Revisiting clinical differences between hypersensitivity vasculitis and Henoch-Schönlein purpura in adults from a defined population.

OBJECTIVES: Hypersensitivity vasculitis (HV) and Henoch-Schönlein purpura (HSP) are the most common entities included within the category of cutaneous vasculitis (CV). Palpable purpura and histological changes characterised by the presence of leukocytoclastic vasculitis are common in both conditions. Therefore, considerable overlap between them is often seen. It is especially true when the CV occurs in adults. To further establish clinical differences between these two conditions, in the present study we assessed the main clinical differences between HV and HSP in a wide and unselected series of adults with CV from a defined population. METHODS: We reviewed the clinical records of 297 consecutive adults (age >20 years) seen at a single centre between January 1975 and December 2012 that were classified as having HSP or HV according to the criteria proposed by Michel et al. (J Rheumatol 1992; 19: 721-8). RESULTS: Based on the inclusion criteria, 102 adult patients (71 men/31 women) were classified as HSP and 195 (104 men/91 women) as HV. The mean age was similar in both groups (55.8±16.5 years in HSP and 56.8±18.3 years in HV). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HV. Both at the beginning of the disease and when the CV was established clinical manifestations were more frequent in patients with HSP than in those with HV. It was the case for gastrointestinal (57.4% vs. 6.8%; p<0.001), joint (51.5% vs. 36.6%; p=0.01) and renal involvement (86.3% vs. 18.3%; p<0.001). Corticosteroid (56.7% vs. 22%; p<0.001) and cytotoxic drug (19.4% vs. 3.2%; p<0.001) use was also more common in patients with HSP. After a median follow-up of 15.5 (interquartile range- IQR; 3-37) months in HSP and 4 (IQR; 2-12) months in HV, the outcome was better in HV than in HSP. In this regard, complete recovery (72.6% vs. 85.4%; p=0.01) was more commonly observed in HV while residual renal involvement (15.3% vs. 4.2%; p<0.001) was more common in HSP. The disease relapsed in 35.3% of patients with HSP and in 24.4% with HV (p=0.07). CONCLUSIONS: Our results confirm the claim that these two diseases presenting with similar cutaneous involvement are certainly two separate entities with greater systemic involvement and less favourable outcome in HSP.

Tocilizumab in refractory aortitis: study on 16 patients and literature review.

OBJECTIVES: Non-infectious aortitis is often refractory to standard immunosuppressive therapy. Since IL-6 has been implicated in the pathogenesis of aortitis, we assessed the efficacy of the anti-IL6 receptor monoconal antibody tocilizumab (TCZ) in a series of patients with refractory non-infectious aortitis. METHODS: Review of 16 patients (14 women/2 men) with refractory aortitis diagnosed by imaging (CT angiography, MR angiography, and/or PET) that were treated with TCZ. RESULTS: The mean age±SD was 51.4±20.1 years. The underlying conditions were: Takayasu arteritis (TakA) (n=7 cases), giant cell arteritis (GCA) (n=7), relapsing polychondritis (RP) (n=1), and aortitis associated with retroperitoneal fibrosis (n=1). TCZ was the first biologic drug used in all patients with GCA and in the patient with aortitis associated with retroperitoneal fibrosis but in only 2 of 7 TakA patients. In the remaining cases anti-TNF inhibitors were prescribed before TCZ (standard dose was 8 mg/kg/iv/4 weeks). After a mean±SD follow-up of 11.8±6.6 months most patients experienced clinical improvement, showing reduction of erythrocyte sedimentation rate from 43±36 mm/1st h to 5±4 mm/1st h at last visit. At TCZ onset, 25% of patients had fever and 19% polymyalgia rheumatica. These manifestations disappeared after 3 months of TCZ therapy. A corticosteroid sparing effect was also achieved (from 27.3±17.6 mg/day of prednisone at TCZ onset to 4.2±3.8 mg/day at last visit). TCZ had to be discontinued in a patient because of severe neutropenia. CONCLUSIONS: TCZ appears to be effective and relatively safe in patients with inflammatory aortitis refractory to corticosteroids or to other biologic immunosuppressive drugs.

Extra-articular disease in patients with spondyloarthritis. Baseline characteristics of the spondyloarthritis cohort of the AQUILES study.

OBJECTIVES: To describe the prevalence of extra-articular disease (uveitis, psoriasis and inflammatory bowel disease [IBD]), in a cohort of patients with spondyloarthritis (SpA). PATIENTS AND METHODS: AQUILES is an observational, prospective and multicentric study of three cohorts of patients with one of the following immune-mediated inflammatory diseases (IMID): SpA, psoriasis, or IBD. In the present cohort, patients ≥18 years of age with SpA were enrolled from Rheumatology clinics. The main objective was to assess the coexistence of these diseases and of uveitis, based on the patients' clinical history up to the study entry. RESULTS: A total of 601 patients with SpA (men: 63.1%; women: 36.9%) were enrolled. The specific diagnoses were: ankylosing spondylitis (55.1%), psoriatic arthritis (25.1%), undifferentiated spondyloarthritis (16.1%), enteropathic arthritis (2.5%), and others (1.3%). In 43.6% (95% CI: 39.7-47.6) of the patients, at least one of the three abovementioned diseases was encountered, predominantly psoriasis (prevalence 27.8%, 95% CI: 24.4-31.5), uveitis (13.6%, CI 95%: 11.1-16.6) and IBD (5.1%, 95% CI: 3.7-7.2). In patients with ankylosing spondylitis the proportion of other disease was 25.3% (IBD: 3.9%, psoriasis: 5.4%, uveitis: 19.0%) whilst it was 94.7% in psoriatic arthritis, due to the presence of psoriasis (94.0%). The coexistence of these diseases was associated with age, female gender and the presence of other extra-articular manifestations associated with SpA. CONCLUSIONS: Extra-articular disease in patients with SpA is common and, in this study, it was associated to age, female gender and the presence of other SpA-related extra-articular manifestations.

Tocilizumab in giant cell arteritis: Multicenter open-label study of 22 patients.

OBJECTIVE: To assess the efficacy of tocilizumab (TCZ) in giant cell arteritis (GCA) patients with refractory disease and/or with unacceptable side effects due to corticosteroids. METHODS: A retrospective multicenter open-label study on 22 GCA patients treated with TCZ at standard dose of 8mg/kg/month. The main outcomes were achievement of disease remission and reduction of corticosteroid dose. RESULTS: The mean age ± standard deviation of patients was 69 ± 8 years. The main clinical features at TCZ onset were polymyalgia rheumatica (n = 16), asthenia (n = 7), headache (n =5), constitutional symptoms (n = 4), jaw claudication (n = 2), and visual loss (n = 2). Besides corticosteroids and before TCZ onset, 19 of 22 patients had also received several conventional immunosuppressive and/or biologic drugs. Of 22 patients, 19 achieved rapid and maintained clinical improvement following TCZ therapy. Also, after a median follow-up of 9 (interquartile range: 6-19) months, the C-reactive protein level had fallen from 1.9 (1.2-5.4) to 0.2 (0.1-0.9)mg/dL (p < 0.0001) and the erythrocyte sedimentation rate decreased from 44 (20-81) to 12 (2-20)mm/1st hour (p = 0.001). The median dose of prednisone was also tapered from 18.75 (10-45) to 5 (2.5-10)mg/day (p < 0.0001). However, TCZ had to be discontinued in 3 patients due to severe neutropenia, recurrent pneumonia, and cytomegalovirus infection. Moreover, 1 patient died after the second infusion of TCZ due to a stroke in the setting of an infectious endocarditis. CONCLUSION: TCZ therapy leads to rapid and maintained improvement in patients with refractory GCA and/or with unacceptable side effects related to corticosteroids. However, the risk of infection should be kept in mind when using this drug in patients with GCA.

Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.

The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3-20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2-38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement.

Manifestaciones extraarticulares en pacientes con espondiloartritis. Características basales de la cohorte de pacientes con espondiloartritis del estudio AQUILES / Extra-articular disease in patients with spondyloarthritis. Baseline characteristics of the spondyloarthritis cohort of the AQUILES study

Objetivos. Evaluar la prevalencia de enfermedad extraarticular (uveítis, psoriasis y enfermedad inflamatoria intestinal [EII]) en una cohorte de pacientes con espondiloartritis (EsA). Pacientes y métodos. AQUILES es un estudio observacional, prospectivo y multicéntrico, en 3 cohortes de pacientes con una de las siguientes enfermedades inflamatorias mediadas por inmunidad (EIMI): EsA, psoriasis y EII. En la cohorte presente se incluyó a pacientes ≥ 18 años con EsA atendidos en consultas hospitalarias de Reumatología. El objetivo principal fue evaluar la coexistencia de estas enfermedades y de uveítis, valorada sobre la base de la historia clínica del paciente hasta el momento de entrar en el estudio. Resultados. Se incluyó a 601 pacientes con EsA (varones: 63,1%, mujeres: 36,9%). Los diagnósticos fueron: espondilitis anquilosante (55,1%); artritis psoriásica (25,2%); espondiloartritis indiferenciada (16,1%); artritis enteropática (2,5%), y otros (1,3%). En el 43,6% (IC del 95%, 39,7-47,6) coexistió al menos una de las 3 enfermedades mencionadas, predominando psoriasis (prevalencia: 27,8%, IC del 95%, 24,4-31,5), uveítis (13,6%, IC del 95%m 11,1-16,6) y EII (5,1%, IC del 95%, 3,7-7,2). En pacientes con espondilitis anquilosante, la prevalencia fue del 25,3% (EII: 3,9%, psoriasis: 5,4%, uveítis: 19,0%) y en pacientes con artritis psoriásica fue del 94,7%, debido a la presencia de psoriasis (94,0%). La coexistencia de estas manifestaciones se asoció a mayor edad, sexo femenino y presencia de otras manifestaciones extraarticulares de las EsA distintas de las estudiadas. Conclusiones. La enfermedad extraarticular en pacientes con EsA es frecuente y en este estudio se asoció a la edad, el sexo femenino y la presencia de otras manifestaciones extraarticulares de EsA (AU)

Objectives. To describe the prevalence of extra-articular disease (uveitis, psoriasis and inflammatory bowel disease [IBD]), in a cohort of patients with spondyloarthritis (SpA). Patients and methods. AQUILES is an observational, prospective and multicentric study of three cohorts of patients with one of the following immune-mediated inflammatory diseases (IMID): SpA, psoriasis, or IBD. In the present cohort, patients ≥18 years of age with SpA were enrolled from Rheumatology clinics. The main objective was to assess the coexistence of these diseases and of uveitis, based on the patients’ clinical history up to the study entry. Results. A total of 601 patients with SpA (men: 63.1%; women: 36.9%) were enrolled. The specific diagnoses were: ankylosing spondylitis (55.1%), psoriatic arthritis (25.1%), undifferentiated spondyloarthritis (16.1%), enteropathic arthritis (2.5%), and others (1.3%). In 43.6% (95% CI: 39.7-47.6) of the patients, at least one of the three abovementioned diseases was encountered, predominantly psoriasis (prevalence 27.8%, 95% CI: 24.4-31.5), uveitis (13.6%, CI 95%: 11.1-16.6) and IBD (5.1%, 95% CI: 3.7-7.2). In patients with ankylosing spondylitis the proportion of other disease was 25.3% (IBD: 3.9%, psoriasis: 5.4%, uveitis: 19.0%) whilst it was 94.7% in psoriatic arthritis, due to the presence of psoriasis (94.0%). The coexistence of these diseases was associated with age, female gender and the presence of other extra-articular manifestations associated with SpA. Conclusions. Extra-articular disease in patients with SpA is common and, in this study, it was associated to age, female gender and the presence of other SpA-related extra-articular manifestations (AU)