A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts.

There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. All paediatric-onset adult patients met the Ankara 2008 (EULAR/PReS endorsed) criteria for childhood PAN. Those with typical angiographic and/or histopathologic findings consistent with PAN were included. 93 (M/F: 51/42) patients (UK: 47, TR: 46) were included. Three were HBV-related, 20 (21.5%) had paediatric onset and 16 (16.5%), cutaneous PAN. TR patients had younger age of disease onset 44 (28.5-59.0) vs. 24.5 (11.8-40.5), p = 0.002. Twelve (26%) of TR patients had monogenic disease (Familial Mediterranean Fever association (n = 7), deficiency of adenosine deaminase 2, DADA2, (n = 5). No difference was found in phenotype between paediatric and adult onset patients except for frequency of cutaneous lesions (p = 0.002). During a median 67.5 (32-126) months follow-up, 13 patients died (12.7% in UK vs. 15.2% in Turkish cohorts). No difference was found between two cohorts in relation to relapse rate, death and vasculitis damage index. This study defined a diagnosis of PAN according to the EMEA algorithm. The TR group had a younger age of disease onset and more cases of monogenic disease; however, disease extent, relapse rate, damage index and death rates were similar between groups.

Polyarteritis nodosa in Croatian children: a retrospective study over the last 20 years.

To analyze the disease characteristics, treatment modalities and outcome of polyarteritis nodosa (PAN) in Croatian children. Cross-sectional study included all children with PAN diagnosed according to EULAR/PRES/PRINTO criteria during the last two decades. PAN was diagnosed in 12 patients (6 girls and 6 boys) mean age (±SD) 11.33 ± 3.08 years. The share of PAN among all vasculitides was 3.8 %. Systemic PAN was diagnosed in 7 children (58 %), microscopic polyangiitis in 3 (25 %), cutaneous PAN in 2 (17 %). The most consistent symptoms were skin involvement (90 %) and arthritis/arthralgia (60 %). The CNS was affected in 33 % of patients. Inflammatory markers (C-reactive protein and erythrocyte sedimentation rate [ESR]) were elevated in all patients, and anti-neutrophil cytoplasmatic antibodies were positive in all patients with microscopic polyangiitis. Therapy mode for all patients was corticosteroids. Immunosuppressive drugs were used as additional therapy for patients with severe symptoms. Two patients (17 %), both suffering from microscopic polyangiitis, died due to renal failure during the follow-up. In comparison with available studies, we found a difference in distribution of childhood polyarteritis nodosa as well as some clinical characteristics (e.g., higher prevalence of neurological and pulmonary symptoms), while other researched features, laboratory and treatment were similar.

Clinical features of polyarteritis nodosa in Korea.

Polyarteritis nodosa (PAN) is a systemic vasculitis characterized by multi-organ involvement with protean manifestations. We evaluated the clinical features of PAN in Korea. Twenty-seven patients were diagnosed as PAN at Seoul National University Hospital between January 1990 and July 2003. The male-to-female ratio was 1.7:1 and mean age at onset (+/-SD) was 47.4+/-20 yr. Their presenting features at diagnosis were similar to those reported previously, i.e., myalgia, muscle weakness or leg tenderness (70%), fever (52%), weight loss >4 kg (44%), skin rash (44%), peripheral edema (33%), abdominal pain (33%), and arthralgia/arthritis (30%). However, the prevalence of testicular pain or tenderness was higher (24%) than reported previously and only three (11.5%) had HBsAg positivity without liver enzyme elevation. Nine patients (33%) had a five-factor score (FFS) of 2. Fourteen patients (52%) responded to treatment, 2 patients relapsed and 4 died within 1 yr of diagnosis. During a median follow-up of 55.5 months, three of the four PAN-related deaths had an initial FFS of 2. The clinical features of PAN were not significantly different from those reported previously. However, testicular pain or tenderness was more frequent and patients with a high FFS tended to have a poorer prognosis.

Clinical Features of Polyarteritis Nodosa in Korea

Polyarteritis nodosa (PAN) is a systemic vasculitis characterized by multi-organ involvement with protean manifestations. We evaluated the clinical features of PAN in Korea. Twenty-seven patients were diagnosed as PAN at Seoul National University Hospital between January 1990 and July 2003. The male-to-female ratio was 1.7:1 and mean age at onset (+/-SD) was 47.4+/-20 yr. Their presenting features at diagnosis were similar to those reported previously, i.e., myalgia, muscle weakness or leg tenderness (70%), fever (52%), weight loss >4 kg (44%), skin rash (44%), peripheral edema (33%), abdominal pain (33%), and arthralgia/arthritis (30%). However, the prevalence of testicular pain or tenderness was higher (24%) than reported previously and only three (11.5%) had HBsAg positivity without liver enzyme elevation. Nine patients (33%) had a five-factor score (FFS) of 2. Fourteen patients (52%) responded to treatment, 2 patients relapsed and 4 died within 1 yr of diagnosis. During a median follow-up of 55.5 months, three of the four PAN-related deaths had an initial FFS of 2. The clinical features of PAN were not significantly different from those reported previously. However, testicular pain or tenderness was more frequent and patients with a high FFS tended to have a poorer prognosis.

Polyarteritis nodosa: a report from Israel.

BACKGROUND: The clinical manifestations and outcome of all adult patients with polyarteritis nodosa (PAN), allocated during a 15-year period in the largest medical center in Israel, were examined. METHODS: A retrospective analysis of patients with PAN who fulfilled the American College of Rheumatology (ACR) 1990 Classification Criteria and were either biopsy- or angiography-proven. RESULTS: Nine patients were included in the report. The clinical and laboratory manifestations were similar to those in previous studies. All patients were treated with combinations of cyclophosphamide and corticosteroids. There were two (22%) deaths, 2 and 5 months after initiation of treatment in patients who probably had microscopic polyangiitis (MPA) rather than classical PAN. Considering the patients with a complete follow-up, 71% had a complete and long-term remission. Moreover, by exclusion of the two patients with probable MPA who died, all of the five patients with classical PAN were alive and well as of this writing. Two patients (22%) had a long history, since childhood, of familial Mediterranean fever (FMF). CONCLUSIONS: The clinical presentation and course of PAN in Israeli patients is comparable with reports elsewhere. However, a distinction should be made between PAN and MPA. The present report emphasizes the good long-term prognosis of patients with typical PAN who are treated adequately. In addition, a possible association of PAN with FMF in Israeli patients is suggested.

Poliangeitis microscópica: reporte de un caso

La poliarteritis o poliangeitis microscópica es considerada como una vasculitis necrotizante sistémica que se asocia al Sindrome Pulmón-riñon cursando con hemorragia alveolar difusa y glomerulonefritis necrotizante segmentaria con compromiso multisistémico. Se presenta un caso de esta entidad, en un paciente de 50 años con su compromiso clínico, hallazgos imagenológicos, de laboratorio e histopalógicos, así como criterios diagnósticos

Microscopic polyarteritis has a poor prognosis in Chinese.

OBJECTIVE: To analyze retrospectively the clinical course and outcome of 10 consecutive Chinese patients with microscopic polyarteritis (MPA) admitted to our hospital over a period of 3 years. METHODS: Ten patients with MPA who presented 1990-93 were studied. Their serum antineutrophil cytoplasmic autoantibody levels were assayed. RESULTS: The male to female ratio was 1:1 and the mean age was 61 years. Eight patients (80%) had renal impairment on presentation and 5 of them (50%) were dialysis dependent. Seven patients (70%), during their clinical course, had pulmonary hemorrhage. Six patients (60%) had hypoxic lung disease requiring ventilatory support. All 10 patients were pANCA positive; 8 patients were tested for antimyeloperoxidase antibody and were all positive. Seven patients were treated with immunosuppressants consisting of steroid and cyclophosphamide with additional plasma exchange. Nine patients died after a mean survival of 9 months (range 1-33); causes of death were respiratory failure (5), infection (2), uremia (one), and stroke (one). The ANCA level reflected the disease activity of the vasculitis. CONCLUSION: MPA in Chinese patients appeared to be a more severe disease with a poor prognosis. This may be related to the older age group, and the more frequent and severe pulmonary and renal manifestations of MPA in these patients.