RESUMO
Relapsing polychondritis (RP) is a rare systemic disorder characterised by recurrent, widespread chondritis of the auricular, nasal, and tracheal cartilages. Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised by the infiltration of IgG4-bearing plasma cells into systemic organs. However, 25-35% of patients with RP have a concurrent autoimmune disease. The coexistence of RP and IgG4 is rare considering that, to the best of our knowledge, there are only four previous reports of RP or RP-like symptoms associated with IgG4-RD. We herein report two cases which could be RP or RP-like symptoms associated with IgG4-RD.
Assuntos
Doenças Autoimunes , Doença Relacionada a Imunoglobulina G4 , Policondrite Recidivante , Doenças Autoimunes/diagnóstico , Humanos , Imunoglobulina G , Doença Relacionada a Imunoglobulina G4/diagnóstico , Policondrite Recidivante/diagnósticoRESUMO
OBJECTIVES: To define the proportion of osteonecrosis (ON) in our patient population with lupus and to identify factors associated with the development of ON in systemic lupus erythematosus, as well as to carry out a descriptive analysis of ON cases. MATERIALS AND METHODS: Observational retrospective study of 158 patients with SLE (ACR 1982 criteria). Demographic and laboratory data, clinical manifestations, SLICC, SLEDAI, cytotoxic and steroid treatments were compared. In patients with ON, we analyzed time of disease progression and age at ON diagnosis, form of presentation, joints involved, diagnostic methods, Ficat-Arlet classification, and treatment. To compare the means, t-test or Mann-Whitney's test were employed and the cHi-2 test or Fisher's exact test, as appropriate, were used to measure the equality of proportions. RESULTS: ON was present in 15 out 158 patients (9.5%), 13 women and 2 men, with a mean age of 30 (r: 16-66) at diagnosis and 35 months of evolution until diagnosis (r: 1-195). Among the 15 patients, 34 joints presented ON, 23 were symptomatic and 22 were diagnosed by magnetic resonance images. Twenty-six occurred in hips (24 bilateral), 4 in knees and 4 in shoulders. In 13 patients, ON involved 2 or more joints. At onset, 28 joints were in stage i-ii, one in stage iii and 5 had no data and; in the end, 14 were in stage iii-iv, 5 in stage i-ii and 15 had no data. Twenty-nine underwent conservative treatment with rest and 8 hips required joint replacement. ON progression was associated with Cushing's syndrome (P=0.014) OR 4.16 (95% CI 1.4-12.6) and 2nd year SLICC (P=0.042). No relation with clinical manifestations, lab results, cytotoxic treatment, steroid treatment (total accumulated dose, mean daily dose and duration) metilprednisolone pulses, nor activity was found. All patients with ON received antimalarials, in contrast to 77% of those without ON. CONCLUSIONS: The proportion of ON was 9.5%, mainly in women, 76% in hips (26) and 92% bilaterally. They were associated significantly with Cushing's syndrome and accumulated damage at second year.
Assuntos
Lúpus Eritematoso Sistêmico/complicações , Osteonecrose/etiologia , Adolescente , Adulto , Idoso , Progressão da Doença , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Osteonecrose/diagnóstico , Osteonecrose/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Adulto JovemRESUMO
The study includes 159 SLE patients seen between 1987 and 2011, of whom 116 were treated in the public health system and 43 in private practice. In the comparison between both groups, it was shown that patients in the public health system were younger at first consultation and at the onset of SLE, and that the mean duration of their disease prior to nephropathy was statistically significantly shorter. They also presented with more SLE activity (measured by Systemic Lupus Erythematosus Activity Index) such as fever, lower levels of C4, and elevated erythrocyte sedimentation rate. Although cyclophosphamide was administered more frequently to patients in the public health system group, there were no statistically significant differences in renal histological findings. A second renal biopsy was performed on 20 patients due to the presence of persistent proteinuria, peripheral edema, urinary casts, or because of previous defective renal specimens. The overall 10-year survival of the patients in the public health system was 78% compared to a survival rate of 91% for the patients in private practices. When survival was evaluated at 15 years, however, no differences were found (log rank test: 0.65). Patients from both public and private groups attended medical specialist practices and received early diagnoses and close follow-ups.
Assuntos
Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/terapia , Adulto , Argentina , Estudos Transversais , Atenção à Saúde , Demografia , Feminino , Humanos , Lúpus Eritematoso Sistêmico/mortalidade , Masculino , Setor Privado , Setor Público , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Se revisaron las historias clínicas de 159 pacientes con LES (criterios ACR 1982), 91% mujeres, con una edad media a la primera consulta: 30 años y un tiempo medio de seguimiento de 73 meses. El 51% presentó compromiso musculoesquelético, el 50% compromiso renal y el 44% presentó rash malar fotosensible. AAN (+) se determinó en 59% de los pacientes, consumo de complemento en 49% y linfopenia en 34.5%. 94/100 pacientes en quienes estuvo disponible el SLEDAI estaban en la primera consulta (media: 8). Durante la evolución, 70% de los pacientes fueron tratados con antipalúdicos y esteroides V.O. 80% de los pacientes permanecían vivos en al última consulta; la mortalidad fue del 17% debido principalmente a infecciones, hemorragía pulmonar e insuficiencia respiratoria.
Assuntos
Lúpus Eritematoso Sistêmico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/históriaRESUMO
Se revisaron las historias clínicas de 159 pacientes con LES (criterios ACR 1982), 91% mujeres, con una edad media a la primera consulta: 30 años y un tiempo medio de seguimiento de 73 meses. El 51% presentó compromiso musculoesquelético, el 50% compromiso renal y el 44% presentó rash malar fotosensible. AAN (+) se determinó en 59% de los pacientes, consumo de complemento en 49% y linfopenia en 34.5%. 94/100 pacientes en quienes estuvo disponible el SLEDAI estaban en la primera consulta (media: 8). Durante la evolución, 70% de los pacientes fueron tratados con antipalúdicos y esteroides V.O. 80% de los pacientes permanecían vivos en al última consulta; la mortalidad fue del 17% debido principalmente a infecciones, hemorragía pulmonar e insuficiencia respiratoria.(AU)
Assuntos
Lúpus Eritematoso Sistêmico , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/históriaRESUMO
We present the clinical and serological characteristics of four patients with rhupus (Simon's definition). The 4 patients with rhupus presented ACR criteria for SLE as well as for RA, ANA positive with titers ranging from 1/80 to 1/5,250, and positive anti-DNA, with the predominance of symmetrical erosive polyarthritis. We found anti-CCP positivity and high titers in 3 of the 4 patients, and positive antiphospholipid antibodies in 2 (anticardiolipin and LA), without manifestation of antiphospholipid syndrome. One patient presented renal affection, and 2 subcutaneous nodules. The 3 patients with RA preceded the manifestations of SLE by an average of 7.7 years. Two patients were refractory to conventional DMAR in combination, requiring biologic and mycophennolate mofetil.