Diagnosis Of Polyarteritis Nodosa Research Articles

A Case of Renal Infarcts Followed by Renal Hematoma: The Importance of Angiography in the Diagnosis of Polyarteritis Nodosa

A Case of Renal Infarcts Followed by Renal Hematoma: The Importance of Angiography in the Diagnosis of Polyarteritis Nodosa

Polyarteritis Nodosa: Clinical Cases in Boys 12 and 9 Years Old

Background. Polyarteritis nodosa is an acute, subacute or chronic immune complex disease associated with peripheral and visceral artery involvement, predominantly of middle and small sizes, development of destructive-proliferative arteritis and subsequent peripheral and visceral ischaemia.Cases description. The present paper describes two clinical cases of polyarteritis nodosa in patient R., aged 12, and patient A., aged 9, and demonstrates the difficulties of diagnosing the disease in its early stages. Patient R., aged 12, was admitted to the Rheumatology Unit of the Krasnodar Krai Children’s Clinical Hospital with complaints of red, patchy, dense rash on the palms and plantar surface of the feet. The child has been ill since September 2017, and after a history of tonsillitis suffered a fever of 37 °C, pain in the right heel area, nodular thickening on the feet, livedo reticularis, swelling of both hands. The disease had a recurrent course. The boy was treated with prednisolone, mycophenolate mofetil, hydroxychloroquine and three courses of rituximab (April 2018, January 2019, September 2020). Repeated courses of human normal immunoglobulin and alprostadil therapy were carried out. The treatment showed positive dynamics, fever was eliminated, general well-being improved, and acute inflammatory markers in blood became normal. The skin retained minimal manifestation of livedo, nodularities on the feet did not progress in dynamics. Patient A. was admitted to the Rheumatology Unit of the Krasnodar Krai Children’s Clinical Hospital in April 2022 with complaints of weakness, myalgia of the lower extremities and necrosis foci in the left lumbar region. The medical history indicates that in March 2022, the boy, being in good health before, developed a bluish, painful rash on his lower legs after a workout. Skin changes and soreness resolved on their own without treatment. After examination, a diagnosis was made as follows: juvenile polyarteritis nodosa, activity score — 3.Conclusion. The diagnosis of polyarteritis nodosa can be often problematic due to the very character of the disease featured by absence of specific symptoms, by polymorphism of clinical manifestations, and by lack of clear diagnostic and laboratory markers.

OA18 A rare presentation of Polyarteritis Nodosa

OA18 A rare presentation of Polyarteritis Nodosa

Posterior Reversible Encephalopathy (PRES) as a Presentation Form of Classical Polyarteritis Nodosa (PAN)

A 21-year-old woman with unremarkable past health presented with an intense headache and visual disturbance, followed by a secondary generalized tonic-clonic focal seizure. She had a history of arthritis, livedo reticularis, myalgia, elevated erythrocyte sedimentation rate, and nonspecific constitutional manifestations (fever and weight loss). Examination revealed new-onset severe hypertension. Complete immunological studies were negative. Electroencephalogram showed abnormalities compatible with secondarily generalized tonic–clonic focal occipital seizures. Brain magnetic resonance imaging (MRI) revealed high signal intensity on T2 in occipital lobes, cerebellum, and brainstem and renal imaging multiple hypoenhancing parenchymal images (renal infarction). Follow-up brain MRI at 3 months showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made.

P087 Polyarteritis nodosa: a case report

Abstract Background Polyarteritis nodosa is a necrotizing vasculitis of small and medium caliber arteries. Rarely described in children, its pathophysiology is complex and remains poorly elucidated. Two main forms were described in the literature: cutaneous and visceral. Material and results We report the cases of two boys, aged of 7 and 11 years old. They had a history of recurrent sore throat with a recurrent aphthosis in the 11-year-old child. Both boys presented with polyarthralgias, myalgias, polymorphous skin lesions made of livedo reticularis with subcutaneous nodosa on the lower limbs in the first child and distal necrosis of the toes with oedema of the lower limbs in the second. Patients suffered also of cough evolving in a context of alteration of the general state made of fever and asthenia. A biological inflammatory syndrome was present in both children and the skin histology confirmed periarteritis nodosa. The evolution was marked by the disappearance of arthralgias and myalgias after a corticosteroid-based treatment with progressive dose reduction until minimal effective dose. Conclusion The diagnosis of Polyarteritis nodosa should be made in any child presenting with the following signs: fever, altered general condition, myalgias, arthralgias and skin manifestations. The prognosis is usually benign but long-term surveillance is necessary.

Clinical Approach to Diagnosis and Therapy of Polyarteritis Nodosa.

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.

Polyarteritis nodosa in a patient on hemodialysis; a case report

We represent a 40-year-old woman with end-stage renal disease (ESRD) of unknown etiology referred to the emergency department with episodes of chills and fever during hemodialysis. Further assessments revealed vasculitic skin rashes, as well as abdominal tenderness and later on, bloody diarrhea. Abdominal pain worsened during the course of admission, and as a result, the patient underwent cholecystectomy and appendectomy. Necrotizing vasculitis was diagnosed based on biopsy specimens taken from gall bladder and skin rashes. The diagnosis of poly arteritis nodosa (PAN) was established based on pathologic report and rheumatologic tests. Methylprednisolone pulses were administrated for 3 days followed by oral prednisolone and cyclophosphamide. After treatment, the signs and symptoms subsided. The present case report highlights the importance of timely diagnosis of PAN to prevent potentially irreversible consequences.

Necrosis of the small intestine leading to a diagnosis of polyarteritis nodosa: a case report

BackgroundPolyarteritis nodosa is a disease that presents with necrotizing vasculitis in small and medium-sized arteries. It may occur in various organs, but approximately half of cases have gastrointestinal involvement. Prognosis is not favorable once organ dysfunction begins as evidenced by gastrointestinal symptoms; thus, treatment with steroids should be promptly initiated. We report the case of a patient who presented with necrosis of the small intestine, which was pathologically diagnosed as polyarteritis nodosa and treated successfully with steroids.Case presentationAn 18-year-old Japanese woman reported a sudden onset of abdominal pain and vomiting that led her to visit our emergency department, where she was evaluated by a physician. On physical examination, tenderness to palpation in the upper umbilical region was noted, and diagnostic imaging with computed tomography showed emphysema of the wall of her small intestine. She was diagnosed as having necrosis of the small intestine requiring urgent surgery. No strangulations were noted intraoperatively but approximately 20 cm of her small intestine was necrotized. The surrounding arteries were examined and no palpable pulse was observed; therefore, segmentectomy of the necrotized regions was performed. Pathological findings revealed active vasculitis with fibrinoid necrosis, as well as destruction, fibrogenesis, and luminal stenosis of the elastic lamina found in the muscular arteries. A diagnosis of polyarteritis nodosa was confirmed as the cause of the necrosis of her small intestine. No recurrence of polyarteritis nodosa symptoms was observed when she was administered 40 mg of prednisolone daily.ConclusionIn cases of idiopathic intestinal necrosis or perforation, systemic diseases such as polyarteritis nodosa should be considered in the differential diagnosis.

Can rheumatoid vasculitis predate a diagnosis of rheumatoid arthritis?

We report regarding a male patient who presented with a systemic vasculitis that was consistent with a diagnosis of polyarteritis nodosa. At presentation, he had no features of inflammatory arthritis but had a high rheumatoid factor titer and low C4 level. Withdrawal of immunosuppression after 6 years resulted in the development of classical rheumatoid arthritis (RA). This case supports previous reports that revealed that vasculitis may predate the development or occur very early in the course of articular RA.

An Uncommon Cause for Colonic Ischemia

We present a case of Polyarteritis Nodosa (PAN) in a hepatitis B negative patient presenting as ischemic colitis with delay in diagnosis leading to renal failure. There have been less than 10 cases reported in literature.A 49 year old male with a history of hypertension with resultant chronic kidney disease Stage 2 and rheumatoid arthritis (on Prednisone) presented to our hospital with generalized malaise, intermittent nausea and vomiting along with loose stools for the past 1 month. He noted a 30lb weight loss over the previous few months. Labs were notable for normocytic anemia, leukocytosis and acute kidney injury ( Creatinine of 3.4 from a baseline of 1.5). Urinalysis showed trace protein, no WBC or RBC with negative urine eosinophils. His renal function improved with hydration, however his gastrointestinal symptoms persisted. Work up revealed no infections and he underwent a colonoscopy which revealed ulcerated mass like lesions in the hepatic, transverse and sigmoid colon. Biopsies revealed ischemic colitis. He underwent an abdominal aortogram with carbon dioxide which showed no evidence of mesenteric stenosis. His renal function underwent progressive rapid decline necessitating a kidney biopsy revealing necrotizing arteritis consistent with a diagnosis of polyarteritis nodosa (PAN). He was started on hemodialysis and immunosuppresion with steroids and cyclosporine. His gastrointestinal symptoms resolved. A repeat follow up colonoscopy revealed normal colonic mucosa. PAN usually presents as ischemic disease in the small bowel and much more rarely in the colon. Colonic presentation can be mistaken for IBD in younger patients and atherosclerotic ischemic colitis in older patients as in this patient. Unlike some other vasculitides such as Wegener granulomatosis, remission can be obtained with PAN if diagnosed early. This patient was started on an immunosuppression regimen, which suppressed recurrence of GI symptoms, however was too late to salvage kidney function. In patients who present with a constellation of laboratory findings in the setting of ischemic colitis a broad differential should be obtained and repeat deep biopsies should be taken to confirm diagnosis of a vasculitis. A delay in diagnosis can lead to life changing consequences and even death.

Attention, la périartérite noueuse existe encore en néphrologie !

Renal involvement of systemic vasculitides is an emergency in nephrology. Although it has become very rare, the diagnosis of polyarteritis nodosa must be considered in some patients. A 70-year-old patient, previously healthy, presented with acute renal failure and malignant hypertension and abundant albuminuria. Subcutaneous nodule, orchitis and mononeuritis appeared subsequently. The search for auto-immunity or viral infection was negative. Markers of thrombotic microangiopathy, present initially, resolved after blood pressure control. After a renal computed tomography with contrast medium injection was considered normal, without any micro-aneurysm, a renal biopsy was performed. It showed vascular lesions and glomerular ischemia. It was complicated by hemorragic shock after 36hours. The diagnosis of periarteritis nodosa was finally made after arterial angiography showed millimetric renal micro-aneurysms. In case of systemic vasculitis with renal involvement, periarteritis nodosa must be part of differential diagnosis, especially in case of malignant hypertension, subcutaneous nodosa and orchitis, and despite albuminuria. This implies the search for micro-aneurysms with arterial angiography, and the contraindication of renal biopsy, associated with a high risk of severe hemorrhage. Periarteritis nodosa still exists in nephrology, even without hepatitis B infection. The association of acute renal failure with medium vessel vasculitis, with negative ANCA, must alert the nephrologist and lead to arterial angiography not to renal biopsy.

CECR1 p.Gly47Arg mutations are not increased in frequency in Turkish Behçet's disease patients compared with healthy controls

Question In Behcet’s disease (BD), vasculitis involving blood vessels of nearly all sizes and types may underlie the diverse tissue and organ involvement. Loss of function mutations in the CECR1 gene (encoding adenosine deaminase 2) have recently been shown to cause a recessive genetic disease, deficiency of adenine deaminase 2 (DADA2). Patients with DADA2 exhibit systemic vasculopathy characterized by intermittent fevers, skin rash, and neurovascular manifestations along with other features that can lead to a diagnosis of polyarteritis nodosa. Patients homozygous for the CECR1 p.Gly47Arg mutation are reported in two nonconsanguineous Turkish families and this mutation is found at low frequency in the Turkish population. We therefore attempted to determine whether some BD cases may be explained by adenosine deaminase 2 deficiency and whether this mutation contributes to BD risk in patients of Turkish ancestry.

Polyarteritis nodosa mimicking a testis tumour: a case report and review of the literature.

A 28-year-old man presented with a seven-day history of testicular pain. Physical examination revealed a mass in the lower pole of the left testis. This mass was a tumour suspect on scrotal ultrasound and MRI. Testicular tumour markers were negative. A radical orchidectomy was performed. Histologically, the diagnosis of polyarteritis nodosa (PAN) was made. Retrospectively, the diagnosis of PAN could have been made earlier. The patient was treated for superficial thrombophlebitis in the months prior to admission. This was considered to be a paraneoplastic phenomenon after radical nephrectomy for a conventional type renal cell carcinoma two years earlier. After the diagnosis of PAN was made on the orchidectomy specimen, the cutaneous lesions were finally recognized as cutaneous PAN. With this knowledge, a simple testicular biopsy could have avoided a radical orchidectomy. A short review of literature on testicular PAN is given.

Polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome: a case report

BackgroundPosterior reversible encephalopathy syndrome is a presentation which is diagnosed clinico-radiologically. The primary aetiological processes leading to posterior reversible encephalopathy syndrome are many, which include autoimmune conditions. Polyarteritis nodosa as an aetiological factor for posterior reversible encephalopathy syndrome is rare. We present a case of polyarteritis nodosa complicated by posterior reversible encephalopathy syndrome.Case presentationA 26-year-old South-Asian female presented with left sided focal seizures with secondary generalization and visual disturbance for 2 days duration. She had a prior history of arthralgia and weight loss with no medically explainable cause for young onset hypertension. Examination revealed a right claw hand with a palpable vasculitic type of rash involving both the palmar surfaces. Symptoms responded to management with anti-hypertensives and anti-epileptics. Whole blood count, iron studies, erythrocyte sedimentation rate and C-reactive protein values portrayed an ongoing chronic inflammatory process. Serological studies such as Anti-nuclear antibody, Anti -double stranded deoxyribonucleic acid, Anti-neutrophil cytoplasmic antibody and Anti-cyclic citrulinated peptide were negative. Magnetic resonance imaging revealed high signal intensity on T2 in both occipital lobes. Skin biopsy of the palm revealed moderate vessel vasculitis. Renal imaging revealed structurally abnormal kidneys with micro aneurysms in the right renal vasculature. Repeat magnetic resonance imaging of the brain two months later showed marked improvement. A diagnosis of polyarteritis nodosa with posterior reversible encephalopathy syndrome was made.ConclusionsPosterior reversible encephalopathy syndrome should not be missed. Investigations for an aetio-pathological cause should be considered including the rarer associations like polyarteritis nodosa.

Polyarteritis nodosa clinically mimicking nonocclusive mesenteric ischemia

Here, we present the case of a 74-year-old Japanese man with segmental intestinal necrosis, which developed after treatment with pulsed methylprednisolone for mononeuritis multiplex. The patient was weakly positive for myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA). Computed tomography and surgical findings were compatible with nonocclusive mesenteric ischemia (NOMI). He underwent small intestinal resection by emergency surgery and an intestinal fistula was made. Pathologically, necrotizing vasculitis with fibrinoid necrosis was present in medium to small-sized arteries, which was equivalent to Arkin's classification II-IV. Most of the arteries had fibrous intimal thickening, which was considered to obstruct the arteries and thus cause segmental intestinal necrosis. A diagnosis of polyarteritis nodosa (PAN) was made, and intravenous cyclophosphamide pulse therapy was added to the therapeutic regimen. This patient was successfully treated with these multidisciplinary therapies and his stoma was finally closed. This is a very rare and indicative case of PAN weakly positive for MPO-ANCA and clinically mimicking NOMI, which occurred even after treatment with pulsed methylprednisolone.

Polyarteritis nodosa: a case presenting with renal mass

An adolescent girl was hospitalized due to fever and abdominal flank pain. A left renal haematoma was detected on magnetic resonance imaging. Renal angiography demonstrated multiple microaneurysms at both hepatic arteries, intrarenal segments of the bilateral renal arteries, and inferior lobar segment of the left pulmonary artery, which is consistent with the diagnosis of polyarteritis nodosa. Vasculitic syndromes should be considered in patients with visceral haemorrhage.

Antiphospholipid syndrome and polyarteritis nodosa: a diagnostic and therapeutic challenge

Antiphospholipid syndrome (APS) can be primary, when it is not associated with another connective tissue disease, or can be secondary, especially associated with systemic lupus erythematosus. In a cohort of 1,000 APS patients, only seven (0.7 %) were associated with systemic vasculitis [1]. In the literature, there are few articles about the association of polyarteritis nodosa with APS comprising a total of 19 cases [2–8]. This study reports two additional cases with this rare condition, APS and polyarteritis nodosa. A 40-year-old Caucasian man was admitted after two epileptic crises. One year before, he reported unintentional weight loss of 47 kg and onset of livedo reticularis. His past medical history included arterial hypertension. Creatinine was 1.3 mg/dl. An arteriography showed multiple small aneurysms in the renal arteries. Antineutrophil cytoplasmic antibody (ANCA) and hepatitis B surface antigen were negative, and hepatitis B core antibody and hepatitis B surface antibody were positive. A diagnosis of polyarteritis nodosa was performed, and he was treated with prednisone 60 mg/day and azathioprine (150 mg/ day). After 5 years, he experienced abrupt ataxia, and magnetic resonance imaging of the brain revealed caudate nucleus and cerebellum infarction. At this occasion, he had positive IgG anticardiolipin ([120GPL) and IgM ([120MPL) antibodies, and aspirin (300 mg/day) was then added to the therapeutical scheme. He experienced complete recovery of this neurological symptom after one month. After 12 weeks, as IgG and IgM anticardiolipin antibodies continued highly positive, APS diagnosis was done. Currently, he is well, asymptomatic, using azathioprine (100 mg/day) and aspirin. The other case is a 43-year-old Caucasian man presented with ulcers on his legs, systemic hypertension and livedo reticularis. Since three years before, he had limb paresthesia in left leg. Electroneuromyography showed mononeuritis multiplex with sensitive and motor nerve fibers involvement in left ulnar nerve, and left posterior tibial and peroneal nerve. The histopathologic features of skin biopsy showed leukocytoclastic vasculitis with perivascular neutrophilic inflammation and necrosis of vessel wall. ANCA was positive. He had a good evolution, with healing of his ulcers. One year later, the patient presented infectious ulcer on hallux, which led to necrosis and amputation. He was treated with antibiotics, prednisone 70 mg/day associated with cyclophosphamide, but these treatments did not demonstrate any efficacy. A new ulcer biopsy was performed and demonstrated leukocytoclastic vasculitis with perivascular neutrophilic inflammation, necrosis of vessel wall and vascular thrombosis. Lupus anticoagulant was positive. APS diagnosis was then performed, and heparin followed by warfarin was started, and he experienced marked improvement of his ulcers. After 12 weeks, lupus anticoagulant continued positive. Nowadays, he is asymptomatic, without ulcers, using azathioprine 75 mg/day and warfarin with INR target of 3–4. C. A. M. Caldas Internal Medicine Department, Centro Universitario do Estado do Para, Universidade Federal do Para, Belem, PA, Brazil

Épididymite aiguë : mode de révélation inhabituel d’une périartérite noueuse

Epididymotesticular manifestations are a very rare mode of revelation of periarteritis nodosa. We report a case of a patient where the diagnosis of periarteritis nodosa was made on the histological analysis of a part of epididymectomy. Through this observation, we will discuss the diagnostic difficulties of periarteritis nodosa.

Renal Artery Aneurysms: Careful Investigation is Critical

We describe a case of a 36-year-old female patient who was investigated for renal artery aneurysms with angiography. Co-existing aneurysms within the mesenteric circulation were also noted, and a diagnosis of polyarteritis nodosa was made.Investigating patients with renal artery aneurysms using magnetic resonance imaging alone may miss cases of polyarteritis nodosa: a serious and potentially treatable condition.

Une vascularite isolée du col et de l’isthme utérins

Isolated lesions of vasculitis are described in different organs, notably female genital tract. Exhaustive clinic and paraclinic exams are necessary to exclude an occult systemic vasculitis. We report a case of vasculitis that was restricted to uterine cervix and isthmus, fortuitously discovered by a 45-years-old woman after hysterectomy. At histological examination, necrotizing vasculitis of small and medium-sized arteries was found, suggesting diagnosis of polyarteritis nodosa. There was no argument for systemic vasculitis.