A young girl with severe polyarteritis nodosa successfully treated with tocilizumab: a case report.

BACKGROUND: Childhood Polyarteritis nodosa (PAN) is a systemic vasculitis with necrotizing inflammation of medium- and small-sized arteries. Disease evolution may be severe and refractory to standard treatment including prednisone, azathioprine and cyclophosphamide. CASE PRESENTATION: We present the case of a young girl with severe PAN resulting in progressive ischemia and necrosis of fingers and toes. Biological work-up revealed increased acute phase reactants and interleukin-6 levels. She was only partially controlled despite high-dose corticosteroids and cyclophosphamide infusions, and eventually achieved rapid improvement and sustained remission on tocilizumab. Further, we review the current evidence of the interleukin-6-inhibitor tocilizumab for the treatment of PAN. CONCLUSION: Tocilizumab may be an efficient therapeutic option in a subset of treatment-refractory children with PAN.

Coronary artery bypass using bilateral internal thoracic artery grafts in polyarteritis nodosa.

Polyarteritis nodosa (PAN) affects small- and medium-sized arteries but rarely occurs in coronary artery aneurysms and stenosis. For patients with PAN, coronary artery bypass grafting (CABG) can be challenging, especially with respect to graft selection. We performed CABG using a bilateral internal thoracic artery (ITA) graft for a 21-year-old patient with PAN, with successful postoperative outcomes. Arterial grafts have the risk of stenosis in PAN, but the patient's condition was controlled by steroids and immunosuppressants, and angiography showed no stenosis. We decided to use the ITA graft as a bypass conduit and found that long-term follow-up and continuous treatment are necessary.

Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea.

Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3-17.5), 7.7 (3.5-17.6), and 7.0 (1.6-16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud's phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2-32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.

Destructive arthritis with cutaneous polyarteritis nodosa requiring surgical intervention: a case report and review of the literature.

Arthralgia is a well-known complication of cutaneous polyarteritis nodosa (CPN). However, few cases of destructive arthritis with CPN resulting in chronic pain and disability have been documented. Here we report a case of CPN associated with destructive arthritis of the right foot, accompanied by seronegative arthritis in the right ankle, for which orthopaedic surgery was necessary. X-ray and computed tomography revealed progressive joint destruction in the right talonavicular joint, and magnetic resonance imaging demonstrated severe synovitis of the right talocrural joint. When the talonavicular joint was opened, we observed marked synovitis and a defect in the cartilage that had expanded within the joint. Although mild cartilage degeneration was observed in the talocrural joint, arthroscopic observation revealed no massive defect on the articulated surface. Arthrodesis and arthroscopic synovectomy yielded a good midterm clinical outcome, indicating that surgical intervention was useful for the treatment for destructive arthritis with CPN. Rheumatologists and orthopaedic surgeons should be aware of potential complications such as destructive arthritis when treating patients with CPN.

Segmental Arterial Mediolysis with Preceding Symptoms Resembling Viral Infection Hampers the Differentiation from Polyarteritis Nodosa.

A middle-aged man presented with a fever, arthralgia, gastrointestinal symptoms, headache, and rash. After two weeks, the patient suddenly complained of severe abdominal pain, and computed tomography revealed aneurysms in the hepatic and splenic arteries, which increased in size progressively. Given the elevated levels of inflammatory markers and orchitis, polyarteritis nodosa (PN) was initially suspected. Catheter embolization for the ruptured hepatic aneurysm and splenectomy for the large splenic ones were performed, and the pathological finding was consistent with segmental arterial mediolysis (SAM). Changes in inflammatory marker levels and aneurysmal size are also informative to differentiate SAM from PN.

Polyarteritis nodosa isolated to the testis and urinary bladder in the setting of cryptorchidism: a case report and literature review.

BACKGROUND: Polyarteritis nodosa is a small vessel to medium vessel vasculitis that frequently presents with multi-organ involvement, but can sometimes be limited to single organs such as the testes. Patients often require treatment with glucocorticoids, plus or minus additional immunosuppressive therapy depending on the severity of the disease. We describe a rare case of polyarteritis nodosa involving the right testis and urinary bladder without other systemic features of vasculitis. CASE PRESENTATION: A previously healthy 54-year-old First Nations Canadian man presented with intermittent gross hematuria. He underwent surgical excision of his right testis for cryptorchidism and a transurethral resection of a bladder mass. Histology showed an active medium vessel vasculitis in both organs. On extensive clinical, laboratory, and radiographic review, he had no systemic features of vasculitis. On 2-year follow-up, he has not required any systemic therapy and has not developed further symptoms. CONCLUSION: Single organ polyarteritis nodosa can sometimes be managed with surgical excision of the involved organ alone. Although our patient had two organs involved, we extrapolated the results of our literature search to guide his care. This case highlights the potential for surgical excision to cure polyarteritis nodosa despite the involvement of two organs in the absence of symptoms and signs of systemic vasculitis.

Extraintestinal Clostridium difficile infection due to a ribotype different from that isolated from the feces of the patient: A case report.

Extraintestinal infections due to Clostridium difficile are uncommon. When such infections occur, extraintestinal C. difficile isolates are usually identical to fecal isolates. We present a rare case of a large postoperative abscess caused by C. difficile infection, in which different C. difficile strains were isolated from the abscess and from feces of the patient. An 82-year-old woman with cutaneous polyarteritis nodosa developed pain, skin ulcers, and extensive necrosis of the right leg. Above-knee amputation was performed without stopping antiplatelet therapy, leading to postoperative hematoma. Six weeks after surgery, a large femoral abscess was detected and C. difficile was isolated. Repeat amputation of the thigh was required to remove the abscess. C. difficile was also cultured from feces despite the lack of intestinal symptoms. However, genetic analysis confirmed that the C. difficile isolates from the abscess and feces were different strains. Thus, C. difficile can cause postoperative infection of a hematoma and the extraintestinal and fecal C. difficile isolates are not necessarily identical in the same patient.

Polyarteritis Nodosum of the Breast in a Patient with History of Bilateral Augmentation Mammoplasty.

Polyarteritis nodosum belongs to a group of inflammatory disorders characterized by necrotizing vasculitis of small and medium-sized blood vessels. To date, there are 14 publications that document involvement of the breast. Our publication is the second study documenting polyarteritis nodosum of the breast in a patient who had previously had augmentation mammoplasty. Level of Evidence V This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

[Asynchronous Bilateral Testicular Infarction with Suspected Polyarteritis Nodosa : A Case Report].

This report documents a case of asynchronous bilateral testicular infarction. The patient was a 42- year-old man who presented with left testicular pain and swelling. He had a past history of right idiopathic testicular infarction and underwent a right orchiectomy 6 years ago. He also had received treatment for 5 years for suspected polyarteritis nodosa (PAN). The left scrotal pain persisted for a week and left orchiectomy was performed. Pathological evaluations demonstrated a benign testis with testicular hemorrhage and chronic vasculopathy. There was no fibrinoid necrosis of medium-size vessel walls which characterizes PAN. In this report, we review the pathogenesis, risk of contralateral testicular infarction, and management of testicular infarction.

Single organ variant of polyarteritis nodosa in epididymis.

Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries, with occasional involvement of small muscular arteries. Unlike some other vasculitides (e.g. microscopic polyarteritis, Wegener's granulomatosis) PAN is not associated with antineutrophil cytoplasmic antibodies. Patients typically present with systemic symptoms such as fever, weight loss, and malaise. The kidneys, skin, joints, muscles, nerves, and gastrointestinal tract are commonly involved, usually in some combination. PAN can affect any organ, but usually spare the lungs. Clinical variants or subsets of PAN include single-organ disease and cutaneous-only PAN. Scrotal involvement is rarely the first presenting sign. We herein report a case of 36-year-old man who presented with a swelling in the left epididymis, which was surgically removed. The swelling histopathologically showed necrotizing inflammation, fibrinoid necrosis of the medium-sized arteries of the epididymis and was diagnosed to be single organ variant of PAN.

Flow diversion in vasculitic intracranial aneurysms? Repair of giant complex cavernous carotid aneurysm in polyarteritis nodosa using Pipeline embolization devices: first reported case.

Intracranial aneurysms in polyarteritis nodosa (PAN) are exceedingly rare lesions with unpredictable behavior that pose real challenges to microsurgical and endovascular interventions owing to their inflammatory nature. We introduce a safe and effective alternative for treating these aneurysms using Pipeline embolization devices (PEDs). A 20-year-old man presented with diplopia, headaches, chronic abdominal pain, and weight loss. Diagnostic evaluations confirmed PAN, including bilateral giant cavernous carotid aneurysms. Cyclophosphamide and steroids achieved significant and sustained clinical improvement, with a decision to follow the aneurysms serially. Seven years later the left unruptured aneurysm enlarged, causing a sudden severe headache and a cavernous sinus syndrome. Treatment of the symptomatic aneurysm was pursued using flow diversion (PED) and the internal carotid artery was successfully reconstructed with a total of four overlapping PEDs. At 6 months follow-up, complete exclusion of the aneurysm was demonstrated, with symptomatic recovery. This is the first description of using a flow-diverting technique in an inflammatory vasculitis. In this case, PEDs not only attained a definitive closure of the aneurysm but also reconstructed the damaged and fragile arterial segment affected with vasculitis.

Successful treatment of Group A ß-hemolytic Streptococcus infection-associated juvenile cutaneous polyarteritis nodosa with tonsillectomy.

Cutaneous polyarteritis nodosa (cutaneous PAN) is a form of necrotizing vasculitis of small- and medium-sized arteries, primarily involving the skin. In juvenile cases, cutaneous PAN is known to be frequently associated with Group A ß-hemolytic Streptococcus (GAS) infections. We herein describe the first reported juvenile case of GAS-associated recurrent cutaneous PAN successfully improved with tonsillectomy. To avoid the use of steroids and immunosuppressive drugs, especially in juvenile cases, tonsillectomy is a possible treatment for GAS-associated recurrent cutaneous PAN.

Catastrophic intracranial hemorrhage as a presenting feature of juvenile polyarteritis nodosa.

Intracranial hemorrhage has been rarely reported during the course of polyarteritis nodosa. We describe a 6-year-old boy who presented with fever, altered sensorium, skin rash, hypertension, and catastrophic intracranial hemorrhage. After surgical evacuation of the intracranial hematoma, he underwent a computerized tomography angiogram that showed narrowing of the right anterior cerebral artery. Skin biopsy showed small vessel vasculitis. Nerve conduction studies were suggestive of mononeuritis multiplex. He was diagnosed as polyarteritis nodosa and managed with immunosuppressants, to which he responded favorably. The most interesting aspect of the child's presentation was the catastrophic onset of altered sensorium with raised intracranial pressure, which was a diagnostic challenge. The mechanisms of intracranial hemorrhage in polyarteritis nodosa and a review of the literature are discussed. The authors emphasize that it is important to recognize intracranial hemorrhage as a life-threatening complication in children with polyarteritis nodosa to institute timely therapy.

Polyarteritis nodosa diagnosed by surgically resected jejunal necrosis following acute abdomen.

The differential diagnosis of acute abdomen is typically extremely broad in range, with vasculitis posing a rare but potentially life-threatening cause of acute abdomen. Here, we report a case of acute abdomen with bowel wall thickening limited to jejunum, accompanied by unexplained renal dysfunction. Later, the patient was diagnosed as having polyarteritis nodosa based on surgically resected jejunal necrosis. Despite aggressive treatment, including the use of steroid pulse therapy and continuous hemodiafiltration, the patient died. Although polyarteritis nodosa is extremely rare in patients with acute abdomen, acute abdomen is relatively common manifestation of that. And it is reported that involvement of small intestine suggests poorer prognosis. Our case highlights the importance of vasculitis as a differential diagnosis of patients with atypical acute abdomen. In this report, we not only review possible clues that might have led to an earlier diagnosis in this case, but also attempt to draw some lessons for treating similar cases in the future.

Coil embolization of intracranial aneurysm in polyarteritis nodosa. A case report and review of the literature.

Polyarteritis nodosa (PAN) is a rare multisystem disease characterized by systemic necrotizing arteritis of small and medium size arteries. The skin, joints, kidneys, gastrointestinal tract and peripheral nerves are most commonly involved. Although aneurysms are commonly seen in the visceral vessels, intracranial aneurysms are rare with 15 reported cases. The intracranial aneurysms are usually multiple and located in supra- as well as infra-tentorial compartments. Most of the cases presented with subarachnoid or parenchymal hemorrhage. The aneurysms were usually small, although large cavernous aneurysms were reported in one case. Treatment guidelines are not clear regarding the management of these cases. Most patients were treated conservatively by medical management with surgical excision performed in only two cases and coiling done in one patient with cavernous aneurysms. Repeat hemorrhages or re-bleed in spite of medical treatment have also been reported. We describe the case of a 22-year-old woman, a known case of PAN who presented with subarachnoid hemorrhage. Cerebral angiogram showed a ruptured right middle cerebral artery bifurcation aneurysm along with unruptured left middle cerebral, right posterior communicating and left posterior inferior cerebellar artery aneurysms. Her previous abdominal angiogram had revealed multiple aneurysms in visceral arteries. Successful coil embolization of the ruptured right MCA bifurcation aneurysm was performed with preservation of the parent vessel. The patient made a complete recovery and was placed on medical treatment for PAN. Follow-up MR angiography at three months revealed stable occlusion of the embolized aneurysm with no change in the unruptured aneurysms. Although rare, PAN can be associated with intracranial aneurysms which can cause subarachnoid or parenchymal hemorrhage. Selected cases can be treated safely by coil embolization.

Drug-eluting stent patency at 6 months in the pedal artery of a patient with polyarteritis nodosa: a case report.

Drug-eluting stents are largely used in coronary arteries and more recently in tibial arteries owing to their potentially better outcomes compared with bare metal stents. A patient with polyarteritis nodosa and critical limb ischemia and a dorsal foot ulcer was previously unsuccessfully treated with multiple angioplasties and subsequently underwent implantation of a drug-eluting stent in the pedal artery. At 6 months, stent patency on color Doppler ultrasound and complete healing of the foot ulcer were observed.

Re-examination of features to distinguish polyarteritis nodosa from superficial thrombophlebitis.

The correct identification of vessel type is crucial in distinguishing cutaneous polyarteritis nodosa from superficial thrombophlebitis. As the treatment and prognosis of these conditions are very different, correct diagnosis is critical, but they have overlapping clinical and histopathologic features, which can sometimes make the distinction difficult. Features commonly used to distinguish an artery from vein include vessel shape and diameter, the presence or absence of an internal elastic lamina, smooth muscle pattern, and the presence or absence of valves. Recently, it has been proposed that the amount and distribution of elastic fibers in the medial muscular layer are the most reliable features to make this distinction. The first part of this study used prosector-identified vessels to determine which of these features is most sensitive and specific for identifying an artery and vein. A total of 19 arteries and 16 veins were dissected from autopsy and amputation specimens. For each specimen, the smooth muscle pattern, elastic fiber pattern, the presence of valves, and the presence or absence of an internal elastic membrane were determined. The quantity of elastic fibers in the muscular wall of each sample was also determined. Although the elastic fiber pattern was the most specific feature in identifying a vein, it suffered from low sensitivity (43.8%). The smooth muscle pattern had the highest combined sensitivity and specificity. In the second part of this study, the histologic features listed above were examined in previously diagnosed cases of superficial thrombophlebitis and arteritis. When inflammation is present within and around the wall of the vessel, all of the studied histologic features become less reliable, and the interobserver reliability of distinguishing arteritis from thrombophlebitis was low. Our findings suggest that no single histopathologic feature is completely reliable and combining the histopathologic features with clinicopathologic correlation is essential for correct vessel identification.

Ruptured intracranial aneurysms in pediatric polyarteritis nodosa: case report.

Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis of small and medium size arteries that leads to aneurysms in various organs. Aneurysms associated with PAN are common in visceral arteries, however, intracranial aneurysms are rare, especially in childhood. A pediatric patient with PAN developed serial hemorrhagic strokes from a ruptured superior cerebellar artery aneurysm (subarachnoid hemorrhage) and a de novo aneurysm of the frontoorbital artery (intracerebral hemorrhage) after 9 months. Patients with PAN who present with intracranial aneurysms are candidates for intervention even if the aneurysm is unruptured and still small, and close observation is needed to detect de novo aneurysms in patients with chronic history of PAN.

Acute inferior myocardial infarction in a young female patient with polyarteritis nodosa.

Coronary involvement of polyarteritis nodosa (PAN) has been mostly identified in postmortem studies. We report a case with inferior myocardial infarction (MI) because of coronary dissection and thrombosis in PAN. A 23-year-old woman with chest pain was admitted to the emergency department. The admission ECG was suggestive of inferior MI with no right ventricular infarction. Coronary angiography revealed an occluded right coronary artery because of coronary dissection and concomitant thrombosis. Coronary angioplasty and stent implantation were performed successfully without complications. This report emphasizes the importance of PAN in spontaneous coronary dissection and thrombosis even in young patients.