To describe the clinical profile and compare the long-term outcomes of patients with systemic polyarteritis nodosa (S-PAN) treated with various treatment regimens at our centre in the last two decades.

Data regarding clinical presentation, treatment allocation, relapses and outcomes of patients fulfilling American College of Rheumatology (ACR) 1990 criteria for PAN in the last two decades were recorded from electronic medical records. Relapse-free survival and predictors were analysed using Kaplan-Meier survival statistics and regression analysis.

Altogether, 53 patients, including two with hepatitis B infection, were included. Cutaneous lesions and peripheral neuropathy were the commonest manifestations. Most patients (64.2%) presented with a five-factor score (FFS) of 0. Disease-attributable hypertension and peripheral gangrene were the most common manifestations of severe disease. During a median follow-up period of 53.5 months in 49 patients who had a follow up, 43 (87.8%) attained complete response while 3 (6.1%) had a partial response. Among 46 patients who had follow up of more than 3 months, 19 (41.3%) patients relapsed at a median duration of 82 (interquartile range 36.3-127.7) months. The relapse-free survival in patients who received induction with mycophenolate (n = 26) was comparable to that with cyclophosphamide (n = 21) [adjusted hazard ratio (HR): 0.68]. Smoking history was an independent predictor of relapse (HR = 6.28, P = 0.013) while age was protective (HR = 0.94, P = 0.015). Overall, fatality was observed in 5 (10%) patients. FFS and BVAS at 3 months were among the predictors of mortality.

In our cohort of S-PAN, relapses were observed in 41.3% of patients. Mycophenolate was similar to cyclophosphamide in maintaining relapse-free survival. Only 10% fatality was recorded. FFS and BVAS at 3 months were predictors of mortality.

Polyarteritis nodosa (PAN), also known as panarteritis nodosa, represents a form of necrotizing vasculitis that predominantly affects medium-sized vessels, although it is not restricted to them and can also involve smaller vessels. The clinical presentation is heterogeneous and characterized by a significant number of patients exhibiting general symptoms, including asthenia, fever, and unintended weight loss. Although PAN can involve virtually any organ, it preferentially affects the skin, nervous system, and the gastrointestinal tract. Orchitis is a rare but specific manifestation of PAN. The absence of granulomas, glomerulonephritis, and anti-neutrophil cytoplasmic antibodies serves to distinguish PAN from other types of vasculitis. Major complications consist of hemorrhagic and thrombotic events occurring in mesenteric, cardiac, cerebral, and renal systems. Historically, PAN was frequently linked to hepatitis B virus (HBV) infection, but this association has dramatically changed in recent years due to declining HBV prevalence. Current epidemiological research often identifies a connection between PAN and genetic syndromes as well as neoplasia. This article provides a comprehensive review of PAN, specifically focusing on the progression of its clinical manifestations over time.

Systemic vasculitides comprise a group of autoimmune diseases affecting blood vessels. [18F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (FDG-PET/CT) plays an important role in the diagnosis and therapeutic monitoring of vasculitides affecting large-sized and medium-sized vessels. FDG-PET/CT also provides complementary information to other vascular imaging tools. The resolution and sensitivity of newer generation scanners continues to increase, hereby improving the ability of FDG-PET/CT to accurately assess the full disease extent in patients with vasculitis. Novel tracers targeting specific immune cells will allow for more detailed detection of vascular infiltrates.

We conducted a medical records review study (1975-2018) that included patients with childhood- and adult-onset PAN. Demographics, organ involvement, phenotype, treatment, and outcomes were assessed and compared against previously published cohorts.

Thirty-one patients were included, 20 (64.5%) female, with a median age at diagnosis of 28 years (interquartile range [IQR], 16-42). Seven (23%) were classified as cutaneous; 23 (74%), systemic; and 1 (3%), progressive systemic phenotype. Eleven patients (35%) had childhood-onset PAN. Most common manifestations were musculoskeletal (71%), cutaneous (68%), constitutional (61%), peripheral neuropathy (39%), and gastrointestinal (29%). The median Birmingham Vasculitis Activity Score and Five-Factor Score at diagnosis were 9 (IQR, 4-13) and 1 (IQR, 0-1), respectively. Most patients were treated with glucocorticoids (94%). Twenty-four (80%) achieved complete and 6 (20%) partial remission at a median follow-up time of 30 months (8-192 months). The median Vasculitis Damage Index at last follow-up was 1 (IQR, 0-1). Nineteen (66%) experienced relapses. Patients with childhood-onset PAN more frequently had central nervous system and gastrointestinal involvement (36% vs 5%, p = 0.04 and 64% vs 10%, p = 0.003, respectively), microaneurysms (100% vs 38%, p = 0.02), and lower levels of C-reactive protein (0.3 vs 15.4 mg/dL, p = 0.03), compared with adult-onset PAN patients.

Our cohort of PAN patients showed predominantly a systemic phenotype. Outcomes were generally good, with most patients achieving complete remission. Childhood-onset differed from adult-onset PAN in terms of clinical and serological characteristics, whereas clinical manifestations and outcomes may be different than the ones reported in other cohorts.

This study aimed to clarify the epidemiological and clinical features and treatment of patients with polyarteritis nodosa (PAN) in Japan.

We used the database of the Ministry of Health, Labour and Welfare (MHLW) of Japan in 2013 and 2014. We analysed 121 patients who were antineutrophil cytoplasmic antibodies negative among the patients certified as PAN according to the MHLW diagnostic criteria.

The analysis included 60 males and 61 females, with a mean age of 52.9 ± 21.0 years. As a general manifestation, fever was observed in 53.7%. Regarding organ involvement, skin manifestations (82.6%), joint and muscle manifestations (75.2%), and neuropsychiatric manifestations (50.4%) were common. Male patients had a higher proportion of mononeuritis multiplex involving motor neuropathy than female patients. Elderly patients had a higher proportion of general and respiratory manifestations. Glucocorticoids were used for treatment in all patients, and 19.0% underwent methylprednisolone pulse. Concomitant immunosuppressants were used in 25.6%, one-third of whom received cyclophosphamide. Methylprednisolone pulse and cyclophosphamide were mostly used in patients with life-threatening organ involvement.

PAN developed in middle-aged people and led to numerous clinical manifestations. The common manifestations varied with age, and treatment was determined based on the type of organ involvement and disease severity.

Abdominal pain is a very common presentation in the accident and emergency department. However, vasculitis is not the usual first differential diagnosis. This paper discusses a case of polyarteritis nodosa presenting with acute abdominal pain alone. Common surgical conditions were obviously considered, but they were not found to cause the patient's problems. We describe how investigations led to this diagnosis discussed in detail in this paper. It is important to remember that prompt recognition of unusual life-threatening conditions can lead to timely intervention.

Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries. Different etiopathogenetic and clinical variants of the disease have been recognized over the past decades. In the present paper, we review the clinical features, diagnosis, and treatment of the different subtypes of the disease.

The diagnosis of polyarteritis nodosa is primarily based on clinical findings, imaging, and histopathological investigations. Microbiological and genetic investigations complement the diagnostic work-up. Idiopathic and hereditary variants of polyarteritis nodosa are treated with immunomodulatory medications such as glucocorticoids, conventional immunomodulatory drugs (e.g., cyclophosphamide) and biologic agents (e.g., tumor necrosis factor inhibitors, interleukin 6 inhibitor), while hepatitis B virus-associated polyarteritis nodosa primarily requires antiviral therapy combined with plasma exchange. PAN is a disease with heterogeneous presentations, severity, and therapeutic approaches. The overall prognosis of this disease is improving, mainly due to early diagnosis and more effective treatments. Treatment choices are guided mainly by the disease subtype and severity. In this review, we have presented the current knowledge on PAN clinical variants, their classification, diagnosis, and treatment approaches.

Human hepatitis viruses (HHVs) include hepatitis A virus, hepatitis B virus (HBV), hepatitis C virus (HCV), hepatitis delta virus, and hepatitis E virus and can cause liver inflammation in their common human host. Usually, HHV is rapidly cleared by the immune system, following acute HHV invasion. The morbidities associated with hepatitis A virus and hepatitis E virus infection occur shortly after their intrusion, in the acute stage. Nevertheless, the viral infectious process can persist for a long period of time, especially in HBV and HCV infection, leading to chronic hepatitis and further progressing to hepatic cirrhosis and liver cancer. HHV infection brings about complications in other organs, and both acute and chronic hepatitis have been associated with clinical presentations outside the liver. Vascular involvement with cutaneous and systemic vasculitis is a well-known extrahepatic presentation; moreover, there is growing evidence for a possible causal relationship between viral pathogens and vasculitis. Except for hepatitis delta virus, other HHVs have participated in the etiopathogenesis of cutaneous and systemic vasculitis via different mechanisms, including direct viral invasion of vascular endothelial cells, immune complex-mediated vessel wall damage, and autoimmune responses with stimulation of autoreactive B-cells and impaired regulatory T-cells. Cryoglobulinemic vasculitis and polyarteritis nodosa are recognized for their association with chronic HHV infection. Although therapeutic guidelines for HHV-associated vasculitis have not yet been established, antiviral therapy should be initiated in HBV and HCV-related systemic vasculitis in addition to the use of corticosteroids. Plasma exchange and/or combined cyclophosphamide and corticosteroid therapy can be considered in patients with severe life-threatening vasculitis manifestations.

Heart involvement in vasculitis is rare, but potentially severe. The ascertainment of cardiac disease in vasculitis is complex and requires an integrated multidisciplinary approach involving the Rheumatologist, Radiologist, Cardiologist, and Heart surgeon.

the authors searched PubMed using the keywords 'heart'[Mesh] and vasculitis"[Mesh].

Virtually any vasculitis can affect the heart, but cardiac involvement is more common in some vasculitides such as Takayasu arteritis, polyarteritis nodosa, and eosinophilic granulomatosis with polyangiitis. Immunosuppressive treatment and when indicated surgery can improve the prognosis.

Vasculitis is a group of several clinical conditions in which the main histopathological finding is fibrinoid necrosis in the walls of blood vessels. This article assesses the main dermatological aspects relevant to the clinical and laboratory diagnosis of small- and medium-vessel cutaneous and systemic vasculitis syndromes. The most important aspects of treatment are also discussed.

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that predominantly affects medium-sized arteries. With the establishment and refinement of vasculitis nomenclature and diagnostic criteria, clinical findings of PAN and distinguishing features from other vasculitides are now well characterized. Although PAN typically manifests in adulthood, cohort studies in paediatric patients have shaped our understanding of childhood-onset PAN. The paradigm of childhood-onset PAN changed considerably with the landmark discovery of deficiency of ADA2 (DADA2), a monogenic cause of vasculitis that is often indistinguishable from PAN. Testing for DADA2 has provided an explanation to numerous challenging cases of familial PAN and early-onset PAN around the world. The ability to distinguish DADA2 from classic PAN have important therapeutic implications as tumor necrosis factor inhibitors have demonstrated remarkable efficacy in the treatment of DADA2. In this review, we will discuss our current understanding of PAN and DADA2 and highlight similarities and differences between these vasculitides.

Hepatitis B virus (HBV) infection leads to hepatic and extrahepatic manifestations including chronic kidney disease (CKD). However, the association between HBV and CKD is not clear. This study investigated the association between chronic HBV infection and CKD in a nationwide multicenter study.

A total of 265,086 subjects who underwent health-check examinations in 33 hospitals from January 2015 to December 2015 were enrolled. HBV surface antigen (HBsAg) positive cases (n = 10,048), and age- and gender-matched HBsAg negative controls (n = 40,192) were identified. CKD was defined as a glomerular filtration rate (GFR) < 60 mL/min/1.73 m2 or proteinuria as at least grade 2+ of urine protein.

HBsAg positive cases showed a significantly higher prevalence of GFR < 60 mL/min/1.73 m2 (3.3%), and proteinuria (18.9%) than that of the controls (2.6%, P < 0.001, and 14.1%, P < 0.001, respectively). In the multivariate analysis, HBsAg positivity was an independent factor associated with GFR < 60 mL/min/1.73 m2 along with age, blood levels of albumin, bilirubin, anemia, and hemoglobin A1c (HbA1c). Likewise, HBsAg positivity was an independent factor for proteinuria along with age, male, blood levels of bilirubin, protein, albumin, and HbA1c. A subgroup analysis showed that HBsAg positive men but not women had a significantly increased risk for GFR < 60 mL/min/1.73 m2.

Chronic HBV infection was significantly associated with a GFR < 60 mL/min/1.73 m2 and proteinuria (≥ 2+). Therefore, clinical concern about CKD in chronic HBV infected patients, especially in male, is warranted.

The objective of this study was to investigate whether clinical and laboratory data, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis could predict relapse in 30 patients with polyarteritis nodosa (PAN) having the follow-up duration for over 12 months. We reviewed the medical charts of 30 patients with PAN. We obtained clinical and laboratory data at diagnosis, and we compared them between the two groups based on relapse. The optimal cut-off values of BVAS and FFS (1996) at diagnosis to predict relapse were extrapolated. The mean age of patients (15 men) was 50.8 years, and the mean follow-up duration was 64.1 months. Nine patients (30.0%) had experience relapse after remission. Patients having relapse showed the higher frequency of weight loss and ocular symptoms and the less frequency of diastolic hypertension than those having not (p < 0.005 for all). On multivariate logistic regression analysis, weight loss was the only independent predictor of relapse, but on Cox Hazard model analysis, its statistical significance disappeared. The mean initial BVAS and FFS (1996) of patients in relapse group were higher than those of patients in no relapse group (p < 0.005 for all). Patients having initial BVAS over 13.5 and FFS (1996) over 1 exhibited significantly higher risk of relapse than those having not (RR 40.0 and RR 7.0, respectively). However, initial BVAS over 13.5 only remained significant in Kaplan-Meier survival analysis. In conclusion, BVAS over 13.5 at diagnosis was the only independent predictor of relapse of PAN.

There has been a significant decrease in the number of published reports of classical polyarteritis nodosa (PAN) in the post-Chapel Hill consensus conference (CHCC) nomenclature era with only two series published from Asia. We report a case series of PAN from north India.

A retrospective study of all patients diagnosed to have PAN according to American College of Rheumatology criteria/CHCC nomenclature. The details of clinical presentation, investigation findings, treatment details and outcomes were noted from the records. These findings between the hepatitis B positive and negative groups were compared.

Twenty-seven patients (20 male, seven female) were diagnosed as having PAN, out of which seven (25.9%) were hepatitis B surface antigen positive. Nervous system involvement was most common with 24 patients (88.9%) having mononeuritis multiplex. Weight loss was present in 20 (74%), fever in 14 (51.9%), renal involvement in 16 (59.3%), cutaneous in nine (33.3%), peripheral gangrene in eight (29.6%), gastrointestinal (GI) involvement in eight (29.6%), testicular pain in 6/20 (30%) and cardiac involvement in four (14.8%). Twenty-three (85.2%) patients recovered, three died (11.1%) and one was lost to follow-up. Median follow-up duration was 37 (interquartile range 22.00-69.75) months. The cumulative survival was 114.16 months (95% CI: 98.27-129.95). There was no significant difference in five factor score (FFS) or revised FFS between those patients who died and those who survived (P = 0.248, 0.894, respectively). Hepatitis B-related PAN had a lower FFS compared to non-hepatitis B-related PAN (P = 0.039). No other significant differences were noted between the two groups.

In comparison to classic PAN in other populations, classic PAN in north India is associated with higher neurological involvement and lower GI involvement.

Microscopic polyangiitis (MPA) is a systemic vasculitis affecting small vessels. To determine the clinical features and outcomes of MPA in Korean patients, we retrospectively reviewed the medical records of patients diagnosed with MPA at a single medical center in Korea between 1989 and 2006. The 18 patients who met the Chapel Hill criteria for MPA had a mean (+/-SD) age at the time of diagnosis of 62.4+/-12.7 yr. Renal manifestations and general symptoms were the most common features of MPA, with lung involvement also very common. Antineutrophil cytoplasmic antibodies (ANCA) were present in 17 of the 18 patients (94%). Of 17 patients treated with steroids and cyclophosphamide, 11 (65%) had stable or improved course. One patient treated with steroids without cyclophosphamide showed disease progression. Ten of the 18 patients (56%) died at a median follow-up of 8 months. MPA in Korean patients was distinguished by a higher rate of lung involvement, especially alveolar hemorrhage, which was the leading cause of death in our patients. Korean patients were also older at MPA onset and were more likely positive for ANCA. Other overall clinical manifestations did not differ significantly.

Polyarteritis nodosa (PAN) is a necrotising vasculitis of medium-sized vessels of unknown origin. This type of vasculitis is usually systemic, but restriction to a single organ, for example the testis, the appendix or the gall bladder, can occur. Testicular pain or tenderness are frequent clinical features. In this report, we present three cases of PAN. In every patient, testicular pain was the main symptom or first sign of systemic disease. We state that a thorough history taking, clinical examination and biochemical analyses are obligatory in patients presenting with acute or chronic scrotal pain. Polyarteritis nodosa should always be taken into account, and a search for systemic spread is mandatory. We emphasize that before initiation of systemic therapy with corticosteroids and/or cyclophosphamide, a Five Factor Score should be obtained, which also gives crucial prognostic information.

In this short review we focus on the problems faced by clinicians caused by the changing definitions of polyarteritis nodosa.

The term polyarteritis nodosa has been used for more than 100 years as a diagnostic term for patients with systemic vasculitis; however, specific vasculitides have been singled out like branches being chopped off a tree. Now, so little is left of the trunk of that tree that it is questionable to what extent we can trust older literature with respect to clinical features, natural history and response to treatment. Many authors of case reports, as well as authors of reviews and book chapters, claim they adhere to the Chapel Hill Consensus Conference definition of polyarteritis nodosa, yet still cite almost exclusively studies using older definitions without highlighting this dilemma. In the past year, two proposals affecting classification have been published: one stating that cutaneous polyarteritis nodosa and hepatitis-associated polyarteritis nodosa are diseases distinct from classical polyarteritis nodosa, and one providing an algorithm to separate microscopic polyangiitis from classical polyarteritis nodosa.

There is hope that a wide acceptance of the new classification principles will lead to a more uniform way to diagnose classical polyarteritis nodosa, which will facilitate clinical studies and eventually improve management.

Protean clinical manifestations of polyarteritis nodosa are described. Hence, a sequential multidisciplinary diagnostic approach, including thorough dermatologic examination and histologic verification in particular, are warranted in patients suspected of having this condition. The lack of both pathognomonic visceral and/or cutaneous features and specific serologic tests for identifying polyarteritis nodosa explains why making the diagnosis is often delayed. Furthermore, in some patients making the diagnosis is hampered because symptoms are missing or only mildly expressed. We report on a 67-year-old man diagnosed with systemic polyarteritis nodosa whose primary complaints included diplopia, extraordinary muscular pain of the lower extremities, and impaired walking. Inconspicuous subcutaneous nodules developed subsequently. The patient was treated initially with a pulse therapy of prednisolone (1000 mg/day for 2 days), followed by prednisolone 100 mg/day, gradually reducing over weeks. Rapid improvement in clinical and laboratory status was noted. The key message from this case report is that symptoms such as severe muscular pain of the lower extremities and acute diplopia, although also common to other systemic vasculitides and systemic autoimmune diseases, should raise early suspicion of a developing polyarteritis nodosa.