A clinical analysis of 13 patients with scleroderma renal crisis and pulmonary arterial hypertension / 中华风湿病学杂志

Objective:To describe a series of systemic sclerosis (SSc) patients with the combination of scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).Methods:The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results:Thirteen patients suffered from SRC and PAH in the SSc patients, 1 case was limited cutaneous SSc, and 12 cases were diffuse cutaneous SSc. Five patients had renal crisis before pulmonary arterial hypertension, 4 patients had pulmonary arterial hypertension before the occurrence of renal crisis, and the remaining 4 patients were found at the same time. Among them, 11 patients had Raynaud's phenomenon, 7 had gastrointestinal bleeding, 6 had pulmonary edema and 3 had telangiectasias. Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies. N-terminal pro-brain natriuretic peptide (NT-proBNP)>1 400 ng/L in 11 patients. Two patients had thrombotic microangiopathy (TMA). Among the 13 patients, 3 patients died during hospitalization, 2 patients were lost to follow-up, and 2 patients died within 5 years of follow-up. Six patients survived, and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion:In patients with scleroderma, SRC can occur earlier, later than, or at the same time with SSc-PAH. Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP. PDE5i or ERAs may be beneficial.

Skin sclerosis, hypertension and renal insufficiency: a report of one case with literture review / 中华风湿病学杂志

Objective:To improve the differential diagnosis of systemic sclerosis (SSc) with hypertension and renal insufficiency.Methods:The clinical characteristics, diagnosis and treatment of a SSc patient with hypertension and renal insufficiency were reported and discussed.Results:A middle-aged female patient with a history of SSc for 5 years, headache and gross hematuria for 10 days was admitted. Abrupt increase in blood pressure and creatinine, glomerular hematuria, proteinuria, low complement C3 and C4, positive antinuclear antibody (ANA), anti-dsDNA antibody and perinuclear antineutrophil cytoplasmic antibody (pANCA) were presented. Renal pathology showed lupus nephritis (LN) (type Ⅳ). After glucocorticoid pulse therapy, followed by cyclophosphamide, belimumab, and symptomatic treatment, the symptoms were relievedand lupus disease activity were decreased.Conclusion:For SSc patients with increased blood pressure and creatinine, the presence of other diseases should be considered in addition to scleroderma renal crisis. Renal biopsy and pathological examination should be performed to confirm the diagnosis and avoid misdiagnosis.

Renal involvement in scleroderma / Alteraciones renales en la esclerodermia

ABSTRACT Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by vasculopathy, inflammation, collagen deposits and fibrosis in the skin and internal organs. Renal complications are common in SSc. The spectrum of renal complications in systemic sclerosis includes scleroderma renal crisis (SRC), abnormal renal function, proteinuria, renal chronic vascular disease, kidney injury from nephrotoxic drugs, oxalate calcium nephropathy, glomerulonephritis and vasculitis associated to anti-neutrophil cytoplasmic antibody. Renal involvement remains an important cause of morbidity and mortality in scleroderma. The objective of this review is to describe the renal complications of scleroderma.

RESUMEN La esclerosis sistémica es una enfermedad autoinmune generalizada que se caracteriza por vasculopatía, inflamación, depósito de colágeno y fibrosis en la piel y órganos internos. Las complicaciones renales son comunes y con un amplio espectro e incluyen crisis renal esclerodérmica, función renal anormal, proteinuria, enfermedad vascular renal crónica, daño renal por fármacos nefrotóxicos, nefropatía por oxalato de calcio, glomerulonefritis y vasculitis asociadas a anticuerpos contra el citoplasma de los neutrófilos. La alteración renal permanece siendo una causa importante de morbilidad y mortalidad en la esclerodermia. El objetivo de esta revisión narrativa es describir las complicaciones renales de esta enfermedad.

Pharmacological treatment of scleroderma renal crisis: A systematic literature review / Tratamiento farmacológico de la crisis renal en esclerosis sistémica: una revisión sistemática de la literatura

ABSTRACT Background: Scleroderma renal crisis is a condition that affects approximately 4-6% of patients with systemic sclerosis, especially with diffuse compromise. Clinical manifestations are variable, representing a diagnostic challenge. Objective: The study aims to describe and analyze the different pharmacological treatments available for the management of scleroderma renal crisis. Materials and methods: A systematic literature review was done based on observational studies and clinical trials about the treatment of scleroderma renal crisis, using monotherapy or combined therapy. The studies were identified using electronic scientific databases, including MEDLINE PUBMED and EMBASE, in English, published between January 1990 and August 2019. Results: Eleven studies were included (ten observational studies and one open clinical trial). Of them, seven were cohorts, one case series, and two case-control studies. Overall, 1113 patients were included in the analyzed studies. All studies used angiotensin-converting enzyme inhibitors as exposition, case definition, and/or comparison in the clinical trial. Regarding the need for dialysis, approximately 53.9% of patients required it temporarily or permanently. Approximately 6-27% of patients required temporal dialysis, and 19-78% required permanent dialysis. One-year survival range was between 64 and 84%; two-year survival was between 53 and 74%; five-year survival between 40 and 90%, and finally ten-year survival between 35 and 47%. Conclusions: Angiotensin-converting enzyme inhibitors continue to be the first line of treatment for scleroderma renal crisis by contributing to a decrease in short-term mortality. However, alternative therapeutic options are required as a high percentage of patients still require dialysis. Future clinical trials are necessary to assess the effectiveness and safety of different therapeutic options.

RESUMEN Introducción: La crisis renal es una condición que afecta aproximadamente a 4-6% de los pacientes con esclerosis sistémica, especialmente con compromiso difuso. Las manifestaciones clínicas son variables, representando un reto diagnóstico en la práctica clínica. Objetivo: El objetivo del estudio fue describir y analizar los diferentes tratamientos farmacológicos disponibles para el manejo de la crisis renal en esclerosis sistémica. Materiales y métodos: Una revisión sistemática de la literatura fue desarrollada con base en estudios observacionales y ensayos clínicos sobre el tratamiento de la crisis renal, utilizando monoterapia o terapias combinadas. Los estudios fueron identificados utilizando bases de datos científicas que incluyeron MEDLINE PUBMED y EMBASE, que estuvieran en inglés y publicados entre enero de 1990 y agosto de 2019. Resultados: Once estudios fueron incluidos (10 estudios observacionales y un ensayo clínico abierto). De estos, siete fueron cohortes, una serie de casos y dos estudios de casos y controles. En total, 1113 pacientes fueron incluidos en los estudios analizados. Todos los estudios utilizaron inhibidores de enzima convertidora de angiotensina como exposición, definición de caso y/o comparador en ensayo clínico. Sobre la necesidad de diálisis, aproximadamente 53,9% de los pacientes la requirieron de forma temporal o permanente. Aproximadamente 6-27% de pacientes requirieron diálisis temporal y 19-78% requirieron diálisis permanente. El rango de sobrevida al año fue de 64-84%; a dos arios 53-74%; a cinco arios 40-90%, y a diez arnos 35-47%. Conclusiones: Los inhibidores de enzima convertidora de angiotensina continúan siendo la primera línea de tratamiento de crisis renal en esclerosis sistémica, al contribuir en la reducción de la mortalidad a corto plazo. Sin embargo, opciones terapéuticas alternativas son requeridas, al continuar muy elevado el porcentaje de requerimiento de diálisis. Ensayos clínicos futuros son necesarios para evaluar la eficacia y seguridad de diferentes opciones terapéuticas.

Eculizumab como opción terapéutica en paciente con crisis renal esclerodérmica asociada a síndrome hemolítico urémico / Eculizumab as a therapeutic option in patients with scleroderma renal crisis associated with haemolytic uraemic syndrome

RESUMEN La crisis renal esclerodérmica (CRS) es una manifestación rara de la esclerosis sistémica (ES). Se presenta como hipertensión arterial de nuevo inicio, empeoramiento o aceleración de la hipertensión arterial crónica o rápido deterioro de la función renal, frecuentemente acompañada de signos de hemolisis microangiopática. Su relación con el síndrome hemo lítico urémico es infrecuente, existiendo tan solo un caso similar reportado en la literatura. Presentamos el caso de una mujer de 36 arios en tratamiento para ES con cefalea global, pulsátil e intensa, convulsión tónico-clónica, cifras de presión arterial altas, falla renal aguda y hemólisis no autoinmune persistente. La evaluación de ADAMTS13 mostró un 60,6% de actividad. El estudio genético para búsqueda de mutaciones predisponentes para sín drome hemolítico urémico atípico (SHUa) reveló variante homocigota en el gen ADAMTS13, c.3287G>A (p.Arg1096His). Se inició tratamiento con eculizumab, observándose en poco tiempo mejoría de la hemólisis, función renal y estado clínico, con algunos efectos benéficos notorios e inesperados sobre la ES.

ABSTRACT Scleroderma renal crisis (SRC) is a rare manifestation of systemic sclerosis (SSc), presented as hypertension of new onset, worsening and / or acceleration of chronic hypertension, or rapid deterioration of renal function, often accompanied by signs of microangiopathic haemolysis. It is rarely associated with haemolytic uraemic syndrome, and there is only one similar case reported in the literature. The case is presented here of a 36-year-old woman on treatment for SSc with global, pulsatile and intense headache, clonic tonic convulsions, high blood pressure levels, acute renal failure, and persistent non-autoimmune haemoly sis. The evaluation of ADAMTS13 showed 60.6% of activity. The genetic study to search for mutations predisposing to atypical haemolytic uraemic syndrome (aHUS) revealed a homozygous variant in ADAMTS13 gene, c.3287G>A (p.Arg1096His). Eculizumab was star ted, with an improvement being observed in a short time in the haemolysis, renal function, and clinical status, with some notable and unexpected beneficial effects on SSc.

Scleroderma renal crisis in a kidney transplant patient / Crisis renal de esclerodermia en una paciente trasplantada renal

Abstract Renal crisis is a complication with significant morbidity and mortality in scleroderma patients and a rare entity in kidney transplantation. It may present with highly variable clinical manifestations, mainly arterial hypertension, acute kidney failure and multisystemic involvement. Following is the report of a patient with late reoccurrence of scleroderma crisis in kidney transplantation, who was given successful treatment with angiotensin-converting enzyme inhibitors.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1215).

Resumen La crisis renal es una complicación con importante morbilidad y mortalidad en pacientes con esclerodermia y una rara entidad en trasplante renal, que se puede presentar con manifestaciones clínicas muy variables, principalmente hipertensión arterial, falla renal aguda y compromiso multisistémico. A continuación se reporta el caso de una paciente con recurrencia tardía de la crisis esclerodérmica en el trasplante renal, que recibió tratamiento exitoso con inhibidores de la enzima convertidora de angiotensina.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1215).

Esclerosis sistémica sin esclerodermia, un desafío diagnóstico para el clínico: caso clínico y revisión de la literatura / Systemic sclerosis sine scleroderma: a clinical diagnostic challenge: clinical case and literature review

La Esclerodermia es una patología poco frecuente que afecta principalmente la piel, de ahí su nombre. Desde ese punto de vista existen diferentes clasificaciones siendo las más frecuentes: forma Difusa y Limitada. Existe también otra forma de presenta-ción, muy infrecuente y difícil de identificar: la esclerosis sistémica sin esclerodermia (eSSe). En este reporte presentamos el caso de una paciente de 50 años, que cursa con injuria renal aguda de origen inexplicado con algunos elementos de laboratorio de microangiopatía trombótica, sin clínica sugerente de esclerosis sistémica progre-siva y con laboratorio inicial difícil, lo que retrasó el diagnóstico y por ende el trata-miento. En este caso se obtiene el diagnóstico de crisis renal esclerodérmica, por los hallazgos compatibles de microangiopatía trombótica arrojados por la biopsia renal.

Systemic sclerosis is a rare connective tissue disease that is characterized by thickening of the skin (scleroderma). The disease has 2 main presentations forms: diffuse and limited. However, it can also manifest without scleroderma, known as systemic sclerosis sine scleroderma (ssSSc). In this report, we describe the case of a 50 years female who debut with acute renal injury and possible microangiopathy thrombotic elements in laboratory findings, in absence of signs of scleroderma at physical examination or initial laboratory test results, causing the delay of accurate diagnosis and management. Renal biopsy findings were compatible with thrombotic microangiopathy and renal sclerodermic crisis, which guided the diagnosis and subsequent management.

A retrospective clinical analysis of 16 patients with scleroderma renal crisis / 中华内科杂志

Objective To analyze the clinical features,laboratory tests,treatments and outcome of patients with scleroderma renal crisis (SRC).Methods We retrospectively reviewed the clinical and laboratory data of 16 patients with scleroderma renal crisis in Peking Union Medical College Hospital from May 2004 to May 2013.The treatment and outcome of SRC patients were also retrospectively analyzed.Results There were a total of 16 SRC patients including 5 male patients and 11 females.The median age at SRC onset was (49.9 ± 12.3) years.It usually took 3.2 years from the diagnosis of systemic sclerosis(SSc) to SRC attack.Ten SRC patients belonged to diffuse cutaneous systemic sclerosis (dcSSc),and 6 patients were limited cutaneous systemic sclerosis (lcSSc).Among SRC patients,16/16 were negative of anticentromere antibodies(ACAs).All these 16 patients had hypertension and renal insufficiency,including 8 dialysis dependent after the onset of SRC and 7 with thrombotic microangiopathy.There were 3 patients receiving renal biopsy.The pathological findings were mainly summarized as intimal thickening and stenosis of renal arterioles.Among 13 patients with long-term followed-up,11 patients received angiotensin converting enzyme inhibitors(ACEI),5 patients died,2 patients were dialysis dependent.Only 1 patient stopped dialysis after the combination treatment of ACEI and endothelin receptor antagonist.Another 5 patients didn't need dialysis.Conclusion SRC usually occurred at the early course of SSc.dcSSc was more frequent than lcSSc.ACAs were rarely found in SRC patients.The immediate and sufficient use of ACEIs was still the cornerstone of SRC treatment.Future studies are needed to evaluate the efficacy of endothelin receptor antagonist in the treatment of SRC.

Treatment for juvenile systemic sclerosis of children / 中国小儿急救医学

Juvenile systemic sclerosis (SSc) is a chronic connective tissue disease characterized by symmetrical thickening and hardening of the skin,associated with fibrous changes in internal organs.In the last years,several therapies have showed promising beneficial effects in the treatment of SSc.We summarized the recent advances in the anti-inflammation and immunosuppressive therapy,treatment of SSc related vascular diseases,anti-fibrosis therapy as well as prospective treatments.

A Case of Scleroderma Renal Crisis without Malignant Hypertension after Steroid Treatment / 대한신장학회잡지

Scleroderma renal crisis is defined as rapidly progressive renal failure and/or new onset of malignant hypertension during the course of systemic sclerosis. Most patients show clinical features of malignant hypertension, but there have been several reports of normotensive renal crisis. We have experienced a 63 year old female patients with acute renal failure due to scleroderma renal crisis who did not show the clinical features of malignant hypertension. She had taken steroid for the treatment of degenerative osteoarthritis and gradually developed shortness of breath and edema. Her blood pressure on admission was 150/90 mmHg and easily controlled by diuretics. Renal biopsy showed onion-skin appearance in the interlobular arteries with varying degree of tubulointerstitial changes. Her renal function rapidly deteriorated despite ACE inhibitor therapy and cytotoxic therapy had to be initiated because of progressive interstitial pneumonitis and myocarditis. We describe a patient with scleroderma renal crisis who did not show the clinical features of malignant hypertension following steroid treatment.

A case of "scleroderma renal crisis" developed from diffuse scleroderma / 대한내과학회지

Polymyositis is an inflammatory, autoimmune disease of the skeletal muscle characterized by symmetrical, proximal muscle weakness, elevated muscle enzymes, and characteristic features on electromyogram and muscle biopsy. The kidneys are generally spared and myoglobinuric renal failure is very rare in polymyositis. There have been infrequent reports of polymyositis developing myoglobinuric renal failure secondary to rhabdomyolysis. The flare-up may occur in polymyositis, usually manifest within several weeks to months of achieving a remission. But, rhabdomyolysis and myoglobinuric renal failure was a very rare feature of the relapse of polymyositis. We present a case report of patient with polymyositis who initially presented and relapsed as rhabodomyolysis that lead to myoglobinuric, oliguric renal failure and required transient dialytic support.

A Case of Scleroderma Renal Crisis / 대한신장학회잡지

Systemic sclerosis is a generalized connective tissue disorder. It is characterized by fibrosis, degenerative changes and vascular lesions affecting the skin, joints, skeletal muscles and multiple internal organs. Clinical renal disease in systemic sclerosis ranges from scleroderma renal crisis to more subtle abnormalities. Scleroderma renal crisis is characterized by malignant hypertension and rapidly progressive renal failure. It can be successfully treated with angiotensin converting enzyme inhibitors, but remains the most feared complication of systemic sclerosis. We have experienced a 57 year old female patient who visited emergency room with a sudden onset of mental deterioration, azotemia and oliguria. She was under symptomatic treatment for Raynaud's phenomenon for 2 years. Renal biopsy of the patient showed onion-skin configuration in the interlobular arteries which is characteristic of scleroderma renal crisis. She proceded to maintenance hemodialysis despite a angiotensin converting enzyme inhibitor treatment. We report a case of SRC with a brief review of literature.