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Chronic recalcitrant erythema nodosum leprosum (ENL) or type 2 reaction (T2R) is a severe condition found in approximately 50% of multibacillary leprosy subjects. T2R is associated with important morbidities and may lead to several disabilities, not only due to nerve damage but also due to the prolonged use of corticosteroids, thalidomide, or immunosuppressors. We describe here four leprosy patients with chronic recalcitrant ENL treated with cyclophosphamide pulse therapy. All subjects had been on prednisone and thalidomide therapy for at least 30 months but showed inflammatory activity when doses were reduced. Pulse therapy with 1.0 g of cyclophosphamide was used every 4-6 weeks for a minimum of three applications. After pulse therapy, all cases presented total or partial regression of symptoms, and we were able to taper thalidomide and prednisone doses, with better control of ENL, avoiding further hospital admissions and disabilities. No side effects were observed during or after infusion therapy. Cyclophosphamide pulse therapy may be useful and safe to control chronic recalcitrant ENL.

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BACKGROUND: Dexamethasone-cyclophosphamide pulse (DCP) and dexamethasone pulse (DP) have been successfully used to treat pemphigus, but DCP/DP outcomes comparing pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are scarce. OBJECTIVE: To compare DCP/DP outcomes in a Brazilian cohort of PV and PF patients according to demographic and clinical data. METHODS: Retrospective analytical cohort study, reviewing medical charts of PV and PF patients (for DCP/DP Phases I‒IV consult Pasricha et al.16‒18). RESULTS: 37 PV and 41 PF patients non responsive to usual treatments were included similarly for DCP or DP therapy. Disease duration was longer among PF before DCP/DP prescription (p < 0.001); PF required a higher number of monthly pulses to acquire remission in Phase I (median 10 and 6 pulses, respectively; p = 0.005). DCP/DP outcomes were similar in both groups: remission in 37.8% of PV and 34.1% of PF after completed DCP/DP cycles following a median of 13 months (1-56 months follow-up); failure occurred in 13.5% of PV and 14.6% of PF in Phase I; relapse in 13.5% of PV and 12.2% of PF, and dropout in 27% of PV and 24.4% of PF in Phases II to IV. Mild side effects were documented. STUDY LIMITATIONS: The severity of PV and PF disease was not assessed by score indexes. CONCLUSIONS: PV and PF patients presented similar DCP/DP outcomes. DCP/DP should be initiated earlier in PF patients due to the longer duration of their disease in order to decrease the number of pulses and the duration of Phase I to acquire remission.

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Abstract Background Dexamethasone-cyclophosphamide pulse (DCP) and dexamethasone pulse (DP) have been successfully used to treat pemphigus, but DCP/DP outcomes comparing pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are scarce. Objective To compare DCP/DP outcomes in a Brazilian cohort of PV and PF patients according to demographic and clinical data. Methods Retrospective analytical cohort study, reviewing medical charts of PV and PF patients (for DCP/DP Phases I‒IV consult Pasricha et al.16‒18). Results 37 PV and 41 PF patients non responsive to usual treatments were included similarly for DCP or DP therapy. Disease duration was longer among PF before DCP/DP prescription (p < 0.001); PF required a higher number of monthly pulses to acquire remission in Phase I (median 10 and 6 pulses, respectively; p = 0.005). DCP/DP outcomes were similar in both groups: remission in 37.8% of PV and 34.1% of PF after completed DCP/DP cycles following a median of 13 months (1-56 months follow-up); failure occurred in 13.5% of PV and 14.6% of PF in Phase I; relapse in 13.5% of PV and 12.2% of PF, and dropout in 27% of PV and 24.4% of PF in Phases II to IV. Mild side effects were documented. Study limitations The severity of PV and PF disease was not assessed by score indexes. Conclusions PV and PF patients presented similar DCP/DP outcomes. DCP/DP should be initiated earlier in PF patients due to the longer duration of their disease in order to decrease the number of pulses and the duration of Phase I to acquire remission.

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ABSTRACT Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition of unknown etiology that can affect the eye. The most common ocular manifestation related to VKH is bilateral diffuse uveitis associated to exudative retinal detachment. Although these patients respond well to steroid pulse therapy, we report a case of a 44-year-old female patient presenting bilateral exudative retinal detachment and clinical diagnosis of VKH, who did not respond to the first cycle of 3-day pulse therapy with methylprednisolone. The exudation was reabsorbed only after a second cycle of steroid therapy.

RESUMO A doença de Vogt-Koyanagi-Harada é inflamatória e de etiologia desconhecida, podendo afetar o olho. A manifestação ocular mais comum relacionada à doença de Vogt-Koyanagi-Harada é a uveíte difusa bilateral associada ao descolamento exsudativo da retina. Embora esses pacientes respondam bem à pulsoterapia com esteroides, relatamos um caso de paciente de 44 anos que apresentou descolamento exsudativo bilateral da retina com diagnóstico clínico de doença de Vogt-Koyanagi-Harada que não respondeu ao primeiro ciclo de pulsoterapia de 3 dias com metilprednisolona. A exsudação apenas reabsorveu após uma segunda rodada de terapia com esteroides.

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SUMMARY The relationship between acute pancreatitis and the administration of glucocorticoids is unclear because most reported cases have been diagnosed with systemic vascular diseases, such as systemic lupus erythematosus, which may be responsible for pancreatitis. A 22-year-old woman with eye involvement of a newly diagnosed systemic lupus erythematosus was admitted to our hospital. Pulse intravenous methylprednisolone therapy was given at 1mg/kg day for 3 days, and oral prednisolone at 40 mg/day thereafter. During pulse steroid therapy, she had abdominal pain, back pain, distention, nausea, and vomiting. Her physical examination was compatible with acute abdomen and peritonitis. Abdomen Computerized Tomography scan revealed diffuse liquid perihepatic and perisplenic area with heterogeneity around the mesentery. Due to the symptoms of acute abdomen, explorative laparotomy was performed. There was diffuse free fluid in the abdomen and edematous changes were observed around the pancreas. Amylase and lipase from intraabdominal fluid were studied and found to be high. The postoperative prednol dose was reduced carefully. On the sixth postoperative day, the drain was removed, and the patient was discharged without any problem. Physicians should keep in mind that acute pancreatitis may also be a cause of differential diagnosis of newly developed abdominal pain in patients receiving pulse steroid therapy with a normal level of serum amylase and lipase.

RESUMO A relação entre pancreatite aguda e a administração de glicocorticoides é incerta pois a maioria dos casos relatados foram diagnosticados com doenças vasculares sistêmicas, como lúpus eritematoso sistêmico, que pode causar pancreatite. Uma paciente de 22 anos com envolvimento ocular e lúpus eritematoso sistêmico recém-diagnosticado foi admitida em nosso hospital. Pulsoterapia intravenosa com metilprednisolona 1mg/kg foi administrada por 3 dias. Depois disso, a paciente foi tratada com prednisolona oral 40 mg/dia. Durante a pulsoterapia com corticoides, a paciente apresentava dor abdominal, dor nas costas, distensão, náusea e vômitos. O exame físico era compatível com quadro de abdome agudo e peritonite. Tomografia computadorizada do abdome revelou líquido difuso na região perihepática e periesplênica, com heterogeneidade ao redor do mesentério. Devido aos sintomas de abdome agudo, foi realizada laparotomia exploradora. Havia líquido livre difuso no abdome e alterações edematosas foram observadas em torno do pâncreas. A amilase e lipase do líquido intra-abdominal foram analisadas e consideradas elevadas. A dose pós-operatória de prednol foi reduzida com cuidado. No sexto dia de pós-operatório, o dreno foi retirado, e a paciente recebeu alta sem qualquer problema. Médicos devem lembrar que a pancreatite aguda também pode ser uma causa de diagnóstico diferencial para dor abdominal recém-desenvolvida em pacientes recebendo pulsoterapia com corticoides e com níveis normais de amilase e lipase séricas.

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We present two cases of severe COVID-19 that were rejected by medical institutions. The management of the disease was done at home with methylprednisolone (MP) pulse therapy for three days. This resulted in a favorable evolution and resolution of most symptoms. COVID-19 infection presents as asymptomatic disease, non-severe symptomatic disease, and severe respiratory inflammatory disease. The first two forms are dependent on viral response and a "cytokine storm" is responsible for the progression into severe disease. Glucocorticoids (GC) reduce inflammation by different mechanism depending of their concentration. Pulses lead to overall apoptosis of immune cells. Studies using pulse MP as treatment for SARS-CoV-1 showed clinical improvement and decreased incidence of ARDS compared with patients who received low dose steroid treatment. Inhibition of excessive inflammation through timely administration of GC in the early stage of inflammatory cytokine storm effectively prevents the occurrence of ARDS.

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Pemphigus is a chronic potentially fatal autoimmune disorder that causes blisters and erosions of the skin and oral mucous membrane. most of the cases present oral manifestations as the first clinical sign along with dermal lesions. only 0.5 to 3.2 of cases are reported each year per 1,000,000 population with oral manifestations without dermal participation, and is at times difficult to diagnose. we report a case of oral pemphigus vulgaris in a 20 year old female patient without dermal manifestations treated with oral mini pulse therapy. pénfigo oral tratado con terapia minipulse. resumen: el pénfigo es un trastorno autoinmune crónico potencialmente fatal que causa ampollas y erosiones de la piel y la membrana mucosa oral. la mayoría de los casos presentan manifestaciones orales como el primer signo clínico junto con lesiones dérmicas. solo se reportan de 0.5 a 3.2 casos cada año por cada 1,000,000 de personas con manifestaciones orales sin afectación de la piel, y algunas veces es difícil de diagnosticar. presentamos un caso de pénfigo vulgar oral en un paciente de 20 años, sin manifestaciones cutáneas tratadas con mini terapia del pulso oral.

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Granulomatous polyangiitis is a systemic disease that may affect any organ, with a predilection for the upper respiratory tract, lungs and kidneys. This article aims to report a case of a patient with atypical nodular scleritis as the initial manifestation of granulomatous polyangiitis (Wegener), mimicking a case of tuberculosis. The patient presented ocular hyperemia and lower progressive visual acuity for 1.5 years, followed by eye pain for two months. The patient had subpleural nodules with soft tissue density, increased pulmonary lymph nodes and discrete bilateral pleural thickening, with negative alcohol-resistant acid bacilli (BAAR). The histological diagnosis revealed a granulomatous vasculitis suggestive of non-infectious vasculitis (granulomatous polyangiitis). Cyclophosphamide pulse therapy was initiated.

Poliangiite granulomatosa é uma doença sistêmica que afeta qualquer órgão, com predileção pelo trato respiratório superior, pulmões e rins. Este artigo tem como objetivo relatar um caso atípico de uma paciente com esclerite nodular como manifestação inicial da poliangiite granulomatosa (Wegener), mimetizando um quadro de tuberculose. A paciente apresentou hiperemia ocular e baixa acuidade visual progressiva por 1,5 anos, seguido por dor ocular por dois meses. A paciente possuía nódulos subpleurais com densidade de partes moles, linfonodomegalia em janela aorto-pulmonar e espessamento pleural bilateral discreto, negativo para bacilos álcool-ácido resistentes (BAAR). O diagnóstico histológico revelou uma vasculite granulomatosa sugestiva de vasculite não infecciosa (poliangiite granulomatosa). Foi iniciada pulsoterapia com ciclofosfamida.

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Known since the 19th century, neuromyelitis optica (NMO), or Devic's disease, is an idiopathic immune-mediated inflammatory demyelinating disease of the central nervous system selectively affecting the optic nerve and spinal cord. Commonly diagnosed in demyelinating diseases reference centers, we report an 18-year-old female patient who sought medical attention with a 3-month history of weight loss, headache, and vomiting, followed by diplopia, a burning sensation over the lower limbs, and difficulty walking. A few days prior to hospital admission, the muscle strength in her lower limbs became worse and ascended to the upper limbs associated with sensory changes in the trunk and voiding dysfunction. At admission, the neurological examination was consistent with a spinal cord syndrome. After few days of hospitalization, she was tetraplegic with severe signs of brainstem involvement requiring mechanical ventilatory support. Intravenous methylprednisolone and cyclophosphamide were promptly started after ruling out the diagnosis of infectious disease and cord compression. Due to no substantial early improvement, intravenous immunoglobulin was also used. From then on, the neurological status gradually improved. Magnetic resonance imaging showed extensive demyelinating features in the spinal cord, and the serum IgG autoantibody was negative. The patient was referred to a tertiary neurological reference center where she remains under treatment.

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Introdução: Foram analisados retrospectivamente dados de 26 pacientes com vitiligo em progressão, submetidos a protocolo de tratamento com minipulso oral de dexametasona. A resposta clínica e os efeitos colaterais foram avaliados em intervalos mensais. Dos 26 pacientes incluídos no protocolo de estudo, 12 (46,2%) apresentaram repigmentação das lesões, 11 (42,3%) estabilização, e três (11,5%) progressão da doença. Dez pacientes (38,5%) apresentaram efeitos colaterais, sendo o aumento de peso a reação adversa mais comum. Houve associação entre tempo de tratamento e frequência dos efeitos colaterais.

Introduction: The authors have carried out a retrospective analysis of data from 26 patients with developing vitiligo who were treated with oral mini-pulse of dexamethasone. Clinical response and side effects were evaluated at monthly intervals. Of the 26 patients included in the study, 12 (46.2%) presented a repigmentation of lesions, 11 (42.3%) stabilized, and 3 (11.5%) experienced a worsening of the condition. Ten patients (38.5%) had side effects, the most common of which was weight gain. There was a correlation between the duration of the treatment and the frequency of side effects.

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OBJECTIVE: Cytomegalovirus (CMV) systemic disease and myocarditis in healthy persons is infrequently reported in the literature, although in increasing numbers in recent years. The importance of the recognition of the syndrome that usually has an initial picture of a mononucleosis like infection in an otherwise healthy person, is the available therapeutic agent, ganciclovir, that can cure the infectious disease. METHODS: We analyzed the clinical result of pulsotherapy with steroids in a patient with CMV myocarditis after 7 days of etiological treatment, with ganciclovir, intravenous vasodilators, and the conventional treatment for congestive heart failure. RESULTS: The clinical condition of the patient improved accordingly to the better function of the left ventricle, and the ganciclovir was kept for 21 days, most of it in an out patient basis. The patient was dismissed from the hospital, with normal myocardial function. CONCLUSION: Potentially curable forms of myocarditis, like M pneumoniae and CMV, for example, can have an initial disproportionate aggression to the myocardium, by the acute inflammatory reaction, that can by itself make worse the damage to the LV function. In our opinion, the blockade of this process by pulsotherapy with steroids can help in the treatment of these patients. We understand that the different scenario of immunosuppressive treatments for the possible auto immunity of the more chronic forms of the presumably post viral cardiomyopathy has been in dispute in the literature, and has stolen the focus from the truly acute cases.

OBJETIVO: Doença sistêmica por citomegalovírus (CMV) com miocardite em pessoas saudáveis é raramente referida na literatura, apesar de em maior número em anos recentes. A importância do reconhecimento da síndrome, que usualmente tem um quadro inicial "mononucleosis like" em uma pessoa sadia é a disponibilidade do agente terapêutico ganciclovir, que pode curar a infecção. MÉTODOS: Nós analisamos o resultado da pulsoterapia com esteróides em um paciente com miocardite por CMV, após 7 dias de tratamento etiológico com ganciclovir, vasodilatadores intravenosos e o tratamento convencional para insuficiência cardíaca congestiva. RESULTADOS: A condição clínica do paciente melhorou com a melhor função do ventrículo esquerdo e o ganciclovir foi mantido por 21 dias após alta hospitalar.A função miocárdica retornou ao normal. CONCLUSÃO: Formas curáveis de miocardites como M pneumonia e CMV, por exemplo, podem ter uma agressão grave ao miocárdio por uma ação inflamatória que pode piorar a função cardíaca. Em nossa opinião, o bloqueio deste processo pela pulsoterapia com esteróides pode auxiliar no tratamento destes pacientes. Entendemos que existe um cenário diferente de tratamento com imunossupressores para possível agressão auto-imune das formas mais crônicas de cardiomiopatias dilatadas e isso está em disputa na literatura, talvez mudando o foco dos casos realmente agudos.

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Este trabalho é um relato de experiência do ensino de Enfermagem Clínica para alunos de graduação, no qual foi utilizada a metodologia de assistência de enfermagem como estratégia de ensino. Aplicada a pessoas adultas, portadoras de Lúpus Eritematoso Sistêmico (LES), em sua maioria mulheres jovens, submetidas a pulsoterapia, uma modalidade terapêutica que, por meio da imunossupressão, reduz o processo inflamatório causado pelos autoanticorpos. Desta forma, a assistência é sistematizada, individualizada e integral e permite ao acadêmico, oportunidades singulares para correlação teórico-prática, com a aplicação de conhecimentos básicos da área de formação, contextualização do processo saúde-doença e focalização ampliada para além do biológico, abrangendo aspectos emocionais, sociais e espirituais e oportunidade para o aluno avaliar as diversas nuanças que interferem nas condições de assistência oferecidas pelos serviços públicos de saúde.

This research is an experience report of clinical teaching for undergraduate nursing students, to whom it was used the assistance methodology as a teaching strategy. It was applied in adults, sufferingSystemic Erythematosus Lupus, young women, in majority, who were submitted to drug pulse therapy, a therapeutic modality which, through immunesuppression, reduces the inflammatory process caused by selfantibody. This way, the assistance is systemized, individualized and integral and it allows single opportunities of theoric-pratic correlation for the students, with the application of basic knowledge of the formation area, context of the health-disease process and the focus going beyond the biologic, including emotional, social and spiritual aspects and opportunities for the student to evaluate several factors that interfere in the assistance conditions offered by public health services.

Este és un relato de experimento de la enseñanza de Enfermería Clínica para alumnos de graduación, utilizando medología de asistencia de enfermería como habilidad medotológica para acompañar el paciente y enseñar. Empleada a la personas adultas, portadoras de Lúpus Eritematoso Sistémico (LES), en su mayoría mujeres jóvenes, sometidas a la pulsoterapia, una modalidad terapéutica que, por medio de la imunosupresión, reducido el proceso inflamatorio causante por los autoanticuerpos. De esta manera, la asistencia és de sistematización, particular y total, concede al académico, ocasiones raras para correlación teoríca-práctica, con la aplicación del conocimiento básico de la área de formación, contextualización del proceso salud-enfermedad y focalización ampliada para más adelante del biológico, abarcando aspectos emocionales, sociales y espirituales y ocasión para el alumno evaluar las diversas matizes que interponen en las condiciones de asisténcia ofrecidas por los servícios públicos de salud.

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PURPOSE--To use corticosteroids in a shorter period to treat rheumatic carditis, keeping the patient in the hospital; and verify the time interval of normalization of rheumatic activity tests with this method. METHODS--In 36 patients (40 episodes) intravenous methyl-prednisolone (1g/day) was administered. The number of series ranged from two to four, according to severity of the disease. The ages ranged from 6 to 17 years old, all of them fulfilled the criteria of Jones for diagnosis of rheumatic fever. They were submitted to treatment to eradicate the streptococcus, worms, PPD and dental focus extraction, before use of corticosteroids. RESULTS--In all patients the signals and symptoms of heart failure improved. In six cases occurred complications during pulse therapy that were easily controlled with clinical measures. Two series of methylprednisolone were used in 10 children, three in nine and four in 21 episodes. Eight patients were sent to valve replacement. The interval of time that laboratory tests of rheumatic activity became negative was 41.2 +/- 13.3 days. CONCLUSION--Using this IV corticotherapy it was possible decrease the amount of days of this medication, keeping the patient in the hospital. In this way we eliminate the problem of interruption of the treatment. There was not significative difference between oral and IV corticotherapy in order to the laboratory tests become negative.

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PURPOSE--To find out new subjects that could be useful to select patients between 9 and 12 points, according to Block's Criteria, to mitral balloon valvotomy. METHODS--One hundred and forty patients underwent mitral balloon valvotomy. Among them, 29 (21) had between 9 and 12 points. These patients were divided into two groups: group A-patients with mitral valve area > or = 1.5cm2, immediately after balloon valvotomy and in the follow-up period; group B-patients with mitral valve area < 1.5cm2 immediately after or during the follow-up period, patients with severe mitral regurgitation after the procedure and patients who died in the follow-up period. All patients were analyzed by echocardiographic relation criteria (ERC): calcification + subvalvar disease/thickness + mobility. Each one was quantified from 1 to 4 points according to the degree of valvular disease. RESULTS--Group A was composed of 17 (51) and group B 12 (41) patients. The variables age, sex, previous mitral commissurotomy and atrial fibrillation did not show difference between groups. In group A mitral valve area (cm2) increased from 1.15 +/- 0.25 to 1.97 +/- 0.26 (p < 0.00001) keeping stable during the follow-up period. In group B percutaneous mitral balloon valvotomy resulted in an increase from 1.26 +/- 0.19 to 1.77 +/- 0.16 (p < 0.00001), however, there was an important decrease in the follow-up period to 1.34 +/- 0.15 (p < 0.00001). The ERC showed that all group A patients had a relation < 1. However, in group B, 10 patients (83), the relation was > or = 1, and in only 2 patients (17) was < 1 (p < 0.00001). CONCLUSION--The group of patients between 9 and 12 points in the Block's criteria is heterogenic, therefore, each case might be evaluated individually and the echocardiographic relation criteria should be used in order to select these patients to the procedure.

Objetivo - Diminuir o prazo de uso de corticoterapia na cardite reumática, mantendo o paciente internado e avaliar o tempo para negativação das provas de atividade da doença. Métodos - Foram tratados 36 pacientes entre 6 e 17 anos (40 episódios) com metilprednisolona venosa (1g/dia) em séries de três dias semanais. O número de séries variou de duas a quatro, dependendo da gravidade. Todos os pacientes preenchiam os critérios de Jones para diagnóstico de febre reumática. Todos foram submetidos a erradicação estreptocócica, PPD, tratamento de verminoses e dos focos dentários antes da corticoterapia. Resultados - Todos os pacientes melhoraram da insuficiência cardíaca na época da alta. Em 6 casos ocorreram intercorrências durante a pulsoterapia, mas foram controladas rapidamente. Foram realizadas duas séries em 10 pacientes, três em 9 e quatro em 21 episódios. Oito foram encaminhados para troca de válvula. O tempo de negativação das provas de atividade reumática e da alta hospitalarioi 41,2±13,3 dias. Conclusão - O esquema utilizado encurtou o tempo total de uso dos corticosteróides, podendo assim manter o paciente internado e eliminando-se a interrupção do tratamento da cardite. Não ocorreu diferença significativa quanto ao prazo de negativação das provas de atividade reumática entre o esquema clássico oral e a pulsoterapia

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