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Secondary Polyarteritis nodosa

Secondary PAN


Presentation

  • Patients typically present with systemic symptoms. Obstructive intestinal symptoms are described but usually resolve with treatment of the underlying vascular disease.[ncbi.nlm.nih.gov]
  • A 20-year-old boy presented to the emergency department with sudden onset severe left flank pain for a week and no associated vomiting or urinary symptoms.[indianjurol.com]
  • Introduction Polyarteritis nodosa (PAN) presents as a necrotizing vasculitis that affects medium-sized arteries.[frontiersin.org]
  • The diagnosis 'systemic vasculitis' covers a wide spectrum of diseases that often overlap and not always present with a clear distinction.[afrjpaedsurg.org]
Pseudotumor
  • […] include: Headaches due to nasal and paranasal sinus disease; Headaches due to vascular disorders; Headaches and brain tumors; Low cerebrospinal fluid pressure syndromes; Painful ophthalmologic disorders and eye pain; Idiopathic intracranial hypertension (pseudotumor[books.google.com]
Spontaneous Hemorrhage
  • They reported five cases of bilateral spontaneous hemorrhage and all of these were secondary to PAN. [1] PAN is a multi-system necrotizing vasculitis that involves small and medium sized vessels.[indianjurol.com]
Failure to Thrive
  • Fred Ferri’s popular "5 books in 1" format provides quick guidance on menorrhagia, Failure to Thrive (FTT), Cogan’s syndrome, and much more.[books.google.com]
Abdominal Distension
  • On physical examination, no abdominal distension was noted. The initial echocardiogram showed right coronary artery aneurysm and left ventricular thrombus. Gastric biopsies yielded acute gastritis with no other diagnostic abnormalities.[afrjpaedsurg.org]
Bruxism
  • […] headaches; The neck and headaches; Drug-induced headaches; Trigeminal neuralgia and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and headaches; Post-traumatic headaches; Metabolic headaches; Temporomandibular joint disorders, bruxism[books.google.com]
Macula
  • (A) Right eye with normal appearance of the optic nerve and macula. (B) Left eye with optic nerve hyperemia, swollen, retinal folds, dilated veins, and arteries. (C) Right eye with normal appearance of the optic nerve and macula.[frontiersin.org]
Eye Pain
  • pain; Idiopathic intracranial hypertension (pseudotumor cerebri); Cough, exertional, and sex headaches; The neck and headaches; Drug-induced headaches; Trigeminal neuralgia and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and[books.google.com]
Trigeminal Neuralgia
  • neuralgia and glossopharyngeal neuralgia; Headaches and vasculitis; Tonsilar ectopia and headaches; Post-traumatic headaches; Metabolic headaches; Temporomandibular joint disorders, bruxism, and headaches.[books.google.com]

Workup

  • (See Presentation, DDx, and Workup .) Stages PAN is divided into subacute, acute, and chronic stages.[emedicine.medscape.com]

Treatment

  • Obstructive intestinal symptoms are described but usually resolve with treatment of the underlying vascular disease.[ncbi.nlm.nih.gov]
  • In severe cases of PAN, mortality remains high despite the appropriate treatment (4–22%) ( 34 ), since treatment-related toxicities are common.[frontiersin.org]
  • Take advantage of expanded coverage of small molecule treatment, biologics, biomarkers, epigenetics, biosimilars, and cell-based therapies.[books.google.de]

Prognosis

  • Unfortunately, a prompt diagnosis is of extreme importance, since the sooner an appropriate treatment is initiated, the better the prognosis and the less probability of permanent visual damage.[frontiersin.org]
  • Prognosis is better if disease remission is achieved within 18 months after diagnosis. Relapses are less common than in other vasculitic disorders.[merckmanuals.com]
  • Gastrointestinal symptoms occur in 14-65% of patients and postprandial abdominal pain from ischaemia is the most common symptom. [ 9 ] Bowel necrosis and perforation are associated with a poor prognosis.[patient.info]
  • Surgery performed for cholecystitis or appendicitis does not appear to worsen prognosis in the same way. The prognosis is better in patients with cutaneous PAN without systemic involvement. This disease is benign but tends to relapse.[emedicine.medscape.com]
  • Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. Br J Rheumatol. 1997 ; 36(12) : 1290 –1297. Perez VL, Chavala SH, Ahmed M, et al.[aao.org]

Etiology

  • The secondary headaches include a diverse and fascinating array of etiologies which can mimic primary headache disorders ranging from the rare to the mundane and from the well established to the highly controversial.[books.google.com]
  • Additionally, in 1931, Lindberg first recognized cutaneous polyarteritis nodosa (cPAN), a rare form of vasculitis of unknown etiology limited to skin, affecting small‐to‐medium‐sized arteries.[deepdyve.com]
  • Polyarteritis nodosa was due to HBV infection, but the etiology of MN was uncertain, as it has rarely been described in PAN. Proteinuria responded to nucleoside analogue therapy.[ncbi.nlm.nih.gov]
  • Etiology of spontaneous perirenal hemorrhage: A meta-analysis. J Urol 2002;167:1593-6. 2. Zapzalka DM, Thompson HA, Borowsky SS, Coleman-Steenson CC, Mahowald ML, O'Connell KJ.[indianjurol.com]
  • A recent decrease in HBV incidence has been associated with a decrease of new cases of PAN ( 5 ), which suggests that the etiology of PAN may be linked to HBV infection.[frontiersin.org]

Epidemiology

  • […] syndrome (SS), by far the most common variant (80–90% of cases), but pharmacological, gestational or paraneoplastic types of SS have been described, representing the hematologic neoplasms the 85% of the latter group, which has specific clinical and epidemiological[deepdyve.com]
  • Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies.[frontiersin.org]
  • Although progress to classical PAN at a later stage has been reported, generally it is thought to be unlikely. [ 5 ] Epidemiology PAN affects approximately 3.1 per 100,000 people per year. [ 6 ] It is seen in all ethnic groups and appears to be present[patient.info]
  • […] gastrointestinal bleeding, infection, myocardial infarction, and/or kidney failure. [15] In case of remission, about 60% experience relapse within five years. [16] In cases caused by hepatitis B virus, however, recurrence rate is only around 6%. [17] Epidemiology[en.wikipedia.org]
  • Hepatitis B-associated polyarteritis nodosa in Alaskan Eskimos: clinical and epidemiologic features and long-term follow-up. Hepatology. 1989 Jan. 9(1):97-101. [Medline]. Selga D, Mohammad A, Sturfelt G, Segelmark M.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology Polyarteritis nodosa (PAN) spares large vessels (the aorta and its major branches), the smallest vessels (capillaries and small arterioles), and the venous system. [7] Vascular lesions affect medium-sized muscular arteries and occur mainly[emedicine.medscape.com]

Prevention

  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • […] exam CURRENT PHARMACY TABLES that highlight indications, toxic effects, and drug interactions sections that present TYPICAL INDICATION CLINICAL SCENARIOS EXTENSIVELY EDITED CHAPTERS on cardiology, pulmonology, nephrology, critical care, geriatrics, preventive[books.google.de]
  • Prevention Avoidance of hepatitis B (by lifestyle choices and hepatitis B immunisation) may reduce the incidence of hepatitis B-associated PAN but it will not eradicate PAN altogether.[patient.info]
  • Treatment with corticosteroids, sometimes with cytotoxic immunosuppressants (mainly cyclophosphamide ), was often effective in the short term but did not prevent relapses and complications (eg, chronic hepatitis, cirrhosis) due to persistence of the hepatitis[merckmanuals.com]

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