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1.
Autoimmun Rev ; 19(2): 102447, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31843713

RESUMO

OBJECTIVE: To evaluate the prevalence, clinical presentation, serological and morphological features of, and therapeutic options for Interstitial Lung Disease (ILD) in primary Sjögren's Syndrome (pSS). METHODS: Pubmed was searched between February 1996 and December 2018 using a combination of MESH terms related to pSS and ILD. Selected works were subjected to blind evaluation by two authors and a senior author in case of disagreement. The work followed PRISMA guidelines and was registered on PROSPERO (CRD42018118669). RESULTS: About 20% of pSS patients have ILD, with a 5-y survival of 84% and a need for supplemental oxygen in the 11-33% range. A significant proportion of ILD patients are seronegative without sicca syndrome. ILD seems to be associated with higher levels of Lactic Dehydrogenases and positivity for Anti-Ro52k. The prevalent pattern in High Resolution Computed Tomography is Nonspecific Interstitial Pneumonia (NSIP), but all other patterns can be present. No difference in mortality was found between patients with NSIP and Usual Interstitial Pneumonia patterns. Amyloidosis and primary lung lymphoma can be observed in about 10% of pSS patients. CONCLUSION: The recognition of pSS underlying an ILD can be challenging in seronegative patients with no or mild sicca symptoms. A complete diagnostic assessment, including minor salivary glands and, in some cases, lung biopsy, should be performed on all patients at risk. A better recognition of the clinical or serological markers of ILD progression in these patients is warranted to drive the physicians to an early diagnosis and an effective treatment.


Assuntos
Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/patologia , Síndrome de Sjogren/complicações , Humanos , Itália/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Prognóstico , Reumatologia , Sociedades Médicas
2.
An Bras Dermatol ; 94(6): 710-712, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31789250

RESUMO

Although the association of multiple autoimmune diseases has already been widely described, no reports of the association between vitiligo, primary biliary cirrhosis and Sjogren's syndrome were retrieved in the SciELO and PubMed databases. The authors describe the case of a female patient who was diagnosed with primary biliary cirrhosis and Sjogren's syndrome at age 54. At age 58, she developed vitiligo restricted to the face, associated with significant impairment of self-esteem and quality of life. Antinuclear antibody was negative at the onset of the condition, but became positive after phototherapy initiation. In general, the occurrence of multiple autoimmune diseases in the same patient is known as a mosaic of autoimmunity. However, specific mechanisms appear to interconnect primary biliary cirrhosis and Sjogren's syndrome, such as PDC-E2-mediated generalized epithelitis.


Assuntos
Cirrose Hepática Biliar/complicações , Síndrome de Sjogren/complicações , Vitiligo/complicações , Autoimunidade , Doença Crônica , Feminino , Humanos , Cirrose Hepática Biliar/patologia , Pessoa de Meia-Idade , Síndrome de Sjogren/patologia , Vitiligo/patologia
3.
Zhonghua Kou Qiang Yi Xue Za Zhi ; 54(12): 829-834, 2019 Dec 09.
Artigo em Chinês | MEDLINE | ID: mdl-31874483

RESUMO

Objective: To analyze the clinical characteristics of oral candidiasis (OC) in in-patients with rheumatism, in order to provide theoretical basis for the prevention and treatment of OC in rheumatism patients. Methods: One thousand eight hundred and eight in-patients were recruited in the Department of Rheumatology, the Second Hospital of Shanxi Medical University from January 2017 to December 2017. The patients included 607 males and 1 201 females. Their average age was (49.5±15.5) years old with a ranging from 14 to 81 years. According to occurrence of OC or not, the patients were divided into OC group and non-OC group. The differences of general data, primary diseases, laboratory examinations, usage of glucocorticoid and immunosuppressant therapy were compared between the two groups, and the risk factors of OC occurrence were analyzed. Results: Two hundred and sixty-nine patients had OC and 1 539 patients had no OC. Age [(54.9±14.7) years], duration of illness [(9.4±4.4) years] and hospital stay [(15.3±5.7) d] in OC group were significantly longer than those in non-OC group. OC incidence in patients with connective tissue disease (CTD) [17.40% (193/1 109)] was higher than that in non-CTD patients [10.87% (75/699)] (P<0.001). OC most likely occurred in patients with such CTD as Sjögren syndrome (SS) and Behcet syndrome. OC incidence in non-CTD patients with osteoarthritis (OA) was highest. The salivary flow rate in OC group [(0.65±0.45) ml/min] was significantly lower than that in non-OC group [(0.78± 0.39) ml/min] (t=2.394, P=0.017). There was no statistical differences in other laboratory examinations between the two groups, including white blood cells (WBC), lymphocyte, platelet count, liver function, renal function, erythrocyte sedimentation rate, C-reactive protein, procalcitonin, immunoglobulin G, immunoglobulin M, immunoglobulin A, C(3), C(4) and so on. OC incidence in patients using prednisone≥15 mg/d [17.16% (133/775)] was higher than that in patients using prednisone<15 mg/d [12.53% (94/750)] and patients not using prednisone [14.84% (42/283)] (P<0.05). The incidence of OC in patients with immunosuppressant therapy [16.11% (226/1 403)] was statistically higher than that in non-immunosuppressant patients [10.62% (43/405)] (P<0.01). Logistic regression analysis showed that the risk factors of OC occurrence included primary diseases (P<0.001), age (P<0.001), duration of illness (P=0.001) and duration of hospitalization (P=0.002). Conclusions: OC occurred commonly in rheumatism in-patients, especially in elder patients, patients with long duration of illness and hospital stay. OC incidence in CTD patients is significantly higher than that in non-CTD patients. Glucocorticoid and immunosuppressant therapy might significantly reduce the anti-fungal immunity of the patients.


Assuntos
Candidíase Bucal/complicações , Doenças Reumáticas/complicações , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Síndrome de Behçet/complicações , Feminino , Humanos , Incidência , Pacientes Internados , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Síndrome de Sjogren/complicações , Adulto Jovem
5.
Best Pract Res Clin Rheumatol ; 33(4): 101440, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31585842

RESUMO

Skin lesions occur, often at very early stages, in many of the most frequent inflammatory rheumatic diseases such as in systemic lupus erythematosus (SLE), dermatomyositis (DM), systemic sclerosis (SSc), Sjögren's syndrome, rheumatoid arthritis (RA), and psoriatic arthritis. It is important to recognize the different specific cutaneous lesions in SLE (e.g., "butterfly" rash in acute, annular or psoriasiform photosensitive lesions in the subacute form, and discoid lesions in the chronic form) for an early diagnosis and to estimate the associated risks of internal disease, whereas nonspecific lesions (exanthema, vasculitis, and alopecia) can be part of SLE flares. Cutaneous lesions in DM (Gottron's papules and sign, heliotrope rash, dystrophic cuticles, and nailfold capillary abnormalities) may occur before any clinically evident muscular or systemic organ involvement and are of utmost importance for early diagnosis. The pattern of cutaneous lesions and associated autoantibodies also allow the distinction of different phenotypes, either more prone to life-threatening interstitial lung disease (MDA-5) or with higher risk for neoplasia (TIF1-γ). Many other skin lesions, although not specific, require further investigation to look for a possible underlying inflammatory rheumatic disease: non-pruritic urticarial lesions in anti-C1q-associated urticarial vasculitis, Still's disease or hereditary auto-inflammatory syndromes, transient macular purpura of vasculitis in Sjögren's syndrome, Behçet's disease, or RA, Raynaud's phenomenon in SSc and mixed connective tissue disease, erythema nodosum or other panniculitis in RA, Behçet's disease and SLE, pustular eruptions in Behçet's disease, psoriasis, and hereditary auto-inflammatory syndromes. After reviewing in detail the cutaneous manifestations of the most frequent inflammatory rheumatic diseases, we describe a topographic and morphological approach to skin rashes, calling attention to facial rashes, hand involvement, scalp, nail, or leg lesions or to some morphological aspects of skin lesions (annular, pustular, urticarial, or exanthematous) that may be the initial manifestations of inflammatory rheumatic diseases. The importance of skin lesions is confirmed by their presence as part of the classification criteria of many inflammatory rheumatic diseases. They also contribute to early diagnosis, to characterize disease phenotypes, to aid in effective patient management, and, ultimately, to impact on disease prognosis.


Assuntos
Artrite Reumatoide , Dermatomiosite , Exantema , Lúpus Eritematoso Sistêmico , Doenças Reumáticas , Síndrome de Sjogren , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Exantema/etiologia , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Doenças Reumáticas/complicações , Doenças Reumáticas/diagnóstico , Síndrome de Sjogren/complicações , Síndrome de Sjogren/diagnóstico , Dermatopatias
6.
J Med Case Rep ; 13(1): 309, 2019 Oct 16.
Artigo em Inglês | MEDLINE | ID: mdl-31615546

RESUMO

BACKGROUND: The association of macrophage activation syndrome and primary Sjögren's syndrome has been rarely reported in the literature. We report the first observation of this association in Africa, south of the Sahara, and we discuss the diagnosis and therapeutic challenge. CASE PRESENTATION: A 26-year-old Mauritanian and Berber woman was followed for primary Sjögren's syndrome. After a voluntary cessation of her usual background treatment, she was admitted to our department for an outbreak of her illness. A clinical examination revealed anemic syndrome, peripheral polyarthritis, coughing rales at both pulmonary bases, and fever at 39.5 °C. On biologic examination, there was bicytopenia with anemia at 5.70 g/dl, lymphopenia at 796/mm3, a biological inflammatory syndrome with a sedimentation rate at 137 mm in the first hour, C-reactive protein at 97 mg/l, hyperferritinemia at 1778 mg/l (9 normal value), and hypergammaglobulinemia at 20.7 g/l of polyclonal appearance. The triglycerides were 1.95 g/l (1.4 normal value) and the lactate dehydrogenase level was 491 IU/l (1.5 normal value). Cytological examination of a medullary puncture revealed an image of hemophagocytosis. An infectious screening was negative. Thoracic computed tomography showed non-specific interstitial lung disease. A diagnosis of macrophage activation syndrome complicating primary Sjögren's syndrome was selected with a probability of 97.2%, according to H-Score. The evolution was favorable under a treatment including etoposide (VP-16). CONCLUSION: Macrophage activation syndrome is a rare entity, rarely reported during primary Sjögren's syndrome. Its spontaneous evolution is invariably fatal. There is no consensus on therapeutic treatment. Etoposide is a therapeutic option especially in forms refractory to corticosteroid therapy.


Assuntos
Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Sjogren/complicações , Adulto , África ao Sul do Saara , Antineoplásicos Fitogênicos/uso terapêutico , Etoposídeo/uso terapêutico , Feminino , Ferritinas/sangue , Febre/etiologia , Humanos , Hipertrigliceridemia/etiologia , Síndrome de Ativação Macrofágica/complicações , Síndrome de Ativação Macrofágica/tratamento farmacológico , Fagocitose
7.
J Drugs Dermatol ; 18(10): 995-998, 2019 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-31584777

RESUMO

Background: Autoimmune connective tissue diseases (ACTDs) are a broad range of diseases featured by immune dysregulation, and often have multisystem involvement with prominent skin manifestations. Pruritus is one of the most common symptoms in these diseases, with significant impact on the quality of life of patients. Objective: To characterize the frequency, location, severity, and timing relative to disease onset of pruritus in different ACTDs. Methods: A chart review of all patients seen in the Rheumatology-Dermatology clinic at Massachusetts General Hospital. Results: Itch was a troubling symptom in 83% of dermatomyositis (DM), 61% of systemic lupus erythematosus (SLE), 59% of Sjogren syndrome (SJO), 22% of systemic sclerosis (SSc), and 60% of mixed connective tissue disease. In DM and SLE, itch paralleled the course of inflammatory skin manifestations in 83% and 45%, respectively. Itch in DM is more intense and more treatment resistant in 12% vs 1% in SLE. In contrast, itch in SSc and SJO tended to occur later in the disease course, 86% vs 42%, respectively. Conclusion: Itch is common in all ACTDs and often under-evaluated and under treated. Pruritus is more common and more severe in DM than in SLE. Treatment of pruritus in ACTDs can be challenging, and sometimes multi-modal therapy is warranted. J Drugs Dermatol. 2019;18(10):995-998.


Assuntos
Dermatomiosite/complicações , Lúpus Eritematoso Sistêmico/complicações , Prurido/diagnóstico , Escleroderma Sistêmico/complicações , Síndrome de Sjogren/complicações , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Terapia Combinada/métodos , Dermatomiosite/imunologia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Masculino , Pessoa de Meia-Idade , Prurido/imunologia , Prurido/terapia , Qualidade de Vida , Estudos Retrospectivos , Escleroderma Sistêmico/imunologia , Índice de Gravidade de Doença , Síndrome de Sjogren/imunologia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
9.
Med. clín (Ed. impr.) ; 153(8): 305-311, oct. 2019. tab
Artigo em Inglês | IBECS | ID: ibc-185414

RESUMO

Objectives: The aim of this study is to identify the clinical characteristics of primary Sjögren's syndrome (PSS) patients with pulmonary involvement and the associated factors for pulmonary involvement in PSS. Methods: We retrospectively reviewed clinical features, laboratory examinations, imaging tests, pathological results and therapeutic strategy of 367 PSS patients. Comparisons were made between two subgroups: PSS with pulmonary involvement and those without. Correlation between the pathology of minor salivary gland biopsy (MSGB) and diverse features with pulmonary involvement were detected by Pearson correlation analysis and associated factors were selected by multivariate logistic regression analysis. Results: The lung involved PSS patients had significantly higher level of inflammatory associated indexes (p<0.05). There is no significant correlation between pathology of MSGB and lung involvements. Age, elevated neutrophils level and hypoproteinemia are significantly associated with lung disease with in PSS cohort (p<0.05). As for therapeutic strategy, moderate dose prednisone (15-40mg/d) and cyclophosphamide (CTX) are mainly different between two subgroups. Conclusions: PSS patients with pulmonary involvements show enhanced inflammation. Age, elevated neutrophils level and hypoproteinemia are independent associated with pulmonary involvements in PSS patients. For those PSS with pulmonary involvement moderate dose of prednisone and CTX were still the mainstream


Objetivos: El objetivo de este estudio es identificar las características clínicas de los pacientes con afectación pulmonar en el síndrome de Sjögren primario (SSp), y los factores relacionados con la afectación pulmonar en el SSp. Métodos: Hemos revisado retrospectivamente las características clínicas, los análisis de laboratorio, las pruebas de imagen, los resultados patológicos y la estrategia terapéutica de 367 pacientes con SSp. Se realizaron comparaciones entre 2 subgrupos: SSp con afectación pulmonar y SSp sin afectación pulmonar. La correlación entre la patología de la biopsia de la glándula salival menor (BGSM) y diversas características con afectación pulmonar se detectó mediante el análisis de correlación de Pearson, y los factores asociados se seleccionaron mediante un análisis de regresión logística multivariable. Resultados: Los pacientes con afectación pulmonar en el SSp tenían niveles significativamente más altos de índices inflamatorios asociados (p<0,05). No encontramos una correlación significativa entre la patología de la BGSM y la afectación pulmonar. La edad, el nivel elevado de neutrófilos y la hipoproteinemia se asociaron de manera independiente con la enfermedad pulmonar en la cohorte de SSp (p<0,05). En cuanto a la estrategia terapéutica: prednisona en dosis moderada (15-40mg/d) y ciclofosfamida (CTX) fueron los principales medicamentos entre los 2 subgrupos. Conclusiones: Los pacientes con afectación pulmonar en el SSp tenían una inflamación más elevada que el grupo de pacientes con SSp sin afectación pulmonar. La edad, el nivel elevado de neutrófilos y la hipoproteinemia se asocian de manera independiente con la afectación pulmonar en pacientes con el SSp. Para aquellos pacientes con afectación pulmonar en el SSp, el tratamiento más común fue una dosis moderada de prednisona y CTX


Assuntos
Humanos , Masculino , Adulto , Pessoa de Meia-Idade , Idoso , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/complicações , Estudos de Coortes , Pneumopatias/patologia , Estudos Retrospectivos , Modelos Logísticos , Glândulas Salivares Menores/patologia , Biópsia , Hipoproteinemia/complicações , Pneumopatias/diagnóstico , Pneumopatias/tratamento farmacológico , Prednisona/administração & dosagem , Análise Multivariada
11.
Invest Ophthalmol Vis Sci ; 60(12): 3708-3716, 2019 09 03.
Artigo em Inglês | MEDLINE | ID: mdl-31479110

RESUMO

Purpose: Sjögren syndrome (SS) is an autoimmune disease characterized by the inflammatory destruction of salivary and lacrimal glands (LG). Chloroquine (CQ) was known as an immunomodulatory drug and in the inhibition of autophagy. The purpose of the study is to investigate the effect of CQ on the development of dry eye in NOD-LtJ mice. Methods: NOD-LtJ mice were observed, during which the occurrence of dry eye was confirmed by tear secretion, corneal staining, and the infiltration of foci into the LG from 13-week-old mice. Intraperitoneal (IP) administration of CQ was performed in 13-week-old mice for 4 weeks and maintained untreated for another 4 weeks. Additionally, CQ was injected IP in 19-week-old mice for 2 weeks from when the disease was fully developed. Results: Interestingly, the expression of autophagy marker ATG5 and LC3B-II was observed in the LG from week 5. When CQ had been administered for 4 weeks from week 13 and then maintained untreated for 4 weeks, tear secretion, corneal staining score, foci formation in the LG, conjunctival goblet cells and proinflammatory cytokine expressions were significantly better than untreated mice. The infiltration of immune cells and the expression of autophagy markers in LG were decreased in the CQ group. These indices improved significantly as well when the 19-week-old mice with severe clinical phenotypes had been treated with CQ for 2 weeks. Conclusions: This study demonstrated that autophagy was induced in the early stages of the SS model and that CQ treatment in the early stages could inhibit disease progression.


Assuntos
Antirreumáticos/farmacologia , Cloroquina/farmacologia , Modelos Animais de Doenças , Síndromes do Olho Seco/prevenção & controle , Síndrome de Sjogren/complicações , Animais , Autofagia/efeitos dos fármacos , Proteína 5 Relacionada à Autofagia/sangue , Biomarcadores/metabolismo , Córnea/metabolismo , Córnea/patologia , Síndromes do Olho Seco/diagnóstico , Síndromes do Olho Seco/etiologia , Ensaio de Imunoadsorção Enzimática , Feminino , Células Caliciformes/efeitos dos fármacos , Células Caliciformes/patologia , Injeções Intraperitoneais , Aparelho Lacrimal/metabolismo , Aparelho Lacrimal/patologia , Camundongos , Camundongos Endogâmicos BALB C , Camundongos Endogâmicos NOD , Proteínas Associadas aos Microtúbulos/sangue , Reação em Cadeia da Polimerase em Tempo Real , Glândulas Salivares/metabolismo , Glândulas Salivares/patologia , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/metabolismo , Lágrimas/fisiologia
12.
Int J Pediatr Otorhinolaryngol ; 125: 196-198, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31374539

RESUMO

Parotitis is a frequent disease in general pediatrics. Pneumoparotitis is a rare affection that belongs to differential diagnoses of parotitis, along with infections, lymphadenitis, autoimmune disorders, inflammatory conditions, vascular malformations or neoplasms. It is usually described in musicians using wind instruments or in other situations involving a Vasalva maneuver. We report the case of a 12 years old boy with severe idiopathic pneumoparotitis without any of these well-known causes and whose autoimmune familial background of Sjögren syndrome might be relevant.


Assuntos
Enfisema/diagnóstico , Parotidite/diagnóstico , Criança , Diagnóstico Diferencial , Enfisema/etiologia , Humanos , Masculino , Parotidite/etiologia , Síndrome de Sjogren/complicações
13.
BMJ Case Rep ; 12(8)2019 Aug 13.
Artigo em Inglês | MEDLINE | ID: mdl-31413059

RESUMO

We present a 44-year-old female with an initial presentation with distal renal tubular acidosis (RTA) after she presented with hypokalaemia and normal anion gap acidosis. Three years following the diagnosis, she presented with progressive renal impairment. In the absence of any clinical, biochemical and radiological clues, she underwent a renal biopsy which showed severe tubulitis secondary to lymphocytic infiltration. Serological investigations subsequently revealed positive anti-nuclear, anti-Sjögren's syndrome related antigen A (SS-A), and anti-Sjögren's syndrome related antigen B (SS-B) antibodies, supporting the diagnosis of Sjögren's syndrome. This case is unique in that distal RTA was the presenting clinical manifestation of Sjögren's syndrome. We hope that a consideration for Sjögren's syndrome is made in patients with seemingly idiopathic RTA.


Assuntos
Acidose Tubular Renal/diagnóstico , Síndrome de Sjogren/diagnóstico , Acidose Tubular Renal/sangue , Acidose Tubular Renal/complicações , Acidose Tubular Renal/urina , Adulto , Feminino , Humanos , Hipopotassemia/sangue , Hipopotassemia/complicações , Hipopotassemia/diagnóstico , Hipopotassemia/urina , Síndrome de Sjogren/sangue , Síndrome de Sjogren/complicações , Síndrome de Sjogren/urina , Urinálise
14.
Clin Exp Rheumatol ; 37 Suppl 118(3): 78-82, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31365332

RESUMO

OBJECTIVES: To characterise the sleep profile of patients with primary Sjögren's syndrome (pSS) and its relationship between hyper-somnolence and other clinical parameters. METHODS: In phase one of the study, we utilised cross-sectional data on daytime hyper-somnolence from the United Kingdom Primary Sjögren's Syndrome Registry (UKPSSR) cohort (n=857, female=92.7%). Phase two relied on clinical data from a cohort of patients (n=30) with PSS, utilising symptom assessment questionnaires and sleep diaries. RESULTS: Within the UKPSSR, daytime hyper-somnolence was prevalent (ESS, 8.2±5.1) amongst pSS patients with a positive correlation between daytime hyper-somnolence and fatigue (Spearman's rs = 0.42, p<0.0001). Amongst the clinical cohort, 100% of patients had problematic sleep. Participants with pSS awoke frequently (NWAK, 2.2±1.3), had difficulty in returning back to sleep (WASO, 59.9±50.2 min vs. normal of <30min) and a reduced sleep efficiency (SE, 65.7±18.5% vs. >85%). Fatigue (FIS, 82.4 ±33.5) and orthostatic symptoms (OGS, 6.7 ±3.7) remained high in these patients. CONCLUSIONS: Sleep disturbances are a problem in pSS, comprising difficulty in maintaining sleep, frequent awakenings throughout the night and difficulties in returning back to sleep. As such, the total time in bed without sleep is much greater and sleep efficiency greatly reduced. These patients in addition have a high symptomatic burden possibly contributing to and/or contributed by poor and disordered sleep.


Assuntos
Síndrome de Sjogren , Transtornos do Sono-Vigília/etiologia , Estudos de Coortes , Estudos Transversais , Fadiga , Feminino , Humanos , Masculino , Fenótipo , Síndrome de Sjogren/complicações , Reino Unido
15.
Int J Rheum Dis ; 22(9): 1775-1781, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31368254

RESUMO

AIM: This study aimed to retrospectively describe 15 new primary Sjögren's syndrome-pulmonary arterial hypertension (pSS-PAH) cases confirmed by right heart catheterization (RHC). Demographic and clinical characteristics were analyzed and risk factors for PAH in pSS were explored. METHOD: We retrospectively described 15 new pSS-PAH cases confirmed by RHC referred to our institution between January 2013 and March 2018. We present PAH and pSS characteristics, hemodynamic evaluations, medical management, and outcomes. A matched case control study was carried out to determine the risk factors of PAH in pSS compared with pSS-non-PAH patients. RESULTS: All patients were female with a mean age at PAH diagnosis of 52.9 ± 14.6 years. The delay between the first symptom and PAH diagnosis was 18.7 ± 19.7 months. The most common primary manifestation at PAH onset was exertional dyspnea (13/15). At diagnosis of PAH, PAH was severe with a mean pulmonary artery pressure of 48.8 ± 13.7 mm Hg (range, 27-72 mm Hg) and a mean cardiac index of 2.3 ± 0.6 L/min/m2 (range, 1.47-3.41 L/min/m2 ). Compared with the pSS-PAH without pericardial effusion, pSS-PAH with pericardial effusion had larger right arterial (53 [45-56.75] vs 38 [35.5-46.5], P = .018) and right ventricular sizes (47 [42.75-51.25] vs 36 [32.5-41], P = .007). Compared with the pSS non-PAH group, we identified 2 risk factors for PAH in pSS: pericardial effusion (odds ratio [OR] [95% CI], 14.29 [1.14-166.67], P = .039) and liver involvement (OR [95% CI], 14.71 [1.14-166.67], P = .035). CONCLUSION: For pSS patients, PAH can be the first manifestation. We believe that systemic evaluation, especially in patients with pericardial effusion and liver involvement, is important to identify high-risk patients for PAH, improving their prognosis.


Assuntos
Pressão Arterial , Artéria Pulmonar/fisiopatologia , Síndrome de Sjogren/complicações , Adulto , Idoso , Anti-Hipertensivos/uso terapêutico , Pressão Arterial/efeitos dos fármacos , China , Dispneia/etiologia , Dispneia/fisiopatologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Hepatopatias/etiologia , Hepatopatias/fisiopatologia , Pessoa de Meia-Idade , Derrame Pericárdico/etiologia , Derrame Pericárdico/fisiopatologia , Prognóstico , /tratamento farmacológico , Artéria Pulmonar/efeitos dos fármacos , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/tratamento farmacológico
16.
Clin Exp Rheumatol ; 37 Suppl 118(3): 249-251, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31464665

RESUMO

OBJECTIVES: Interstitial lung disease is a life-threatening complication of many systemic autoimmune diseases with diverse clinical and histopathological features. Among them, lymphocytic interstitial pneumonia (LIP) is mainly associated with primary Sjögren's syndrome (pSS). A case of a middle-aged man with LIP, anti-Ro/La, anti-Scl70 autoantibodies and overlapping histopathological features of pSS and systemic sclerosis (SSc) is presented and discussed. METHODS: A 65-year-old man complaining for easy fatigue and dry cough was evaluated. Physical examination revealed bibasilar crackles on auscultation. Imaging tests showed areas of centrilobular nodules with tree-in-bud sign on the medial lobe of the right lung. Pulmonary function tests demonstrated small airways disease. Laboratory evaluation revealed elevated ESR and CRP, ANA titre >1/320, positive Ro52, Ro60 and La autoantibodies but also, weakly positive anti Scl70 autoantibody. RESULTS: Right lobe lung biopsy showed diffuse fibrosis with altered alveolar architecture and diffuse infiltration of alveolar septa by lymphocytes and mast cells. Ectopic germinal centres were disclosed, adjacent to the small bronchi causing lumen obstruction and validated after the demonstration of CD23 expression, specific for follicular dendritic cells. Biopsy of minor salivary glands revealed intense periductal fibrosis with limited round cell infiltrates, not fulfilling the histopathological criteria for pSS. The diagnosis of LIP was established and the patient received corticosteroids with poor response. Subsequently he was treated with rituximab with satisfactory results. CONCLUSIONS: This case with LIP and disease-specific autoantibodies for pSS and SSc teaches the complexity and overlapping nature of both diseases, extending from autoimmune epithelitis with ectopic germinal centres to fibrosis-related SSc. It points out the significance of the affected tissue biopsy, which may uncover the different disease phenotypes. To this end, treatment with anti-CD20, acting at the crossroads of the pathogenetic mechanisms of both diseases may serve as a first choice therapy.


Assuntos
Doenças Pulmonares Intersticiais , Síndrome de Sjogren , Idoso , Anticorpos Antinucleares , Autoanticorpos , Humanos , Doenças Pulmonares Intersticiais/etiologia , Masculino , Glândulas Salivares Menores , Síndrome de Sjogren/complicações
17.
Clin Exp Rheumatol ; 37 Suppl 118(3): 225-233, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31464667

RESUMO

OBJECTIVES: Sjögren's syndrome (SS) is an autoimmune disorder causing irreversible damage to the exocrine glands. Evidence whether SS patients are at a higher risk to develop periodontal disease is conflicting. Therefore, we systematically reviewed the literature on the prevalence of periodontal disease in patients with SS. METHODS: Searches were performed in MEDLINE and CENTRAL databases on prevalence of periodontal diseases in SS. Meta-analyses were performed for gingival index (GI), plaque index (PI), probing pocket depth (PPD), clinical attachment level (CAL), DMFT and DMFS (Decayed Missing Filled Teeth, respectively, Surfaces). RESULTS: Out of 512 studies, 10 studies were eligible for quantitative synthesis. Meta-analyses of the data indicated that in SS patients CAL, GI, PPD and PI are comparable to controls. DMFT and DMFS values were higher in SS patients than controls. CONCLUSIONS: No significant differences in the GI, PI, CAL, and PPD were observed in patients with SS compared to controls. These results indicate that there is no evidence of a higher risk for periodontal disease in patients with SS, while SS patients are more susceptible to caries compared to non-SS patients.


Assuntos
Doenças Periodontais , Síndrome de Sjogren , Índice de Placa Dentária , Humanos , Doenças Periodontais/etiologia , Índice Periodontal , Fatores de Risco , Síndrome de Sjogren/complicações
18.
Clin Exp Rheumatol ; 37 Suppl 118(3): 123-132, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31464673

RESUMO

OBJECTIVES: Overt renal disease in primary Sjögren's syndrome (pSS) manifests as interstitial nephritis and glomerulonephritis. This single centre study aims to describe the natural history and treatment outcome of renal disease in pSS. METHODS: pSS patients with renal disease were identified, and clinical features, renal biopsy findings, treatment details and renal outcome were recorded. RESULTS: Of the 20 pSS patients with renal disease, 14 had interstitial nephritis (IN), 3 had glomerulonephritis (GN) and 3 had both entities. In the IN group, 3 patients presented with chronic kidney disease (CKD), 4 with renal tubular acidosis (RTA), 2 with symptomatic hypokalaemia, 4 with renal colic and 1 with haematuria/proteinuria. Eight of 14 patients with IN received systemic immunosuppression (IS) during renal disease course and in 6 patients no beneficial effect was observed on renal function, hypokalaemia and RTA. Six of 14 IN patients developed CKD while 5 of them preserved normal renal function during follow-up. In the GN group, 2 patients presented with CKD, 3 with proteinuria/haematuria and 1 with nephrotic proteinuria. GN renal biopsy findings revealed membranoproliferative (MPGN) (n=3), focal segmental glomerulosclerosis (n=1) and fibrillary glomerulopathy (n=1). All 3 MPGN patients had cryoglobulinaemia and in 1 patient cryoglobulinaemic MPGN was clinically diagnosed. All GN patients were treated with immunosuppressive therapy, with stabilisation or improvement of renal function in the 4 cryoglobulinaemia-associated GN patients only. CONCLUSIONS: Interstitial nephritis follows a slow course and does not improve with systemic immunosuppression while GN has a favourable treatment response in those with MPGN pathology.


Assuntos
Glomerulonefrite , Nefrite Intersticial , Síndrome de Sjogren , Glomerulonefrite/etiologia , Glomerulonefrite/terapia , Humanos , Rim , Nefrite Intersticial/etiologia , Nefrite Intersticial/terapia , Síndrome de Sjogren/complicações , Resultado do Tratamento
19.
Clin Exp Rheumatol ; 37 Suppl 118(3): 192-198, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31464676

RESUMO

Neurologic involvement is a common extraglandular manifestation of primary Sjögren's syndrome (pSS), is varied and can be divided anatomically into 3 categories: central nervous system, peripheral neuropathies and autonomous nervous system manifestations. According to different study cohorts, neurological manifestations can occur in 18-45% of pSS patients, with the peripheral nervous system being the most frequent site of involvement compared to the central nervous system and autonomic system. Some neurologic complications share convergent pathophysiology, although the pathological basis of other conditions, namely cognitive impairment in pSS, is less clear. The heterogeneity of neurologic manifestations in pSS complicates the diagnosis and approach to treatment, which should be directed toward the underlying neuro-pathologic mechanism. The diagnosis and treatment of these manifestations must be optimised in order to avoid severe disability. However, for the majority of the complications, evidence for treatment efficacy is limited and requires further investigation.


Assuntos
Doenças Autoimunes do Sistema Nervoso/etiologia , Síndrome de Sjogren , Doenças Autoimunes do Sistema Nervoso/patologia , Doenças do Sistema Nervoso Central/etiologia , Humanos , Doenças do Sistema Nervoso Periférico/etiologia , Síndrome de Sjogren/complicações
20.
Clin Exp Rheumatol ; 37 Suppl 118(3): 133-139, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31464678

RESUMO

OBJECTIVES: The aim of the present study was to verify whether artificial neural networks (ANNs) might help to elucidate the mechanisms underlying the increased prevalence of cardiovascular events (CV) in primary Sjögren's syndrome (pSS). METHODS: 408 pSS patients (395 F: 13 M), with a mean age of 61 (±14) years and mean disease duration of 8.8 (±7.8) years were retrospectively included. CV risk factors and events were analysed and correlated with the other pSS clinical and serological manifestations by using both a traditional statistical approach (i.e. Agglomerative Hierarchical Clustering (AHC)) and Auto-CM, a data mining tool based on ANNs. RESULTS: Five percent of pSS patients experienced one or more CV events, including heart failure (8/408), transient ischaemic attack (6/408), stroke (4/408), angina (4/408), myocardial infarction (3/408) and peripheral obliterative arteriopathy (2/408). The AHC provided a dendrogram with at least three clusters that did not allow us to infer specific differential associations among variables (i.e. CV comorbidity and pSS manifestations). On the other hand, Auto-CM identified two different patterns of distributions in CV risk factors, pSS-related features, and CV events. The first pattern, centered on "non-ischaemic CV events/generic condition of HF", was characterised by the presence of traditional CV risk factors and by a closer link with pSS glandular features rather than to pSS extra-glandular manifestations. The second pattern included "ischaemic neurological, cardiac events and peripheral obliterative arteriopathy" and appeared to be strictly associated with extra-glandular disease activity and longer disease duration. CONCLUSIONS: This study represents the first application of ANNs to the analysis of factors contributing to CV events in pSS. When compared to AHC, ANNs had the advantage of better stratifying CV risk in pSS, opening new avenues for planning specific interventions to prevent long-term CV complications in pSS patients.


Assuntos
Doenças Cardiovasculares , Síndrome de Sjogren , Idoso , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Comorbidade , Diagnóstico por Computador/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Síndrome de Sjogren/complicações , Síndrome de Sjogren/epidemiologia
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