ENT involvement and orobuccal movements' disorders in Pandas patients: assessment and rehabilitations tools.

OBJECTIVE: PANDAS are known as the spectrum of autoimmune pathologies related to a previous or current infection by group A beta-hemolytic streptococcus (SBEGA), dealing with several neuropsychiatric manifestations that mainly affect pediatric age. The main features consist of behavioral disease or movement disease characterized by acute-onset, presenting especially through infant period or adolescence. Specific manifestations, occurring during the progression of the disease, are the presence of otorhinolaryngologic symptoms (ENT) and orofacial movement disorders associated with temporomandibular joint pain. PATIENTS AND METHODS: We enrolled 130 children (5-15 years) with a clinical diagnosis of PANDAS between 2012 and 2018. Participants were assessed using ENT specific parameters, PSG to examine respiratory disorders and conventional audiological evaluation. Descriptive and comparative statistical analyses were performed with a control group of 51 healthy patients. RESULTS: The prevalence of ENT symptoms associated was significantly detected in 88 patients of 130 in Group A (relative frequency (%) 67.6; p=0.041) and in 51 patients of 130 in the control Group B (relative frequency (%) 39.2; p=0.063). In relation to prevalence of SDB, 54 subjects have presented nocturnal respiratory obstructive symptoms from mild to severe (relative frequency (%) 61.3; p=0.033) vs. 20 patients of Group B (relative frequency (%) 39.2; p=0.055). The obstructive severity average type was correlated to the consensual adenotonsillar development (size 3-4), (relative frequency (%) 45.4; p=0.047). The audiological deficits found were mostly of transmissive type with OME correlated and linked to the presence of occasional episodes of AOM. The four PANDAS patients who presented orobuccal dystonia (relative frequency (%) 4.54; p=0.091) achieved an improvement of the algic symptoms through the exercises of self-rehabilitation. CONCLUSIONS: Findings from our study show that respiratory diseases, characterizing a group of patients with pandas, are the direct consequences of the malformed or hypertrophic condition and suggesting in these conditions surgical therapy as an approaching tool.

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies.

BACKGROUND: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). MAIN BODY: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. CONCLUSIONS: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strength-building and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

Guidelines of the Brazilian Society of Rheumatology for the treatment of systemic autoimmune myopathies

Abstract Background: Recommendations of the Myopathy Committee of the Brazilian Society of Rheumatology for the management and therapy of systemic autoimmune myopathies (SAM). Main body: The review of the literature was done in the search for the Medline (PubMed), Embase and Cochrane databases including studies published until June 2018. The Prisma was used for the systematic review and the articles were evaluated according to the levels of Oxford evidence. Ten recommendations were developed addressing the management and therapy of systemic autoimmune myopathies. Conclusions: Robust data to guide the therapeutic process are scarce. Although not proven effective in controlled clinical trials, glucocorticoid represents first-line drugs in the treatment of SAM. Intravenous immunoglobulin is considered in induction for refractory cases of SAM or when immunosuppressive drugs are contra-indicated. Consideration should be given to the early introduction of immunosuppressive drugs. There is no specific period determined for the suspension of glucocorticoid and immunosuppressive drugs when individually evaluating patients with SAM. A key component for treatment in an early rehabilitation program is the inclusion of strengthbuilding and aerobic exercises, in addition to a rigorous evaluation of these activities for remission of disease and the education of the patient and his/her caregivers.

Physical exercise among patients with systemic autoimmune myopathies.

Systemic autoimmune myopathies (SAMs) are a heterogeneous group of rare systemic autoimmune diseases that primarily affect skeletal muscles. Patients with SAMs show progressive skeletal muscle weakness and consequent functional disabilities, low health quality, and sedentary lifestyles. In this context, exercise training emerges as a non-pharmacological therapy to improve muscle strength and function as well as the clinical aspects of these diseases. Because many have feared that physical exercise exacerbates inflammation and consequently worsens the clinical manifestations of SAMs, it is necessary to evaluate the possible benefits and safety of exercise training among these patients. The present study systematically reviews the evidence associated with physical training among patients with SAMs.

Revisión de las miopatías inflamatorias a propósito de 3 casos en unidades de recuperación funcional de hospitales de media estancia / Review of inflamatory myopathies based on 3 cases in functional recovery units in long-term care hospitals

Las miopatías inflamatorias idiopáticas son un grupo heterogéneo de enfermedades sistémicas de etiopatogenia autoinmune. Se caracterizan por debilidad muscular, alteraciones miopáticas en el electromiograma y elevación de las enzimas musculares. Pueden darse de forma aislada, o asociarse a otras enfermedades autoinmunes o a neoplasias. Realizamos una aproximación a estos procesos a propósito de 3 pacientes tratados en Unidades de Recuperación Funcional de 2 hospitales de media estancia. En todos se produce debilidad muscular bilateral y simétrica de predominio en cinturas, con grave repercusión funcional. Por este motivo precisan realizar tratamiento en régimen de ingreso hospitalario. El objetivo terapéutico es doble: por un lado, frenar de forma urgente la actividad autoinmune para evitar el daño muscular progresivo y, por el otro, instaurar un tratamiento rehabilitador de forma precoz que minimice el deterioro funcional. En todos los casos se obtiene beneficio de la intervención, ya que permite la vuelta al domicilio con distintos grados de independencia (AU)

Idiopathic inflammatory myopathies are a heterogeneous group of systemic autoimmune diseases characterised by bilateral and symmetric muscle weakness, myopathic alterations on electromyography and elevated muscle enzymes. They can be associated with other autoimmune diseases or neoplastic processes. We provide a review of these processes based on 3 patients in the Functional Recovery Units of 2 long-term care hospitals. All 3 patients had bilateral and symmetrical muscle weakness, predominantly in the waist, with severe functional impairment. Consequently, these patients required hospital admission. The goal of rehabilitation is twofold: on the one hand, to urgently halt the autoimmune activity in order to avoid further muscle damage, and, on the other hand, to start early rehabilitation treatment to minimise functional impairment. All 3 patients benefited from the intervention, allowing discharge to home, with varying degrees of independence (AU)

Recién nacidos de madre con enfermedad autoinmunitaria. Experiencia en un hospital comarcal / Newborns whose mother has autoimmune disease. A community hospitals’ experience

Las madres con enfermedad autoinmunitaria (EAI) pueden presentar exacerbaciones de su enfermedad durante la gestación y el puerperio, con implicaciones fetales y neonatales. El objetivo del presente estudio fue describir las incidencias de estas madres y la afección neonatal asociada. Se realizó un análisis retrospectivo entre los años 2004 a 2010, controlándose 29 madres con EAI. Se registraron 52 embarazos, 39 RN vivos y 13 abortos. Durante la gestación se produjeron 10 complicaciones: una vasculitis digital, una pancreatitis, una glomerulonefritis, una diabetes gestacional, 2 amenazas de parto prematuro, 3 preeclampsias y 1 eclampsia. En el posparto, una exacerbación lúpica. Entre los RN 20,5% presentaron bajo peso y 4 transferencia de anticuerpos maternos con un lupus neonatal (LNN). Posteriormente, 8 niños (20,5%) desarrollaron asma, uno oligoartritis ANA negativa y otro púrpura trombocitopénica autoinmunitaria. En nuestro hospital la tasa de abortos y prematuridad es similar a la descrita en la literatura. Destaca la presencia de un caso de LNN con paso transplacentario de anti-Sm(AU)

Mothers with autoimmune diseases (AID) may have exacerbations of their disease during pregnancy and postpartum period, with fetal implications and neonatal complications. The aim of this study was to describe miscarriages during pregnancy and postpartum problems among mothers with AID and associated neonatal pathology. Retrospective data was recorded from 2004 to 2010. 29 mothers with AID were analyzed, 65% of whom had lupus erythematosus (SLE). There were 52 pregnancies, which resulted in 39 newborns. There were 10 instances of maternal complications (25.6%) during the pregnancies, including 1 with digital vasculitis, 1 with pancreatitis, 1 outbreak of glomerulonephritis, 1 case of gestational diabetes, 2 patients at risk for preterm birth, 3 with preeclampsia and 1 with eclampsia. During the postpartum period, there was one case of SLE exacerbation. Among the newborns 20.5% had low birth weight and 4 exhibited the transplacental passage of maternal antibodies with one case of neonatal lupus. Among complications beyond the neonatal period, 8 (20.5%) children developed asthma, one presented negative ANA oligoarthritis and another presented immune thrombocytopenic purpura. In our hospital, the rates of miscarriage, prematurity and LBW among the newborns of mothers with AID are similar to those reported in the literature. The observation of a case of NL with the transplacental passage of anti-Sm is remarkable(AU)

Autoimmune diseases and rehabilitation.

This review gives an overview of the rehabilitation of autoimmune diseases. After general remarks on rehabilitation, the effects of acute and chronic exercises on inflammatory markers are summarized. Most of the available literature deals with rheumatoid arthritis (RA) and multiple sclerosis (MS), and therefore, rehabilitation of these diseases is described in more detail. Exercise is the main component in the rehabilitation of patients with RA and aims at increasing physical capacity, muscle strength, aerobic endurance, cardiovascular fitness and functional abilities, and helps to prevent secondary deconditioning due to reduced activity levels. Since MS causes a wide range of symptoms, the rehabilitation of these patients requires a multidisciplinary approach and encompasses physiotherapy, exercise therapy, hippotherapy, cognitive rehabilitation, psychological therapy, strategies to improve fatigue and coping programs. The ultimate goal of rehabilitation is to enable patients with chronic conditions to reach and maintain their optimal physical, sensory, intellectual, psychological and social functional levels, and to attain independence and self-determination as far as possible.

[Special training therapy to reduce inflammation in Anti-Jo-1 syndrome]. / Spezielle Trainingstherapie zur Reduktion der Inflammation bei Anti-Jo-1-Syndrom.

A 46-year-old patient was frequently seen with a medically treated Anti-Jo-1 syndrome. The patient had already been treated with azathioprine and oral corticosteroids on account of decreasing lung function, dyspnoea, fatigue, and beginning signs of myositis. Although high doses of steroids and azathioprine were administered, the muscleskeletal syndromes increased steadily. The patient used to be an active long-distance runner (20 km), but now was unable to perform that kind of physical exercise. It was decided to start a treatment with the GalileoTM training device for active muscle training of the lower extremities. Before and after three months of training the following assessment was performed: measurement of health-related quality of life (St. Georges respiratory questionnaire, SGRQ), ultrasound measurement of the cross-sectional area of the quadriceps muscle, 6 minute walk test (6 MWT), lung function testing, and assessment of serum markers of inflammation (TNF-alpha, interleukin-8, CRP, CK, myoglobin). After only two months, training with the GalileoTM five times a week has improved the patient's conditions dramatically. The training will be continued.

Patient perceptions about illness self-management in ANCA-associated small vessel vasculitis.

OBJECTIVES: To characterize patient perceptions, related to eight self-management behaviours relevant for adults with ANCA-associated small vessel vasculitis (ANCA-SVV), and to determine if these perceptions were associated with performance of each behaviour. METHODS: Adults with ANCA-SVV (n = 202) completed a self-administered questionnaire that assessed eight self-management behaviours (adherence to recommendations for medication, health service use, diet, exercise, infection avoidance and symptom monitoring; prompt reporting of symptoms and side effects; and adjusting activities in response to symptoms), perceptions about these behaviours, socio-demographics, clinical factors and social desirability bias. Descriptive statistics were generated to characterize patients' perceptions about difficulty of, importance of, and specific barriers to performing each behaviour. Regression analyses explored whether these variables were associated with performing each behaviour, controlling for potential confounders. RESULTS: With few exceptions, higher perceived importance and lower perceived difficulty of each behaviour were associated with more frequent performance of the behaviour. For each behaviour, several specific barriers were frequently endorsed by patients and a number of these were associated with lower levels of self-management. CONCLUSION: This study reveals that patient perceptions about the illness and its treatment influence ANCA-SVV self-management. Perceived barriers to medication, health services, diet and exercise adherence were similar to those in other illnesses. This study also provides insight into barriers experienced by patients in performing behaviours (infection avoidance, symptom monitoring, reporting symptoms and side-effects and adjusting activities) not often previously studied. How the identification of these barriers can help inform future interventions for ANCA-SVV patients is to be discussed.

Findings of interest from immunology and psychoneuroimmunology.

The biopsychosocial paradigm is now the main model when dealing with most human health disorders. One of the strengths of this model is that it encourages broader thinking when assessing and managing patients. It also encourages broader reading into areas not traditionally associated with manual therapy. Immunology and neuroscience are amongst the fastest growing medical sciences. These fields come together in the relatively new area of psychoneuroimmunolgy. This article examines some findings from these fields that are not widely discussed in the physical therapy professions. These findings are of relevance to many of the disciplines within the physical therapies. It is the authors aim to stimulate further interest in the relevant, yet often under explored areas of immunology and psychoneuroimmunology.

[Internet application for medical purposes in chronic patients cared in a systemic autoimmune diseases unit]. / Utilización de Internet como fuente de información por los pacientes de una consulta de Enfermedades Autoinmunes Sistémicas.

OBJECTIVE: In recent years Internet has become an important tool for medical information search, not only for physicians but also for the patients. Little is known about Internet patient's use in Spain. Our objective was to study the frequency with which patients with different chronic autoimmune diseases cared in our unit use Internet as a source of medical information, and to identify the factors associated with its use. METHODS: A survey was carried out on Internet use to patients cared in a teaching hospital Systemic Autoimmune Diseases unit along three consecutive weeks. RESULTS: Hundred thirty-eight patients completed the questionnaire. Eighty-seven (63%) were women. Fifty-four patients (39.1%) were older than 55 years. Mean disease progression was 34.2 +/- 39.2 months. 22% of the respondents were uneducated, 39% had elementary education level; 20% had average education level, and 20% had advanced education level. Forty-two patients (30.4%) used regularly Internet for any purpose. Factors associated to Internet use for medical purposes were regular Internet use (OR: 4.6; CI: 1.1-18.8;p = 0.03), and average or advanced educational degree (advanced education: OR: 22.9; CI: 2.6-202.7; p = 0.005; average education: OR: 8.5; CI: 1.2-59.6; p = 0.03). Eighty-one patients (58.7%) expressed their need of information on quality web pages. CONCLUSIONS: Many patients use Internet for medical purposes in our environment and even more consider to use it in the future. It is important to be aware of this fact and facilitate information about quality web pages for interested patients.

Impairment of lung function, health status and functional capacity in patients with ANCA-associated vasculitis.

OBJECTIVE: To determine the effects of lung involvement on respiratory function in patients with ANCA-associated vasculitis and the relation to impaired health status. METHODS: Thirty patients with ANCA-associated vasculitis in remission (15 with lung involvement at diagnosis as determined by an abnormal chest X-ray) were examined. We measured lung function, skeletal muscle strength [quadriceps force (QF), respiratory muscle strength (Pi(max))], exercise capacity (VO(2) peak) using treadmill exercise tests, and health status using the Short Form 36 and St George's respiratory questionnaires. RESULTS: Exercise capacity was reduced compared with predicted values (58.2%, range 23-123%) and 18 patients showed functional aerobic impairment. Respiratory muscle function was reduced (72.1% predicted, range 20-108%) and was not related to lung involvement or steroid usage. Transfer factor correlated significantly with exercise capacity, suggesting inadequate delivery of oxygen to muscles. Nine patients had reduced transfer factor (seven with lung involvement). Patients with lung involvement had impaired gas transfer compared with those without lung involvement (96.9 +/- 6 vs 113.3 +/- 4.7% predicted, P = 0.04). However, there were significant abnormalities in other lung function parameters not related to previous lung involvement (eight patients had reduced forced expiratory volume in 1 s, and five patients had reduced residual volume). Twelve patients (five with previous lung involvement) had obstructive airways disease. Physical health status was impaired to a greater degree than mental health status across the whole group and was not related to lung involvement or original disease severity, but correlated with transfer factor. CONCLUSION: Patients with ANCA-associated disease may have significant lung function impairment irrespective of lung involvement at the time of diagnosis. Patients showed reduced respiratory muscle strength, health status and exercise capacity, which correlated with reduced transfer factor.

[Obliterated cochlea in Cogan's syndrome -- implications for cochlear implant surgery]. / Obliteration der Cochlea beim Cogan-Syndrom -- Bedeutung für die Cochlear-Implant-Chirurgie.

BACKGROUND: Acute, often bilateral deafness in Cogan's syndrome or other autoimmune diseases is caused by autoimmune mediated inflammatory attack on the membranous labyrinth. Auditory rehabilitation in case of bilateral deafness can be achieved by cochlear implant surgery. METHODS: A retrospective analysis of all patients suffering from Cogan's syndrome that had received a cochlear implant, was carried out. RESULTS: 6 of 295 adult patients (2.6 %) that had received a cochlear implant, had become deaf due to Cogan's syndrome. Partial obliteration or ossifikation was encountered in all cases and influenced surgical procedure. In one case a fibrous obliteration of the scala tympani was found 8 weeks after acute onset of complete deafness. CONCLUSIONS: The course of obliteration is unknown. With regard to our results a fibrous obliteration may occur as early as 8 weeks after complete deafness. This has to be considered in counseling of patients. Only early cochlear implant surgery facilitates best possible rehabilitation results.

[Scientifically based patient education exemplified by patients with primary systemic vasculitis. The vasculitis patient education program--development, contents and initial results of evaluation]. / Wissenschaftlich begründete Patientenschulung am Beispiel der Patienten mit primär systemischen Vaskulitiden. Das Vaskulitis-Schulungsprogramm--Entwicklung, Inhalte und erste Ergebnisse der Evaluation.

Referring to the literature, it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Most programs are based on cognitive behavioral interventions. Within the last few years, an interdisciplinary approach for providing information on disease, therapies, side effects, coping strategies, nutrition and physiotherapy has been developed for patients with primary systemic vasculitides (PSV) in the vasculitis center of the Medical University of Lübeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. New slides and handouts for patients were developed. To evaluate the new form of the program, a documentation system consisting of patient and physician-administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters was designed. Patients are trained in closed groups (n = 10-15) and asked to complete questionnaires at baseline, 4 weeks and 6 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

[Whole-body cryotherapy in rehabilitation of patients with rheumatoid diseases--pilot study]. / Die Bedeutung der Ganzkörperkältetherapie im Rahmen der Rehabilitation bei Patienten mit rheumatischen Erkrankungen.--Ergebnisse einer Pilotstudie.

UNLABELLED: Cryotherapy as a whole-body cold therapy (with cold air cooled by addition of nitrogen blown on the patients in an open cabin) for treatment of inflammatory rheumatic diseases already started in Bad Säckingen in 1986. In 1996, a new cold chamber (this time a closed chamber without any addition of nitrogen) based on compressor technology was introduced. The aim of our study was to test whether significant pain relief could be achieved by means of this cold therapy. Furthermore, we were interested in the practicability and acceptance of this new technique. Wellbeing during the treatment application and pain level were assessed using verbal and numerical rating scales. The sample consisted of 120 consecutive patients (75% women, age: 30-67 yrs, M = 52.6 yrs). These patients were suffering from primary fibromyalgia (40.7%), rheumatoid arthritis (17.3%), chronic low back pain (16.4%), ankylosing spondylitis (10.9%), osteoarthritis (9.1%), secondary fibromyalgia (3.6%) and other autoimmune diseases (1.8%) (mean duration of symptoms: 4 yrs). The patients were treated 2.5 minutes on average in the main chamber (mean temperature: -105 degrees C). The patients' statements concerning their pain level were analyzed by means of analyses of variance with repeated measures and paired-sample t-tests. RESULTS: The pain level after application of the cold therapy decreases significantly. The pain reduction lasts about 90 minutes. The initial pain level decreases during the whole time of treatment, no significant improvement, though, can be shown from the middle to the end of the four-weeks treatment. According to the results of our study, there is evidence that the whole-body cold therapy generates important short-term effects and somewhat weaker effects over the treatment period as a whole. Short-term pain reduction facilitates intensive application of physiotherapy and Occupational Therapy. The treatment procedure is practicable, and all in all well tolerated. From the patients' point of view, whole-body cold therapy is an essential part of the rehabilitation programme.