by neoplastic epithelium was identified in the lesion. Infiltration of IgG4-positive plasma cells was discovered around the cystic lesion. Finally, a diagnosis of intraductal papillary neoplasm of bile duct with IgG4-related sclerosing cholangitis was made. Autoimmune diseases, including IgG4-related diseases, require careful observation because of their potential for malignancy.


Assuntos
Doenças Autoimunes/diagnóstico , Colangite Esclerosante/diagnóstico , Imunoglobulina G/metabolismo , Miastenia Gravis/diagnóstico , Pancreatite/diagnóstico , Idoso , Doenças Autoimunes/complicações , Ductos Biliares , Colangite Esclerosante/complicações , Humanos , Masculino , Miastenia Gravis/complicações , Pancreatite/complicações
15.
Dig Dis ; 37(5): 416-421, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31079114

RESUMO

BACKGROUND: Diagnosis of pancreatic cancer (PC) in early stages is still challenging for gastroenterologists. The early detection of cancer is one of the utmost importance for the successful therapy of this malignancy. An accurate differential diagnosis of focal pancreatic lesions plays also an important role, whether it is differential diagnosis of chronic pancreatitis from PC or autoimmune pancreatitis (AIP) from PC. Raised serum immunoglobulin G4 (IgG4) levels to twice the normal value are considered one of significant diagnostic features of type 1 AIP. However, IgG4 can be increased also in patients with PC, but levels usually do not exceed twice the normal value. METHODS: In years 2012-2017, IgG4 serum levels were examined in 115 patients with histologically confirmed PC. Patients with PC and elevated IgG4 level (above 135 mg/dL) had tested their histological resection specimens or bioptic specimens from pancreatic lesion, with targeted detection of the presence of IgG4 and plasmocytes in the pancreatic tissue and changes characteristic for type 1 AIP. RESULTS: A plasmatic IgG4 level in 115 patients with diagnosed PC was higher than 135 mg/dL in 14 patients (12.2%). Out of them, 2 patients (1.7%) revealed a serum IgG4 level higher than double the normal value, that is, higher than 270.0 mg/dL (suggestive of AIP). One patient met histological criteria for diagnosis of AIP in the simultaneous presence of PC. CONCLUSION: Diagnosis of early cancer stages, particularly differentiating AIP from PC can be sometimes problematic. IgG4 levels can be slightly elevated also in case of PC. A targeted biopsy of the pancreas is the method of choice in cases suspected from a focal form of AIP and we recommend to prefer it over other modalities, such as, for example, response to steroid therapy.


Assuntos
Doenças Autoimunes/diagnóstico , Neoplasias Pancreáticas/diagnóstico , Pancreatite Crônica/diagnóstico , Idoso , Doenças Autoimunes/sangue , Doenças Autoimunes/diagnóstico por imagem , Doenças Autoimunes/patologia , Doença Crônica , Diagnóstico Diferencial , Endossonografia , Feminino , Humanos , Imunoglobulina G/sangue , Masculino , Pessoa de Meia-Idade , Neoplasias Pancreáticas/sangue , Neoplasias Pancreáticas/diagnóstico por imagem , Neoplasias Pancreáticas/patologia , Pancreatite Crônica/sangue , Pancreatite Crônica/diagnóstico por imagem , Pancreatite Crônica/patologia
16.
Adv Clin Chem ; 90: 25-80, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31122611

RESUMO

Acute-phase reactant serum amyloid A (A-SAA) plays an important role in acute and chronic inflammation and is used in clinical laboratories as an indicator of inflammation. Although both A-SAA and C-reactive protein (CRP) are acute-phase proteins, the detection of A-SAA is more conclusive than the detection of CRP in patients with viral infections, severe acute pancreatitis, and rejection reactions to kidney transplants. A-SAA has greater clinical diagnostic value in patients who are immunosuppressed, patients with cystic fibrosis who are treated with corticoids, and preterm infants with late-onset sepsis. Nevertheless, for the assessment of the inflammation status and identification of viral infection in other pathologies, such as bacterial infections, the combinatorial use of A-SAA and other acute-phase proteins (APPs), such as CRP and procalcitonin (PCT), can provide more information and sensitivity than the use of any of these proteins alone, and the information generated is important in guiding antibiotic therapy. In addition, A-SAA-associated diseases and the diagnostic value of A-SAA are discussed. However, the relationship between different A-SAA isotypes and their human diseases are mostly derived from research laboratories with limited clinical samples. Thus, further clinical evaluations are necessary to confirm the clinical significance of each A-SAA isotype. Furthermore, the currently available A-SAA assays are based on polyclonal antibodies, which lack isotype specificity and are associated with many inflammatory diseases. Therefore, these assays are usually used in combination with other biomarkers in the clinic.


Assuntos
Reação de Fase Aguda , Doença , Inflamação/sangue , Inflamação/diagnóstico , Proteína Amiloide A Sérica/análise , Amiloidose/sangue , Amiloidose/diagnóstico , Amiloidose/metabolismo , Animais , Doenças Autoimunes/sangue , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/metabolismo , Humanos , Inflamação/metabolismo , Hepatopatias/sangue , Hepatopatias/diagnóstico , Hepatopatias/metabolismo , Doenças Metabólicas/sangue , Doenças Metabólicas/diagnóstico , Doenças Metabólicas/metabolismo , Neoplasias/sangue , Neoplasias/diagnóstico , Neoplasias/metabolismo , Proteína Amiloide A Sérica/metabolismo
19.
Hautarzt ; 70(4): 277-282, 2019 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-30941468

RESUMO

Autoimmune blistering skin disorders represent a rare group of autoantibody-induced dermatoses against desmosomal and hemidesmosomal molecules. The common age of onset for pemphigus and pemphigoid, as well as dermatitis herpetiformis, encompasses the adult age, but all these disorders can be observed neonatally and/or during childhood. If the disease occurs postpartum or neonatally, physicians should consider transplacental transmission of pathogenic maternal immunoglobulin G (IgG)-autoantibodies, and both mother and child should be included in the diagnostic work up. If the disorder is suspected in childhood, early immunoserological testing and skin biopsies for direct immunofluorescence analyses are recommended for the correct diagnosis and subsequently for the right choice of treatment. First-line recommendations are nonhalogenated topical steroids, followed by oral dapsone. All therapies require preliminary examinations, e. g. enzyme-activity testing (as is glucose-6-phophate dehydrogenase in dapsone treatment). In refractory cases, further treatment choices like high-dose intravenous immunoglobins, plasmapheresis/immunoadsorption or targeted therapies like anti-CD20 autoantibody therapies are indicated. An intense dermatological support and good medical care are essential for an age-appropriate development of the child and to lower possible treatment-associated adverse events.


Assuntos
Autoanticorpos/imunologia , Doenças Autoimunes/diagnóstico , Vesícula , Imunoglobulina A/imunologia , Penfigoide Bolhoso/imunologia , Pênfigo/imunologia , Dermatopatias Vesiculobolhosas/diagnóstico , Autoanticorpos/sangue , Doenças Autoimunes/imunologia , Criança , Humanos , Inflamação/complicações , Penfigoide Bolhoso/fisiopatologia , Dermatopatias Vesiculobolhosas/imunologia
20.
Rev Bras Ginecol Obstet ; 41(3): 203-205, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30939605

RESUMO

INTRODUCTION: Autoimmune progesterone dermatitis (APD) is a rare autoimmune dermatosis characterized by recurrent cutaneous and mucosal lesions during the luteal phase of the menstrual cycle that disappear some days after the menses. CASE REPORT: A 34-year-old primipara woman with no significant past medical history and no prior exogenous hormone use, who presented with cyclic skin eruptions starting 1 year after the delivery. The lesions occurred ∼ 6 days before the menses and disappeared in between 1 and 2 days after the menstruation ceased. The patient was diagnosed after a positive response to an intradermal test with progesterone and was successfully treated with combined oral contraceptives. The skin eruptions have not returned since the initiation of this therapy. CONCLUSION: Dermatologists, gynecologists, and obstetricians should be aware of this rare entity. Furthermore, if this condition is suspected, a thorough history taking on the menstrual cycle and results of the intradermal progesterone test are mandatory.


Assuntos
Doenças Autoimunes/tratamento farmacológico , Anticoncepcionais Orais Combinados/administração & dosagem , Dermatite/tratamento farmacológico , Distúrbios Menstruais/tratamento farmacológico , Progesterona/efeitos adversos , Adulto , Androstenos/administração & dosagem , Doenças Autoimunes/diagnóstico , Dermatite/diagnóstico , Etinilestradiol/administração & dosagem , Feminino , Humanos , Distúrbios Menstruais/diagnóstico , Recidiva , Testes Cutâneos , Resultado do Tratamento
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