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1.
Medicina (Kaunas) ; 56(7)2020 Jul 17.
Artigo em Inglês | MEDLINE | ID: mdl-32708858

RESUMO

The evolving pandemic of Coronavirus Disease 2019 has posed a substantial health risk worldwide. However, there is a paucity of data regarding the clinical course and the therapeutic management of patients with chronic kidney disease and COVID-19 infection. To date, most evidence has come from renal transplantation, with about 45 patients reported thus far, and the current data from the ERA-EDTA (ERACODA) registry for transplanted patients and patients on Renal Replacement Therapy (RRT); as for those with glomerular diseases, data are lacking. Herein, we report the case of a 62-year-old patient with severe membranoproliferative glomerulonephritis who had been receiving a high burden of immunosuppression until four months before the COVID-19 infection. He developed severe disease with acute respiratory failure requiring mechanical ventilation. After treatment with hydroxychloroquine and azithromycin, despite his low chances, he gradually recovered and survived. To the best of our knowledge, this is one of the few reported patients with glomerulonephritis who had COVID-19 Besides our single case with glomerulonephritis early during the disease outbreak, the very low prevalence of COVID-19 infection in the country's transplant recipients (0.038%) and dialysis patients (0.24%) reflects the impact of the rapid implementation of social distancing rules as well as of preventive measures for disease control in the hospitals and dialysis units in our country.


Assuntos
Infecções por Coronavirus/complicações , Crioglobulinemia/complicações , Glomerulonefrite Membranoproliferativa/complicações , Pneumonia Viral/complicações , Insuficiência Respiratória/etiologia , Antibacterianos/uso terapêutico , Azitromicina/uso terapêutico , Betacoronavirus , Ceftriaxona/uso terapêutico , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/imunologia , Infecções por Coronavirus/terapia , Creatinina/metabolismo , Crioglobulinemia/imunologia , Ciclofosfamida , Inibidores Enzimáticos/uso terapêutico , Glomerulonefrite Membranoproliferativa/tratamento farmacológico , Glomerulonefrite Membranoproliferativa/imunologia , Glomerulonefrite Membranoproliferativa/metabolismo , Glucocorticoides/uso terapêutico , Grécia , Humanos , Hidroxicloroquina/uso terapêutico , Hospedeiro Imunocomprometido , Fatores Imunológicos/uso terapêutico , Falência Renal Crônica/terapia , Transplante de Rim , Leucemia Linfocítica Crônica de Células B/complicações , Leucemia Linfocítica Crônica de Células B/imunologia , Pulmão/diagnóstico por imagem , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Pandemias , Pneumonia Viral/diagnóstico , Pneumonia Viral/imunologia , Pneumonia Viral/terapia , Diálise Renal , Respiração Artificial , Insuficiência Respiratória/terapia , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Rituximab/uso terapêutico , Tomografia Computadorizada por Raios X
2.
J Card Surg ; 35(8): 2050-2052, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32652608

RESUMO

Acute pulmonary embolism (APE) is a well-described complication following surgical procedures. The incidence of such a complication can be related to the presence of a peculiar patient's condition. Cryoglobulinemia, which consists in the presence of one or more immunoglobulins in the serum that precipitate at temperatures below 37°C and redissolve on warming, seems to increase the risk of thrombotic events. Treatment options of APE, according to clinical severity, include systemic thrombolysis, surgical embolectomy, and systemic anticoagulation. Thrombolysis is considered the first-line treatment, whereas surgery is reserved in case of extremely-compromised hemodynamic conditions related to massive central embolism, and in case of contraindication to thrombolysis. Here, we report a case of acute massive pulmonary embolism occurring at the end of a surgical procedure for a thymic carcinoma resection, in a patient with cryoglobulinemia, which required an emergent surgical pulmonary embolectomy.


Assuntos
Crioglobulinemia/complicações , Embolectomia/métodos , Posicionamento do Paciente/efeitos adversos , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/cirurgia , Embolia Pulmonar/etiologia , Embolia Pulmonar/cirurgia , Timoma/cirurgia , Neoplasias do Timo/cirurgia , Doença Aguda , Ecocardiografia Transesofagiana , Emergências , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico por imagem , Embolia Pulmonar/diagnóstico por imagem , Índice de Gravidade de Doença , Toracotomia , Tomografia Computadorizada por Raios X
3.
Reumatol. clín. (Barc.) ; 16(3): 242-244, mayo-jun. 2020. ilus
Artigo em Espanhol | IBECS | ID: ibc-194359

RESUMO

Se presenta el caso de un paciente de 47 años con antecedente de artritis psoriásica (AP) de 9 años de evolución en quien se encuentra compromiso renal, hipocomplementemia, neuropatía periférica, lesiones necróticas acrales y crioglobulinas positivas. Luego de realizar el abordaje diagnóstico se concluye que el cuadro clínico corresponde a una vasculitis crioglobulinémica concomitante a la AP. Se expone además del caso una revisión de la literatura referente a la presencia de estas 2 enfermedades en un solo paciente


We report the case of a 47-year-old man with a 9-year history of psoriatic arthritis (PsA) in whom we detected renal involvement, hypocomplementemia, peripheral neuropathy, acral necrotic lesions and positive cryoglobulins. The results of the diagnosis led us to conclude that the clinical picture corresponded to cryoglobulinemic vasculitis concomitant with PsA. In addition, we present a review of the literature on the presence of these two diseases in a single patient


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Vasculite/etiologia , Crioglobulinemia/complicações , Artrite Psoriásica/etiologia , Vasculite/complicações , Vasculite/diagnóstico , Crioglobulinemia/diagnóstico , Psoríase/complicações , Psoríase/diagnóstico , Cotovelo/patologia , Braço/patologia , Metotrexato/uso terapêutico , Glucocorticoides/uso terapêutico , Rituximab/uso terapêutico
6.
BMJ Case Rep ; 12(11)2019 Nov 28.
Artigo em Inglês | MEDLINE | ID: mdl-31780611

RESUMO

Cryoglobulins are abnormal serum immunoglobulins that tend to precipitate in intravascular compartments at temperatures lower than 37°C causing blood flow restriction to vital organs. They are divided into type I, II and III based on the immunoglobulin subtypes of the cryoprecipitates. Type II cryoglobulinemia is most commonly associated with viral infections, autoimmune diseases and lymphoproliferative disorders. Here, we reported an 80-year-old man who presented with fatigue, acute kidney injury, palpable purpura, anaemia and altered mental status. He was diagnosed with type II cryoglobulinemia with concomitant positive autoimmune markers, varicella IgM antibody and IgM hepatitis B core antibody. The patient responded well to intravenous and oral steroid treatment.


Assuntos
Doenças Autoimunes/complicações , Varicela/complicações , Crioglobulinemia/complicações , Hepatite B/complicações , Idoso de 80 Anos ou mais , Doenças Autoimunes/sangue , Biomarcadores/sangue , Varicela/sangue , Crioglobulinemia/sangue , Crioglobulinemia/classificação , Hepatite B/sangue , Humanos , Masculino
7.
Int J Hematol ; 110(6): 751-755, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31494832

RESUMO

Chronic lymphocytic leukemia (CLL) can trigger autoimmune phenomena, with immune thrombocytopenia (ITP) the most common presentation. Upon cessation of CLL therapy, including ibrutinib, autoimmune flares can occur. In a 68-year-old man with CLL, ibrutinib was held for 2 weeks prior to elective shoulder surgery. Eleven days after stopping therapy, he presented with a purpuric rash on his right hip, buttock, and lower extremities. He experienced two episodes of seizure activity while hospitalized. MRI brain demonstrated patchy areas of altered signal involving deep white matter and sub-cortical white matter structures concerning for cerebral vasculitis. Although there was no evidence of hemolysis, serum cold agglutinin titer was elevated at > 1:512 and cryoglobulin levels were positive at 36%. He was diagnosed with type I cryoglobulinemia and treated with rituximab, plasmapheresis, methylprednisolone, and ibrutinib was restarted. This regimen resolved his symptoms. A rare complication of CLL is the production of cryoglobulins, which can present at initial diagnosis or in relapsed disease. Our case demonstrates that the cessation of ibrutinib therapy, even for a short time, can precipitate complications. To our knowledge, we report the first case of a patient with well-controlled CLL who rapidly developed cryoglobulinemic vasculitis after stopping ibrutinib therapy.


Assuntos
Crioglobulinemia/complicações , Leucemia Linfocítica Crônica de Células B/tratamento farmacológico , Pirazóis/uso terapêutico , Pirimidinas/uso terapêutico , Vasculite/etiologia , Suspensão de Tratamento , Idoso , Crioglobulinemia/diagnóstico , Crioglobulinemia/etiologia , Humanos , Leucemia Linfocítica Crônica de Células B/complicações , Masculino , Plasmaferese , Prednisolona/uso terapêutico , Pirazóis/efeitos adversos , Pirimidinas/efeitos adversos , Rituximab/uso terapêutico
8.
Medicine (Baltimore) ; 98(31): e16571, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31374024

RESUMO

RATIONALE: IgG4-related disease (IgG4-RD) is a systemic autoimmune disease and mixed cryoglobulinemia may be caused by autoimmune diseases. However, so far only 1 case of IgG4-RD complicated with mixed cryoglobulinemia is reported. Our case further confirms the close relationship between these 2 diseases. PATIENT CONCERNS: A 55-year-old female was admitted because of dry mouth and teeth falling off. DIAGNOSES: The patient was diagnosed as IgG4-related sialadenitis (IgG4-RS) complicated with type III mixed cryoglobulinemia. IgG4-RS was confirmed by elevated serum IgG4 levels and diffuse IgG4 plasmocyte infiltration and storiform fibrosis in the interstitium of labial gland. Type III mixed cryoglobulinemia was confirmed by positive serum cryoglobulins and no monoclonal immunoglobulin in serum and urine. INTERVENTIONS AND OUTCOMES: After treatment with prednisone and cyclophosphamide, serum cryoglobulins rapidly turned negative with the remission of IgG4-RS. LESSONS: Type III mixed cryoglobulinemia can be caused by IgG4-RS, and the underlying mechanisms need to be further explored.


Assuntos
Crioglobulinemia/complicações , Doença Relacionada a Imunoglobulina G4/complicações , Sialadenite/complicações , Crioglobulinemia/tratamento farmacológico , Feminino , Humanos , Doença Relacionada a Imunoglobulina G4/tratamento farmacológico , Imunossupressores/uso terapêutico , Pessoa de Meia-Idade , Sialadenite/tratamento farmacológico
9.
Medicine (Baltimore) ; 98(28): e16382, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31305439

RESUMO

RATIONALE: Type I monoclonal cryoglobulinemia is usually associated with lymphoproliferative disorders, such as monoclonal gammopathy of undetermined significance (MGUS), myeloma, chronic lymphocytic leukemia (CLL) and lymphoplasmocytic lymphoma (LPL). Clinical symptoms related to Type I cryoglobulin (CG) isotype often include skin, neurological and renal manifestations. PATIENT CONCERNS: A 42-year-old woman who initially presented urticaria, palpable purpura in both her upper extremities and legs, eosinophilia and Raynaud phenomenon. Skin biopsy revealed eosinophil infiltration. Monoclonal immunoglobulin (Ig) G-κprotein was detected and CG was also positive. DIAGNOSES: The patient was finally diagnosed as MGUS related Type I CG. INTERVENTIONS: Cyclophosphamide-dexamethasone-thalidomide (CDT) therapy was initiated. OUTCOMES: The treatment relieved the skin symptoms efficiently. LESSONS: To our knowledge, this is a rare case of Type I cryoglobulinemic vasulitis with eosinophilia complicated by MGUS, and the effective treatment of cyclophosphamide combined with thalidomide and prednisone may provide a new therapeutic option for cryoglobulinemic vasulitis.


Assuntos
Crioglobulinemia/complicações , Eosinofilia/complicações , Gamopatia Monoclonal de Significância Indeterminada/complicações , Vasculite/complicações , Adulto , Crioglobulinemia/diagnóstico , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Eosinofilia/diagnóstico , Eosinofilia/tratamento farmacológico , Feminino , Humanos , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/tratamento farmacológico , Vasculite/diagnóstico , Vasculite/tratamento farmacológico
11.
Clin Rheumatol ; 38(12): 3677-3687, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31172367

RESUMO

Newer treatment protocols involving direct-acting antiviral agents (DAAs) have been associated with high rates of sustained virologic response (SVR) and clinical remission in patients with hepatitis C virus (HCV) associated cryoglobulinemic vasculitis (HCV-CV), but clinical response in those with renal involvement is less clear. Our goal was to evaluate the clinical course following DAA therapy in one of the largest cohorts of patients with HCV-associated cryoglobulinemic glomerulonephritis (HCV-GN) reported to date. This is an observational study of patients with chronic HCV infection and circulating cryoglobulins (CC) treated with DAAs in our department from January 2015 to January 2019. We identified a total of 67 patients with HCV and CC out of which nine patients fulfilled the criteria of HCV-GN and had adequate clinical follow-up time. We describe a cohort of nine patients with a mean age of 57 years and known duration of HCV infection ranging 3-20 years (four with evidence of compensated cirrhosis). All patients received the ritonavir-boosted paritaprevir/ombitasvir/dasabuvir regimen for 12 weeks and achieved SVR without subsequent viral relapse. Following DAAs completion, one patient developed "new-onset" cryoglobulinemic glomerulonephritis, six showed either persistent or worsening glomerulonephritis, and only two patients had a complete clinical response (CCR). Of the six patients with either persistent or worsening CV, 67% received additional immunosuppressive (IS) therapy for uncontrolled CV. Of the two patients that had a CCR, one patient received prior IS therapy while the other one improved without any additional intervention. Newer HCV treatment protocols involving DAAs are highly successful in eradication of HCV infection; however, in our experience, DAA treatment alone is insufficient in improving the renal outcomes of patients with HCV-GN and additional IS therapies should be considered.


Assuntos
Antivirais/uso terapêutico , Glomerulonefrite/tratamento farmacológico , Hepatite C/complicações , Idoso , Crioglobulinemia/complicações , Crioglobulinemia/virologia , Feminino , Glomerulonefrite/virologia , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento
12.
BMJ Case Rep ; 12(5)2019 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-31138600

RESUMO

Cryofibrinogenaemia is a rare haematological disorder characterised by cold temperature-induced precipitation of plasma proteins causing small-vessel occlusive vascular disorder with a hallmark of skin ulceration. It remains an underdiagnosed entity because of a lack of diagnostic criteria. Cryoglobulinaemia vasculitis is a small-vessel vasculitis involving the skin, the joints, the peripheral nerve system and the kidneys. Its association with cryofibrinogenaemia causes more severe phenotype with poor prognosis. We describe the case of a 59-year-old woman presenting with cold-induced extensive bilateral foot gangrene due to coexisting cryofibrinogenaemia and cryoglobulinaemic vasculitis that required bilateral amputation and rituximab perfusions as maintenance therapy.


Assuntos
Crioglobulinemia/diagnóstico , Pé/irrigação sanguínea , Gangrena/etiologia , Amputação/métodos , Antirreumáticos/uso terapêutico , Crioglobulinemia/complicações , Crioglobulinemia/tratamento farmacológico , Diagnóstico Diferencial , Feminino , Pé/patologia , Gangrena/patologia , Gangrena/cirurgia , Humanos , Pessoa de Meia-Idade , Doenças Raras , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Resultado do Tratamento , Vasculite/complicações
13.
Arthritis Rheumatol ; 71(11): 1904-1912, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31136095

RESUMO

OBJECTIVE: Cryoglobulins are cold-precipitating immunoglobulins. Through progress in techniques, we undertook this study to update information on the biologic characteristics of cryoglobulins in a very large population. METHODS: A cohort of 13,439 patients was tested for cryoglobulins from January 2010 to December 2016. The analysis included cryoglobulin isotype, clonality, concentration, and IgM rheumatoid factor (IgM-RF) in cryoprecipitate, as well as serum complement and RF. Markers of gammopathy, viral infection, and autoimmunity were also investigated. RESULTS: Of the 13,439 patients, 1,675 (12.5%) tested positive for cryoglobulins: 155 patients (9.3%) with type I, 788 (47%) with type II, and 732 (43.7%) with type III cryoglobulins. Nine percent of patients who were retested after initially testing negative for cryoglobulins showed a positive result on a follow-up test (196 of the 2,213 retested patients). In type I cryoglobulins, IgM was more frequent but occurred at lower concentrations than IgG. Mixed cryoglobulins were found in 34.8% of the tested patients who were positive for hepatitis C virus and <5% of those who were positive for hepatitis B virus or HIV. Of the patients with anti-double-stranded DNA, anti-SSA, or anti-cyclic citrullinated peptide autoantibodies, 25.4% tested positive for mixed cryoglobulins, with type III occurring more frequently than type II. Both cryoprecipitate and serum were RF-positive in 21.6% of type II and 10.1% of type III cryoglobulins. A decrease of C4, with or without accompanying decreases of C3 and CH50, was found in 23.6% of cryoglobulin samples. CONCLUSION: Obtained with the use of modern assays, our findings from this very large collection of cryoglobulins provide an update on cryoglobulin distribution and characteristics, with minimal selection bias. Despite strict preanalytical conditions, a negative finding for the presence of cryoglobulin must be confirmed in a second sample. RF activity and complement decreases were rarely detected.


Assuntos
Anticorpos Anti-Proteína Citrulinada/imunologia , Anticorpos Antinucleares/imunologia , Proteínas do Sistema Complemento/imunologia , Crioglobulinemia/imunologia , Crioglobulinas/imunologia , Imunoglobulina M/imunologia , Fator Reumatoide/imunologia , Adulto , Idoso , Estudos de Coortes , Complemento C3/imunologia , Complemento C4/imunologia , Ensaio de Atividade Hemolítica de Complemento , Crioglobulinemia/complicações , Feminino , Infecções por HIV/complicações , Infecções por HIV/imunologia , Hepatite B/complicações , Hepatite B/imunologia , Hepatite C/complicações , Hepatite C/imunologia , Humanos , Transtornos Linfoproliferativos/complicações , Transtornos Linfoproliferativos/imunologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
14.
Acta Dermatovenerol Croat ; 27(1): 28-32, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31032788

RESUMO

Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic vasculitis is a necrotizing vasculitis with eosinophilic vascular infiltration, in which eosinophils mediate vascular damage in the disease process. We present a case of an 18-year-old girl who developed palpable purpura and hemorrhagic bullae over the lower extremities associated with itching, 7 days after the commencement of penicillin therapy. Plasma cryofibrinogen was positive. Histopathology showed an infiltration of eosinophils within and around the vessel walls and a complete absence of nuclear dust and neutrophils. Oral prednisone at 1 mg/kg induced remission in 2 weeks; the prednisone dose was tapered and discontinued after 2.5 months. There was no evidence of recurrence after 37 months of follow-up. Our patient represents a rare case of drug/penicillin-induced CNEV associated with cryofibrinogenemia, without systemic organ involvement.


Assuntos
Antibacterianos/efeitos adversos , Crioglobulinemia/complicações , Eosinofilia/induzido quimicamente , Penicilinas/efeitos adversos , Vasculite/induzido quimicamente , Adolescente , Crioglobulinemia/diagnóstico , Crioglobulinemia/terapia , Eosinofilia/diagnóstico , Eosinofilia/terapia , Feminino , Humanos , Vasculite/diagnóstico , Vasculite/terapia
15.
CEN Case Rep ; 8(3): 205-211, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30927247

RESUMO

A 65-year-old male patient with nephrotic syndrome was admitted to our hospital due to worsening systemic edema and purpura on the limbs. He had an impaired renal function, low serum complement level, and elevated rheumatoid factor level. He was positive for cryoglobulin (monoclonal IgM-κ and polyclonal mixed-type IgG), and the results of his kidney biopsy showed a tissue profile of membranoproliferative glomerulonephritis (MPGN). Due to the fact that the secondary cause was unclear, he was diagnosed with MPGN due to essential mixed cryoglobulinemia. On hospital day 20, he was initiated on 50 mg/day prednisolone (PSL). On hospital day 43, oral mizoribine (MZR) at a dose of 150 mg/day was prescribed. On hospital day 49, cryofiltration was performed because the disease was steroid resistant. The treatment promptly decreased urine protein levels. Serum albumin and serum complement levels increased, and complete remission was achieved approximately three months after the initiation of treatment. The PSL and MZR doses were gradually reduced to 2 mg/day and 100 mg/day, respectively, without any reemergence of the symptoms of cryoglobulinemia or relapse of the nephrotic syndrome for three years. Here, we report this case with essential mixed cryoglobulinemia in whom we could achieve complete remission of the disease by adding cryofiltration to the oral corticosteroid and immunosuppressant therapy with mizoribine and could maintain for a long time.


Assuntos
Remoção de Componentes Sanguíneos , Crioglobulinemia/complicações , Glomerulonefrite Membranoproliferativa/terapia , Imunossupressores/uso terapêutico , Ribonucleosídeos/uso terapêutico , Idoso , Glomerulonefrite Membranoproliferativa/etiologia , Glomerulonefrite Membranoproliferativa/patologia , Glucocorticoides/uso terapêutico , Humanos , Rim/patologia , Masculino , Prednisolona/uso terapêutico
18.
Reumatol Clin ; 15(3): 173-178, 2019.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-30691946

RESUMO

OBJECTIVE: To review the efficacy and safety of rituximab in vasculitic neuropathy (VN) METHODS: A literature search was performed on Medline and Embase up until 2017. It included terms related to "vasculitis","vasculitic neuropathy" and "Rituximab". Research was carried out by two reviewers. The main outcome was rituximab efficacy. RESULTS: Of an initial selection of 702 articles, 5 remained with a level of evidence between 1+ and 3 and variable recommendation degree. In the only clinical trial included, rituximab was superior to conventional therapy for cryoglobulinemic vasculitis with VN showing an increase in drug retention rate (64.3% vs. 3.5%; P<.001)and with a lower rate of serious adverse effects (.12 vs. .48). Cohort studies of patients with cryoglobulinemic vasculitis showed improvement and complete/partial remission of VN. In a series of 5 cases of refractory EGPA suffering from VN, 60% and 20% of patients achieved complete and partial remission respectively. CONCLUSIONS: Rituximab seems an effective and safe treatment for VN in the context of cryoglobulinemic vasculitis. Evidence for specific efficacy in VN in the context of other types of vasculitis is lacking.


Assuntos
Crioglobulinemia/tratamento farmacológico , Imunossupressores/uso terapêutico , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Rituximab/uso terapêutico , Vasculite/tratamento farmacológico , Ensaios Clínicos como Assunto , Estudos de Coortes , Crioglobulinemia/complicações , Humanos , Imunossupressores/efeitos adversos , Metanálise como Assunto , Estudos Multicêntricos como Assunto , Estudos Observacionais como Assunto , Doenças do Sistema Nervoso Periférico/etiologia , Rituximab/efeitos adversos , Vasculite/complicações
19.
Clin Rheumatol ; 38(5): 1257-1262, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30628015

RESUMO

Type I cryoglobulinemia is associated with B cell proliferative diseases, whereas essential mixed cryoglobulinemia is classically associated with infections, malignancy, and autoimmune diseases, but may be idiopathic. Prognosis in patients with grave manifestations and renal involvement is often poor. We report a case of a 40-year-old woman, 2 weeks post-partum for pre-eclampsia who was hospitalized with nephritic syndrome and acute renal failure. The patient harbored type I and type II cryoglobulinemia. Renal and cutaneous biopsies confirmed the diagnosis; however, an underlying etiology was not established. A bone marrow biopsy suggested monoclonal gammopathy of undetermined source (MGUS). Despite therapy with intravenous cyclophosphamide, rituximab, plasmapheresis, dialysis, and bortezomib, the patient succumbed after 8 months of hospitalization. We suggest that an overlap entity of types I and II cryoglobulinemia with severe multi-organ involvement not only is rare but also may be resistant to conventional therapy and fatal.


Assuntos
Crioglobulinemia/complicações , Crioglobulinemia/terapia , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/terapia , Adulto , Ciclofosfamida , Diálise , Evolução Fatal , Feminino , Humanos , Cadeias kappa de Imunoglobulina/análise , Rim/patologia , Plasmaferese , Rituximab , Pele/patologia
20.
Medicine (Baltimore) ; 98(4): e14201, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30681592

RESUMO

RATIONALE: Concomitant cryoglobulinemic vasculitis and cold agglutinin disease (CAD) is an extremely uncommon clinical scenario. The role of bortezomib in the treatment of cryoglobulinemic vasculitis needs further investigation. PATIENT CONCERNS: A 72-year-old Chinese woman presented with a 25-year history of cyanosis of the extremities after cold exposure, which worsened and was accompanied with purpuric skin lesions and proteinuria in recent years. Laboratory data demonstrated hemolysis. Cold agglutinin and cryoglobulin tests were positive. There was no evidence for malignancies after blood, image, and pathologic tests. DIAGNOSES: Concomitant cryoglobulinemic vasculitis and CAD. INTERVENTIONS: The patient was treated with bortezomib-based regimen, including bortezomib, cyclophosphamide, and dexamethasone. OUTCOMES: The patient responded well to the treatment. Both symptoms and laboratory tests significantly improved. The patient's condition was in a state of sustained remission in the 6-month follow-up. LESSONS: This rare case promotes further understanding of these 2 diseases and suggests that bortezomib is a promising treatment in type I cryoglobulinemic vasculitis.


Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Bortezomib/uso terapêutico , Crioglobulinemia/tratamento farmacológico , Vasculite/tratamento farmacológico , Idoso , Anemia Hemolítica Autoimune/complicações , Crioglobulinemia/complicações , Ciclofosfamida/uso terapêutico , Dexametasona/uso terapêutico , Feminino , Humanos , Resultado do Tratamento , Vasculite/complicações
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