Diagnosis and immunosuppressive treatment of inflammatory cardiomyopathy: a case report

2020 ◽  
pp. 1-5
Author(s):  
L. Naesens ◽  
M. Penicka ◽  
W. Heggermont
Keyword(s):  
Case Report ◽  
Immunosuppressive Treatment ◽  
Inflammatory Cardiomyopathy

Apresentação e Evolução Atípicas da Granulomatose de Wegener: Relato de Caso/Atypical Presentation and Evolution of Wegener's Granulomatosis: Case Report

1970 ◽  
Vol 5 (3) ◽  
pp. 53-67
Author(s):  
Aline Dos Santos ◽  
Ana Caroline Balducci Scafi ◽  
Luciene Azevedo Morais ◽  
Pablo Girardelli Mendonça Mesquita
Keyword(s):  
Renal Failure ◽  
Renal Function ◽  
Case Report ◽  
Chronic Renal Failure ◽  
Early Diagnosis ◽  
Wegener’S Granulomatosis ◽  
Immunosuppressive Treatment ◽  
Clinical Manifestations ◽  
Wegener's Granulomatosis ◽  
Creatinine Concentration

RESUMOIntrodução: A Granulomatose de Wegener (GW) é uma vasculite rara e idiopática associada à presença do anticorpo Anticitoplasma de Neutrófilo (ANCA) que acomete, preferencialmente, os pequenos vasos. As manifestações clínicas são diversas, ocorrendo em mais de 90% dos casos, sintomas do trato respiratório. O comprometimento renal é tardio e preditor de mau prognóstico.  Sua morbidade a médio e longo prazo inclui insuficiência renal crônica. A probabilidade de sucesso de manutenção da função renal depende da concentração sérica de creatinina ao início do tratamento, o que indica a importância do diagnóstico e terapêutica adequada precoces. Casuística: Relata-se o caso de uma paciente do sexo feminino, 61 anos, portadora de GW com comprometimento renal avançado à apresentação não precedido por sintomas pulmonares esperados. O tratamento imunossupressor associado a plasmaferese permitiu a melhora da função renal da paciente poupando-a de tornar-se dialítica- dependente. Discussão: A paciente iniciou a doença através de insuficiência renal assintomática, com valores de função renal compatíveis com o estágio mais avançado de doença renal crônica, ultrassonografia dos rins sem alterações compatíveis e sem os sintomas respiratórios esperados. Segundo a literatura, a combinação de imunossupressores e plasmaferese associa-se à recuperação renal em três meses com sobrevivência sem necessidade de diálise por 12 meses, no caso relatado, obteve-se tal resultado em 22 dias sem a necessidade de diálise após um ano. Conclusão: Devido ao diagnóstico precoce, o tratamento adequado foi instalado rapidamente proporcionando à paciente um aumento da expectativa e da qualidade de vida, evitando dependência de terapia renal substitutiva.Palavras-Chave: Granulomatose de Wegener, Plasmaferese, Doença renal crônica.  ABSTRACTIntroduction: The Wegener's Granulomatosis (WG) is a rare and idiopathic vasculitis associated with the presence of Antineutrophil Cytoplasmic Antibody (ANCA), that affects, preferentially, the small vessels. The clinical manifestations are diverse, occurring in over 90% of cases, symptoms in the respiratory tract. Kidney damage is a late and bad prognostic predictor. Morbidity in the medium and long term includes chronic renal failure. The probability of renal function maintenance success depends on serum creatinine concentration at the beginning of treatment that indicates the importance of early diagnosis and deployment of an appropriate therapy. Case Report: We present a case of a 61-year-old female patient, carrier of GW with advanced renal impairment presentation, not preceded by expected pulmonary symptoms. The immunosuppressive treatment associated with plasmapheresis allowed the improvement of the patient’s renal function, saving her from becoming dialysis-dependent Discussion: The patient developed the disease through asymptomatic renal failure, renal function with values that are compatible with the most advanced stage of chronic kidney disease, ultrasound of the kidneys without compatible changes and without the expected respiratory symptoms. According to the literature, the combination of immunosuppressive drugs and plasmapheresis is associated with renal recovery in three months with survival without dialysis for 12 months. In this case, a result was obtained in 22 days without the need for dialysis after one year. Conclusion: Due to the early diagnosis, appropriate treatment was quickly installed giving the patient increased life expectancy and quality, preventing dependence on renal replacement therapy.Keywords: Wegener’s granulomatosis, Plasmapheresis, Chronic renal failure.

scholarly journals Sipuleucel‐T associated inflammatory cardiomyopathy: a case report and observations from a large pharmacovigilance database

2021 ◽  
Author(s):  
Melissa Y.Y. Moey ◽  
Rahim A. Jiwani ◽  
Kotaro Takeda ◽  
Karyn Prenshaw ◽  
R. Wayne Kreeger ◽  
...  
Keyword(s):  
Case Report ◽  
Inflammatory Cardiomyopathy ◽  
Pharmacovigilance Database

scholarly journals Disseminated Nocardia infection with a lesion occupying the intracranial space complicated with coma: a case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Mei-Hong Yu ◽  
Xiao-Xin Wu ◽  
Chun-Lei Chen ◽  
Song-Jia Tang ◽  
Jian-Di Jin ◽  
...  
Keyword(s):  
Case Report ◽  
Immunosuppressive Treatment ◽  
Lung Infection ◽  
C Reactive Protein ◽  
Altered Consciousness ◽  
Blood Cell Count ◽  
Case Presentation ◽  
Reactive Protein ◽  
Nocardia Cyriacigeorgica ◽  
The Right

Abstract Background Disseminated Nocardia infection is a disease that is easily overlooked in patients with lesions occupying the intracranial space complicated with coma. Early diagnosis and treatment are crucial. Case presentation A 65-year-old man was admitted to the First Affiliated Hospital of Zhejiang University in October 2018 with weakness in the right limbs for 3 days and altered consciousness for 1 day. Five months earlier, he had been diagnosed with membranous kidney disease and had received cyclophosphamide and prednisone. At admission, the white blood cell count was 1.37 × 1010/L (with 86.4% neutrophils), and C-reactive protein was 115.60 mg/L. Imaging examinations revealed a lesion occupying the intracranial space, lung infection, and multiple abscesses in the rhomboid muscle. The abscesses were drained. Pus culture confirmed Nocardia cyriacigeorgica infection. With antibiotics and vacuum-sealed drainage of the back wound, the patient improved and was discharged from the hospital. Conclusions This case report shows that infection should be considered during the differential diagnosis of lesions in the intracranial space, especially in patients receiving immunosuppressive treatment. In patients with disseminated N. cyriacigeorgica infection, combination antibiotic therapy and surgical drainage of localised abscesses can be effective.

scholarly journals Visceral leishmaniasis in a patient with ulcerative colitis: A case report

2020 ◽  
Vol 77 (4) ◽  
pp. 435-439 ◽  
Author(s):  
Goran Jankovic ◽  
Lena Martinovic ◽  
Zorica Dakic ◽  
Dragana Mijac ◽  
Milos Stulic ◽  
...  
Keyword(s):  
Ulcerative Colitis ◽  
Case Report ◽  
Visceral Leishmaniasis ◽  
Female Patient ◽  
Liver Toxicity ◽  
Immunosuppressive Treatment ◽  
Laboratory Data ◽  
High Grade Fever ◽  
Travel History ◽  
Mediterranean Countries

Introduction. There is a rise of visceral leishmaniasis in immunocompromised patients due to increased availability of immunomodulatory drugs. In order to point at the occurrence of visceral leishmaniasis in patients with inflammatory bowel disease (IBD), we reported a case of female patient with a travel history to European Mediterranean countries, who was on immunosuppressive treatment due to ulcerative colitis. Case report. A 29-year-old female patient was admitted to hospital due to severe relapse of ulcerative colitis. Corticosteroid therapy was administered in addition to previous longterm azathioprine, with clinical response to the treatment. During the course of the disease she had recurrent high-grade fever with marked hepatosplenomegaly and pancytopenia. The diagnosis of leishmaniasis was established by positive serology tests and microscopic finding of amastigotes in bone marrow smears. The disseminated infection was responsive to treatment with liposomal amphotericin B, but therapy had to be discontinued due to urticarial rush. Subsequent therapy with antimony was administered, but it had to be stopped too due to liver toxicity. No further treatment for leishmaniasis was initiated as the clinical and laboratory data suggested that the patient had responded to the treatment. She was discharged from hospital in IBD remission and without signs of the infection. Conclusion. Visceral leishmaniasis should be considered in IBD patients with fever of unknown origin and relevant travel history in order to achieve favorable disease outcome.

scholarly journals O30 Case report of Cogan syndrome in a child with audiovestibular dysfunction and ocular inflammation

2021 ◽  
Vol 5 (Supplement_1) ◽  
Author(s):  
Samy Zakout ◽  
Shwan Mohamad
Keyword(s):  
Case Report ◽  
Immunosuppressive Treatment ◽  
Disease Onset ◽  
Ocular Inflammation ◽  
Nuclear Antigen ◽  
Treatment Modalities ◽  
Cogan Syndrome ◽  
Interstitial Keratitis ◽  
Balance Problems ◽  
Prompt Response

Abstract Case report - Introduction Cogan syndrome is a very rare disorder of probable autoimmune vasculitic origin, first described in 1934. The age of onset seems to range from 3 to 50 years with an average age of disease onset at 29 years. It is characterised by audiovestibular dysfunction and ocular inflammation. The cause remains unknown and the epidemiology of the disease is purely based on case reporting. As of 2015, there were fewer than 250 case reports. Interestingly, it seems extremely rare in Arabic and Middle Eastern countries in which a new case has been recently diagnosed and is being reported in this abstract. Case report - Case description A 14-year-old girl of Arabic origin first presented to the ENT specialist with a 3-week history of balance problems, left tinnitus and sensorineural hearing loss in both ears worse on the left. She first received treatment for vestibular neuritis with no improvement. Two months later, she complained of bilateral red eyes and excessive lacrimation when diffuse interstitial keratitis was diagnosed by the ophthalmologist. An array of blood tests including a full autoimmune disease was arranged and a rheumatology opinion was sought. The patient had a normal inflammatory response and her screen for antinuclear antibodies, extractable nuclear antigen, rheumatoid factor and antineutrophil cytoplasmic antibodies were all negative. A full virology screen was also negative. Having failed to respond to all supportive treatment modalities, a trial of a tapering 2-week course of prednisolone starting at 25mg daily was instigated. The patient’s symptoms improved by more than 50% on glucocorticoids subsequent to which the diagnosis of Cogan syndrome was anticipated. As the patient’s symptoms relapsed when glucocorticoids were stopped, she was recommenced on prednisolone 25mg in line with azathioprine 100mg daily. The patient’s hearing, which was almost lost in the left ear, improved significantly by more than 70% initially, and then to normal hearing as stated by the patient supported by special ENT testing. Her balance problems and vertigo also improved remarkably. Her tinnitus, which was the last symptom to resolve, almost went away over the subsequent 2 months. The patient had already come off glucocorticoids 6 months down the line, and stayed on azathioprine 75-100mg daily for almost a year after which gradual weaning was trialled and then eventually succeeded. She has now been off azathioprine for 6 months without experiencing any disease relapse. Case report - Discussion Cogan syndrome can be difficult to diagnose particularly in children. The diagnosis is essentially clinical depending on the presence of audio-vestibular symptoms and interstitial keratitis along with a prompt response to immunosuppressive medication. In this patient, the consistent clinical picture supported by the positive response to glucocorticoids especially in the absence of an alternative diagnosis would have made the diagnosis of Cogan syndrome likely. Given the rarity of this disorder, there are no classification criteria as yet hence the ongoing need for case reporting. Moreover, there is no clear guidance on the duration of immunosuppressive treatment of this condition. Case report - Key learning points It is pivotal in managing conditions with a suspected autoimmune origin to have a multidisciplinary approach involving other relevant specialties in a holistic approach. The prompt response to glucocorticoid therapy still remains the key in securing the diagnosis of a number of conditions in clinical practice especially in the absence of diagnostic criteria and/ or supportive investigations such as serological and tissue-based testing. Case reporting has a crucial role in enhancing awareness of rare medical conditions.

scholarly journals Necrotizing Anterior Scleritis in a Woman with Terrien’s Marginal Degeneration: A Case Report

2021 ◽  
Vol 15 (1) ◽  
pp. 318-321
Author(s):  
María Alejandra Fonseca-Mora ◽  
Paula Tatiana Muñoz-Vargas ◽  
Juliana Reyes-Guanes ◽  
William Rojas-Carabali ◽  
Miguel Cuevas ◽  
...  
Keyword(s):  
Case Report ◽  
Systemic Disease ◽  
Immunosuppressive Treatment ◽  
Disease Association ◽  
Slit Lamp ◽  
Systemic Corticosteroids ◽  
First Case ◽  
Anterior Scleritis ◽  
Inflammatory Component

Purpose: The aim of the study was to report the first case of a patient with Terrien’s Marginal Degeneration (TMD) who developed necrotizing anterior scleritis without systemic disease association, requiring systemic immunosuppressive treatment. Case Report: A 32-year-old female consulted for bilateral ocular burning and hyperemia. Initially, she was diagnosed with conjunctivitis and treated with topical antibiotics and corticosteroids, with mild transitory improvement but the progression of the disease. Years later, she attended the ocular immunology consultation for a second opinion where TMD with ocular inflammatory component OU was diagnosed. Seven months later, she presented with severe pain, decreased visual acuity, and photophobia in OS. At the slit-lamp examination, necrotizing anterior scleritis with a high risk of perforation in OS was observed. The patient was referred to the rheumatologist and started treatment with systemic corticosteroids and cyclophosphamide, exhibiting a clinical improvement. The patient did not meet the criteria for any systemic illness associated with scleritis, such as autoimmune diseases or vasculitis. Thus, scleritis was related to the adjacent inflammatory process associated with TMD, as an atypical presentation of this disease. Conclusion: Although an inflammatory type of TMD has been proposed, it is essential to follow up closely these patients and consider necrotizing anterior scleritis, a severe ocular disease that requires prompt immunosuppressive management, as a possible atypical associated presentation of this disease.

Gastrointestinal vasculitis due to systemic lupus erythematosus treated with rituximab: a case report

Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 640-643
Author(s):  
Gökçe Kenar ◽  
Kadri Atay ◽  
Gül Emek Yüksek ◽  
Burak Öz ◽  
Süleyman Serdar Koca
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Abdominal Pain ◽  
Case Report ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Immunosuppressive Treatment ◽  
Rapid Progression ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus

Background Patients with systemic lupus erythematosus may present with variable gastrointestinal manifestations including peritonitis, pancreatitis, enteritis, and vasculitis. Gastrointestinal vasculitis is one of the most devastating complications of systemic lupus erythematosus, with a mortality rate of 50% when it progresses to bowel ischemia and is complicated by hemorrhage or perforation. Case report A young female patient known to have systemic lupus erythematosus, lupus nephritis, and antiphospholipid antibody syndrome, on immunosuppressive treatment presented to the emergency department with acute abdominal pain and nausea. The clinical findings were first associated with an acute flare of lupus nephritis according to the assessments with active laboratory parameters. However, over a short time the abdominal pain worsened and was accompanied by peritonitis and gastrointestinal hemorrhage. The computed tomography scans demonstrated a dilated bowel and thickening of the bowel compatible with gastrointestinal vasculitis. The upper and lower gastrointestinal endoscopy supported the diagnosis of vasculitis by showing multiple ulcerative lesions along the gastrointestinal tract. The patient was successfully treated with pulse corticosteroids urgently, with a fast response to subsequent rituximab therapy without any relapses. Treatment with cyclophosphamide was not preferred because the patient had a high cumulative dose. Conclusion Gastrointestinal vasculitis should be a primary differential diagnosis in patients with systemic lupus erythematosus presenting with abdominal pain because of its rapid progression and high mortality. The treatment choice has been suggested as cyclophosphamide for severe cases in the literature. In this case report, a patient successfully treated with rituximab without any relapses was documented.

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