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Polyarteritis Nodosa

PAN

Polyarteritis nodosa refers to the condition resulting from inflammation of small and medium-sized arteries. It may affect many organs and can be life threatening in some cases.


Presentation

PAN has mulitsystem involvement and progresses within few weeks to months [13]. It may affect a single organ or lead to visceral failure. Symptoms depend on the affected organs

Fever
  • The first case of CPAN reported in the English-language literature may be that of a 7-year-old boy who, in 1870, died of "scarlet fever" at St. Bartholomew Hospital in London.[emedicine.com]
  • A 24-year-old male was admitted to the hospital with complaints of cough, fever and dyspnoea. He was receiving immunosuppressive therapy for polyarteritis nodosa.[ncbi.nlm.nih.gov]
  • Monogenic disorders similar to PAN have been described (familial Mediterranean fever, Adenosine Deaminase-2 deficiency), and cutaneous PAN and single organ vasculitis, discussed.[ncbi.nlm.nih.gov]
  • It is limited to the skin and has a recurrent and chronic course, possibly associated with fever, arthralgia, myalgia and neuropathy, but without visceral involvement.[ncbi.nlm.nih.gov]
Weight Loss
  • CASE PRESENTATION: We report a 42-year-old male Han Chinese who received adalimumab (ADA) alone during his vasculitis relapse, manifesting with weight loss, ankle arthritis, testicular inflammation and new-onset hypertension.[ncbi.nlm.nih.gov]
  • He had symptoms of myalgia and weight loss, was afebrile but had an elevated erythrocyte sedimentation rate and acute-on-chronic renal impairment.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • 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 KEYWORDS: Birmingham vasculitis activity score; Five factor scores; Polyarteritis nodosa;[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
Malaise
  • 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.[ncbi.nlm.nih.gov]
  • Cutaneous polyarteritis nodosa (CPAN) is a chronic, indolent, single organ arteritis that generally presents with lower extremity nodules and/or livedo racemosa, accompanied by malaise and arthralgias.[ncbi.nlm.nih.gov]
  • His symptoms are associated with malaise, fever, and weight loss. He does not have a family history of malignancy and his last colonoscopy was unremarkable. His blood pressure is 155/98 mmHg (his blood pressure is usually 125/74 mmHg).[medbullets.com]
  • Neurological – mononeuropathy multiplex Renal – renovascular hypertension, vascular nephropathy Pediatrics Epidemiology Age – peak is 9-11 years Sex – M Clinical Presentation Tends to be a more limited disorder than in adults Constitutional – fever, malaise[arupconsult.com]
  • Clinical features: Weight loss, malaise, fever are most common symptoms.[ucsfmed.wordpress.com]
Anemia
  • A 73-year-old gentleman who presented with progressive anemia and scant hemoptysis developed acute kidney injury over the course of hospital admission.[ncbi.nlm.nih.gov]
  • […] nonspecific abdominal pain Dermatological – livedo reticularis, skin nodules, skin infections Indications for Testing Unexplained systemic illness with multiple system involvement (renal, neurologic, dermatologic) Laboratory Testing Nonspecific testing CBC – anemia[arupconsult.com]
  • The white blood cell count and platelet count can be elevated, while the red blood count is decreased ( anemia ). Hepatitis B virus (testing for either antigen or antibody) can be found in 10%-20% of patients with polyarteritis nodosa.[medicinenet.com]
  • Common side effects of cyclophosphamide include alopecia (40-60%), anemia, leukopenia, hemorrhagic cystitis, nausea, vomiting, sterility.[clinicaladvisor.com]
Raynaud Phenomenon
  • Skin: About 40% of patients develop a rash, bruising, nodules and Raynaud phenomenon. Cardiovascular system: Cardiac disease affects 35% of patients with PAN.[myvmc.com]
  • Other systemic manifestations include cutaneous involvement (eg, subcutaneous nodules), purpura or Raynaud phenomenon, coronary arteritis, pericarditis, and hematologic abnormalities. CNS disease associated with PAN is rare.[aao.org]
  • A 41-year-old white man presented with a 7-year history of erythematous papules, livedo reticularis, swelling, myalgias, arthralgias, Raynaud phenomenon, and very large, painful and progressive ulcers on the lower part of both legs ( Figure 1 ).[doi.org]
Abdominal Pain
  • Symptoms on admission included a generalised rash, colicky abdominal pain, hypertension, proteinuria and fresh rectal bleeding. Abdominal pain and distension worsened and serial ultrasounds suggested bowel ischaemia.[ncbi.nlm.nih.gov]
  • Our case, together with other reports, suggests that PAN should be considered in patients presenting with right upper abdominal pain.[ncbi.nlm.nih.gov]
  • Abstract A 52-year-old woman presented with severe acute right upper quadrant abdominal pain and signs of intra-abdominal haemorrhage.[ncbi.nlm.nih.gov]
  • When his fever, abdominal pain, and renal dysfunction progressed, he was admitted to our hospital. He was diagnosed with polyarthritis nodosa. His renal function rapidly deteriorated despite immunosuppressive therapy.[ncbi.nlm.nih.gov]
  • Polyarteritis nodosa mnemonic It usually presents with vague symptoms like myalgias, arthralgias, abdominal pain, fatigue. Renal involvement manifests as hypertension, renal insufficiency.[medicowesome.com]
Loss of Appetite
  • They can include skin rashes and ulcers, muscle and joint aches, fever, fatigue, weakness, abdominal pain, and loss of appetite. Nerve pain and numbness also can be associated with PAN.[kidshealth.org]
  • Patients who have PAN may feel generally ill and fatigued, have fevers, or have loss of appetite and weight.[my.clevelandclinic.org]
  • Constitutional symptoms are seen in up to 90% of affected individuals and include fever, fatigue, weakness, loss of appetite, and unintentional weight loss. Muscle and joint aches are common.[en.wikipedia.org]
Acute Abdomen
  • Here we report a case of a young Chinese patient who presented with an acute abdomen requiring surgery and made good recovery post operatively. He remains free of symptoms while on steroid therapy. 2017 BMJ Publishing Group Ltd.[ncbi.nlm.nih.gov]
  • A localized type of PN in the gallbladder has been rarely reported with or without signs of autoimmune diseases or acute abdomen [ 8 - 12 ].[alliedacademies.org]
  • Perforation of the gallbladder with acute abdomen may occur. Vasculitis of medium-sized mesenteric arteries causes abdominal pain, nausea, vomiting (with or without bloody diarrhea), malabsorption, intestinal perforation, and acute abdomen.[msdmanuals.com]
  • abdomen is associated with a 50 percent mortality rate. 1 Prompt treatment with corticosteroids and cyclophosphamide improves 5- year survival to 80 percent, 5 making early recognition paramount.[appliedradiology.com]
Blood in Stool
  • When the condition affects gastrointestinal tract, abdominal pain and blood in stools are common symptoms. Shortness of breath and chest pain is common when inflammation affects heart.[symptoma.com]
Hypertension
  • Classifying hypertension as ‘primary’ or ‘secondary’ depends on the underlying mechanism.[ncbi.nlm.nih.gov]
  • A 27-year-old man, with known diastolic hypertension diagnosed 2 years earlier, was admitted with chronic urinary obstruction complicated by hydronephrosis.[ncbi.nlm.nih.gov]
  • We report a case of a 75-year-old male presented with solely hypertension and mild renal function impairment as a consequence of diffuse renal necrotising vasculitis with occlusive but non-stenotic lesions associated with PAN.[ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
  • CASE PRESENTATION: We report a 42-year-old male Han Chinese who received adalimumab (ADA) alone during his vasculitis relapse, manifesting with weight loss, ankle arthritis, testicular inflammation and new-onset hypertension.[ncbi.nlm.nih.gov]
Vascular Disease
  • disease w polyarteritis Polyneuropathy in collagen vascular disease with polyarteritis Polyneuropathy in polyarteritis nodosa Clinical Information Form of necrotizing vasculitis involving small- and medium-sized arteries; signs and symptoms result from[icd9data.com]
  • Obstructive intestinal symptoms are described but usually resolve with treatment of the underlying vascular disease.[ncbi.nlm.nih.gov]
  • The most frequent aetiologies are renal tumours and vascular diseases. The authors report a case of a patient admitted with hypovolaemic shock due to a retroperitoneal haematoma in the context of polyarteritis nodosa (PAN).[ncbi.nlm.nih.gov]
  • - A dictionary of medical eponyms Related people Adolf Kussmaul Rudolf Robert Maier A narcotising vascular disease involving the small and medium sized muscular arteries.[whonamedit.com]
  • Abstract Periarteritis nodosa is an obliterative, inflammatory, vascular disease involving the small arteries and arterioles.[annals.org]
Tachycardia
  • She was in septic shock with tachycardia, tachypnea, and hypotension.[surgicalcasereports.springeropen.com]
  • Protein in urine Gastrointestinal System: GI bleeding Abdominal pain Nausea/vomiting Constipation Melena Hematochezia Possible hemmorrhage or perforation (rare) Musculoskeletal System: Arthralgia Myalgia Arthritis (less common) Cardiac System: Chest pain Tachycardia[physio-pedia.com]
Hypotension
Myalgia
  • It is limited to the skin and has a recurrent and chronic course, possibly associated with fever, arthralgia, myalgia and neuropathy, but without visceral involvement.[ncbi.nlm.nih.gov]
  • At study entry, mononeuritis multiplex was less frequent in systemic cPAN than systemic aPAN (P   0.04), and purpura and myalgias were less frequent in cutaneous cPAN than cutaneous aPAN (P CONCLUSION: Systemic PAN is equally severe in children and adults[ncbi.nlm.nih.gov]
  • He had symptoms of myalgia and weight loss, was afebrile but had an elevated erythrocyte sedimentation rate and acute-on-chronic renal impairment.[ncbi.nlm.nih.gov]
  • Myalgias, weakness or leg tenderness : diffuse myalgia (excluding shoulders and hip girdle) or weakness of muscles or tenderness of leg muscles.[rheumtutor.com]
  • Herein is described the case of a 14-year-old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation.[ncbi.nlm.nih.gov]
Arthralgia
  • It is limited to the skin and has a recurrent and chronic course, possibly associated with fever, arthralgia, myalgia and neuropathy, but without visceral involvement.[ncbi.nlm.nih.gov]
  • Abstract We report an F-FDG linear uptake of the right popliteal artery in a 25-year-old man who underwent a PET/CT scan for unexplained chronic asthenia associated with fever symptoms, arthralgia, and subcutaneous nodes, in favor of vasculitis.[ncbi.nlm.nih.gov]
  • Cutaneous polyarteritis nodosa (CPAN) is a chronic, indolent, single organ arteritis that generally presents with lower extremity nodules and/or livedo racemosa, accompanied by malaise and arthralgias.[ncbi.nlm.nih.gov]
  • Arthralgia/arthritis and skin involvement were more common in children (86.7% vs. 59.1%; 93.3% vs. 72.7%, respectively) while renal and neurologic involvement were more frequently observed in adult patients (50% vs. 20%; 59.1% vs. 40%, respectively) ([ncbi.nlm.nih.gov]
  • 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.[ncbi.nlm.nih.gov]
Purpura
  • Abstract We describe the case of a previously healthy 12-year-old boy admitted to a tertiary paediatric centre with the clinical diagnosis of Henoch-Schonlein purpura (HSP).[ncbi.nlm.nih.gov]
  • Small-vessel vasculitis includes Wegener granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis (microscopic polyarteritis), Henoch-Schönlein purpura, essential cryoglobulinemic purpura, and cutaneous leukocytoclastic angiitis.[emedicine.com]
  • At study entry, mononeuritis multiplex was less frequent in systemic cPAN than systemic aPAN (P   0.04), and purpura and myalgias were less frequent in cutaneous cPAN than cutaneous aPAN (P CONCLUSION: Systemic PAN is equally severe in children and adults[ncbi.nlm.nih.gov]
  • PANpable: Palpable purpura is seen in PAN. That's all! -IkaN[medicowesome.com]
Palpable Purpura
  • Feels like there is an outbreak of vasculitis recently at the SFVA with a few morning reports on palpable purpura and it’s characteristic finding on pathology: leukocytoclastic vasculitis (LCV)![ucsfmed.wordpress.com]
  • PANpable: Palpable purpura is seen in PAN. That's all! -IkaN[medicowesome.com]
  • Microscopic polyangiitis commonly manifests with palpable purpura, alveolar hemorrhage, symptomatic cardiac involvement but is less likely to have hypertension, neuropathy, or hepatitis B.[rheumaknowledgy.com]
  • Livedo vascular pattern typical of cutaneous polyarteritis nodosa Expected results of diagnostic studies In the case of palpable purpura the cutaneous biopsy will show leukocytoclastic vasculitis.[dermatologyadvisor.com]
  • Skin manifestations of PAN include palpable purpura and livedo reticularis in some individuals. Abdominal pain may also be seen. Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness (peripheral neuropathy).[en.wikipedia.org]
Skin Ulcer
  • Other features include livedo reticularis, skin ulcers and tender indurated plaques. CONCLUSION: We report a 51-year old woman with a primarily livedo reticularis presentation of cutaneous polyarteritis nodosa without a nodular component.[ncbi.nlm.nih.gov]
  • Cutaneous: Livedo reticularis, skin ulcers, tender erythematous nodules, bullous or vesicular eruptions, infarction and gangrene of fingers or toes, or a combination may occur.[msdmanuals.com]
  • ulcers livedo reticularis purpura renal glomerulonephritis hematuria new onset hypertension pulmonary typically spares lungs, though may involve bronchial arteries gastrointestinal abdominal pain rectal bleeding Imaging Angiography indication considered[medbullets.com]
  • Case 7-2007: a 59 year-old woman with diabetic renal disease and nonhealing skin ulcers. N Engl J Med. 2007; 356:1049–57 PubMed CrossRef Google Scholar Callen JP. Livedoid vasculopathy: what it is and how the patient should be evaluated and treated.[link.springer.com]
  • The symptoms of polyarteritis nodosa vary from one patient to another and include fever, fatigue, weight loss, loss of appetite, joint and muscle pain, skin outbreaks, swollen legs, skin ulcers and/or nodules.[therapeutique-dermatologique.org]
Cutaneous Manifestation
  • The most frequent cutaneous manifestations were ulcers, livedo racemosa, subcutaneous nodules, atrophie blanche lesions and purpuras; with lower limb involvement in all cases, however other areas were also involved.[ncbi.nlm.nih.gov]
  • Francois Chasset and Camille Francès, Cutaneous Manifestations of Medium- and Large-Vessel Vasculitis, Clinical Reviews in Allergy & Immunology, 10.1007/s12016-017-8612-9, (2017).[doi.org]
  • Although it is distinct from systemic polyarteritris nodosa in that it lacks significant internal organ involvement, extra-cutaneous manifestations may be evident.[bjmp.org]
  • “Comparison of cutaneous manifestations in systemic polyarteritis nodosa and microscopic polyangiitis”. Br J Dermatol. vol. 159. 2008. pp. 615-620.[dermatologyadvisor.com]
Urticaria
  • The second case is a man in his 20s with recurrent urticaria. In both patients, plasma D-dimer levels increased with clinical evidence of disease activity and decreased with treatment and resolution of the disease flare.[ncbi.nlm.nih.gov]
Hematuria
  • Herein, we describe a patient who was initially diagnosed with lupus nephritis based on her clinical presentation, proteinuria, hematuria, positive anti-nuclear antibody, and a kidney biopsy.[ncbi.nlm.nih.gov]
  • PRESENTATION: We describe the case of a 51-year-old woman who presented with a 4-day-history of intense pain in her left flank, hypertension, fever, microscopic hematuria, and acute renal failure.[ncbi.nlm.nih.gov]
  • Unexplained systemic illness with multiple system involvement (renal, neurologic, dermatologic) Laboratory Testing Nonspecific testing – helpful in excluding other diagnoses or identifying organ dysfunction CBC – rule out infection Urinalysis (UA) – hematuria[arupconsult.com]
  • […] adult males Spares the arterioles, capillaries, venules and glomeruli Associated with hepatitis B antigenemia Signs and symptoms Abdominal pain Systemic hypertension Anorexia and weight loss Abdominal distention Hematemesis, melena Jaundice Painless hematuria[learningradiology.com]
  • Clinically, HTN, sub-nephrotic range proteinuria, mild hematuria are commonly seen. RBC casts are rare in PAN and should make you consider one of the ANCA associated vasculidities or SLE.[ucsfmed.wordpress.com]
Stroke
  • Copyright 2018 National Stroke Association. Published by Elsevier Inc. All rights reserved. KEYWORDS: Polyarteritis nodosa (PAN); cerebral aneurysms; ischemic stroke; lateral medullary infarct; posterior inferior cerebellar artery (PICA); vasculitis[ncbi.nlm.nih.gov]
  • This report shows that ischemic strokes may occur as a result of minocycline-induced vasculitis.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: DADA2 accounts for paediatric patients diagnosed with PAN-like disease and strokes and might explain an unrecognised condition in patients followed by adult rheumatologist.[ncbi.nlm.nih.gov]
  • RECENT FINDINGS: DADA2 was first described in patients with systemic inflammation, mild immune deficiency, and vasculopathy manifested as recurrent stroke or polyarteritis nodosa (PAN).[ncbi.nlm.nih.gov]
  • Death is often a consequence of kidney failure, myocardial infarction, or stroke. PAN may affect nearly every organ system and thus it can present with a broad array of signs and symptoms.[en.wikipedia.org]
Seizure
  • CASE PRESENTATION: A 26-year-old South-Asian female presented with left sided focal seizures with secondary generalization and visual disturbance for 2 days duration.[ncbi.nlm.nih.gov]
  • Serious complications can include strokes , seizures , and kidney failure. Keep in Mind While polyarteritis nodosa can be serious, many people with the condition do very well.[kidshealth.org]
  • Serious complications can include strokes, seizures, and kidney failure. Keep in Mind While polyarteritis nodosa can be serious, many people with the condition do very well.[kidshealth.org]
  • Nervous system:Decreased alertness Peripheral neuropathy Muscle pain, numbness or tingling Tingling, numbness and pain in feet, legs, hands and arms Seizures Slurred speech Kidneys: Fluid retention Hypertension Kidney failure Skin: Hives Nodules Rashes[foundationforpn.org]
  • Damage to the nervous system may cause strokes or seizures. No specific lab tests are available to diagnose polyarteritis nodosa. There are a number of disorders that have features similar to polyarthritis nodosa. These are known as "mimics."[nlm.nih.gov]
Peripheral Neuropathy
  • There maybe nausea, vomiting and abdominal pain due to GI involvement (Untreated patients may die of bowel infarcts, perforations) Peripheral neuropathy, rash, purpura, CHF, CVA, etc are other manifestations of the disease.[medicowesome.com]
  • neuropathy Tender subcutaneous nodules Gangrene of fingers and toes Kidney (most frequently affected): 85% Multiple intrarenal aneurysms Multiple aneurysms of renal vessels in Polyarteritis Aneurysms may thrombose and disappear Appear in new locations[learningradiology.com]
  • Nervous system:Decreased alertness Peripheral neuropathy Muscle pain, numbness or tingling Tingling, numbness and pain in feet, legs, hands and arms Seizures Slurred speech Kidneys: Fluid retention Hypertension Kidney failure Skin: Hives Nodules Rashes[foundationforpn.org]
  • The patient should also be monitored for: Headache Dementia Psychosis Encephalopathy Cerebrovascular events Peripheral neuropathy Prognosis Almost half will die within three months of diagnosis if untreated, usually secondary to chronic kidney disease[patient.info]
  • Peripheral nervous system involvement – about 60% of the patients present peripheral neuropathy. Mononeuritis multiplex, distal polyneuropathy, and cutaneous neuropathy are also reported.[symptoma.com]
Tingling
  • Nervous system:Decreased alertness Peripheral neuropathy Muscle pain, numbness or tingling Tingling, numbness and pain in feet, legs, hands and arms Seizures Slurred speech Kidneys: Fluid retention Hypertension Kidney failure Skin: Hives Nodules Rashes[foundationforpn.org]
  • The most common symptoms are: Blood in the urine Fever High blood pressure High levels of protein in the urine Numbness, tingling or weakness of the hands and feet Pain in the joints, especially the large ones Skin rash with raised reddish-purple patches[cedars-sinai.edu]
  • Common problems include: General: Fever Weight loss Joint and muscle pain Skin: Rashes Open sores Small bumps under the skin Bruises Death of skin tissue Nervous system: Tingling, burning, pain, or numbness in your feet, hands, legs, arms, and face Decreased[cancercarewny.com]
  • If the peripheral nerves are affected, patients may notice muscle weakness, tingling sensations or burning- or electric shock-type pains in the hands and/or feet.[therapeutique-dermatologique.org]
  • Nerve injury in the arms or legs, which may cause numbness, tingling, or loss of sensation and movement caused by injury to one specific nerve bundle. In the brain, damage can trigger strokes, seizures or sudden alterations in brain function.[drugs.com]
Headache
  • Seven years later the left unruptured aneurysm enlarged, causing a sudden severe headache and a cavernous sinus syndrome.[ncbi.nlm.nih.gov]
  • Neurologic manifestations resolved with treatment including corticosteroids and cyclophosphamide; however, persistent fevers, rash, and headaches interfered with attempts at a corticosteroid taper.[pediatrics.aappublications.org]
  • Some authors state that the incidence is falling, others that it is rising but this may be due to increased recognition. [ 8 ] Presentation Diagnosis is not easy, as PAN often presents in a vague manner with symptoms including fever, weight loss, headache[patient.info]
  • The commonest symptoms can be fever, weight loss, abdominal pain, high blood pressure ( hypertension ), heart trouble, nerve pain (neuralgia) and numbness in the limbs, inflamed joints, muscle pain, headaches, kidney failure and even bleeding from the[netdoctor.co.uk]

Workup

PAN is an uncommon disease with many clinical manifestations that make diagnosis difficult. Many diseases that affect the vascular integrity or result in vascular occlusion may present PAN-like symptoms. Certain drugs are also known to cause manifestations similar to that of vasculitis. Thus, it is important to distinguish the different causes for better treatment and prognosis.
Among the different imaging techniques for diagnosis, angiography is suggested when the affected tissue is inaccessible. This is preferred for aneurysms and stenosis in medium-sized blood vessels. It is also useful in case of gastrointestinal involvement, particularly when liver and kidney functions are affected. Microaneurysms in small arteries can be revealed using arteriograms. CT scan and MRI are other imaging methods that are used to check varied manifestations of PAN. These imaging techniques may be used in case of nonspecific presentations like thickening in the wall of intestine, obstruction in bowel movement, and thickening of mucosal folds. MRI is also suggested in CNS involvement where intracranial hemorrhage is suspected. Electromyography facilitates the detection of peripheral neuropathy, one of the most common complication associated with PAN.
When the affected tissue is accessible, biopsy is recommended. This includes skin, skeletal muscle and testes. Biopsy of muscles and arteries enables identification of inflammation. Histology of the affected tissue will show the presence of cellular infiltration within the wall of the vessel. In nerve cells, axonal degeneration is seen with loss of fibrous tissue. Laboratory studies are helpful in finding the systemic nature of PAN. Some of the common finding include elevated ESR, hepatitis B surface antigen, leukocytosis, proteinuria, elevated levels of liver enzymes and hypergammaglobulinemia. HBV-related PAN have increased levels of cryoglobulins.
Thus for a definitive diagnosis of the condition there should be one of the following findings: 1) histological proof of vasculitis, 2) positive serology of anti-neutrophil cytoplasmic antibodies (ANCA), 3) angiography or biopsy findings suggestive of vasculitis, and 4) elevated ESR [14].

Pericardial Effusion
  • effusion Pleural effusion Discoid atelectasis Nodules which may cavitate Patchy consolidation Liver: affected 66% of cases Treatment Corticosteroids 50% 5 year survival Bockus, Henry L.[learningradiology.com]
Thrombocytosis
  • Diagnostic Testing: Nonspecific inflammatory markers include elevated ESR or CRP, leukocytosis, anemia of chronic disease, and thrombocytosis.[rheumaknowledgy.com]
  • Patients may have thrombocytosis, markedly elevated ESR, anemia caused by blood loss or renal failure, hypoalbuminemia, and elevated serum immunoglobulins. AST and ALT are often mildly elevated. Testing for hepatitis B and C should be done.[msdmanuals.com]
Elevated Sedimentation Rate
  • Ordinary blood tests often show elevated sedimentation rates (SR) and anaemia as signs of general inflammation. Kidney function may be affected, which can manifest as blood and protein in the urine.[socialstyrelsen.se]
Leukocytes Increased
  • Despite ongoing methotrexate treatment, the number of leukocytes increased after 18 months and was accompanied by worsening of the clinical picture ( Figure 2 ).[doi.org]
Eosinophils Increased
  • Eosinophils increased focally in some parts of the arteries. Among the infiltrating lymphocytes, CD8 were more predominant than CD4, and they had infiltrated all the arterial walls ( Fig. 4D ).[alliedacademies.org]

Treatment

The main component of treatment for PAN is corticosteroids. If any of the major organs are involved or if the patient is steroid refractory, cyclophosphamide is also added to the treatment. The normal course suggested is high-dose of oral prednisone at 1mg/kg/day. Once the symptoms improve and ESR normalize, prednisone dose can be lowered gradually over a period of time. Those who are receiving steroid treatment for a long-term should be given calcium and vitamin D supplementation to overcome the risk of osteoporosis.
For those with HBV-related PAN, corticosteroids are recommended with antivirals and plasmapheresis. Antiviral medication is started after steroid withdrawal. Along with antivirals, plasma exchange is recommended 3 times a week for 3 weeks. This is gradually reduced and can be stopped once seroconversion occurs. The same treatment modality is being followed for PAN related to HCV infection. For cutaneous involvement in PAN, agents like NSAIDs, colchicine, and dapone are used. In severe cases of skin involvement, steroids and additional cyclophosphamide are recommended. Those with PAN induced by streptococcal infection, penicillin is recommended. These patients may need long-tern prophylaxis and this is particularly true of children. Further consultations depend on the organ affected.
Patients with PAN should be monitored carefully to look out for relapse or symptoms of a new organ being affected. Dosage of cyclophosphamide should be adjusted based on immunosuppression.

Prognosis

Prognosis of PAN is related to the amount of damage to the organs. The chances of mortality increases with factors including renal insufficiency, proteinuria, gastrointestinal complications, cardiomyopathy, and involvement of central nervous system [11]. A prospective study on 342 patients showed that when none of the factors are present, the mortality rate is 11.9% in five years. If left untreated, the survival rate in 5 years is 13%. But treatment with corticosteroids may improve the survival rate which might go up to 50-60%. In general, relapse of PAN is rare. Worst prognosis is seen in the case of central nervous system involvement [12]. Those who have abdominal syndromes tend to have poor prognosis. If there is no systemic involvement, the outcome is better. It is uncommon for patients to have permanent morbidity. Death occurs with untreated and serious vasculitis, as a result of complications that arise from immunosuppression, myocardial infarction and stroke.

Etiology

The actual cause of PAN is still elusive. There are some cases in which infections, particularly viral, are involved in the development of this condition. There is a strong relationship between hepatitis B infection and development of PAN. It was one of the most common causes of this condition. Vasculitis may occur at any stage but is usually seen within 6 months of HBV infection, irrespective of its course [4]. PAN linked to hepatitis infection is induced by immune complex. As the use of hepatitis vaccines increased, the incidence of HBV-related PAN decreased considerably.
Although it is not a very important etiological factor, hepatitis C infection is also found to be related to PAN in some cases. Other viral infections including HIV, herpes zoster, and herpesvirus 68 are possibly involved in the development of this vasculitis. PAN is also known to be associated with some cancers, rheumatic diseases and blood diseases. There is an increased association of PAN with rheumatoid arthritis. Group A streptococcal infections are known to cause a flare-up of this disease, particularly in children. Many other infectious organisms cause PAN-like diseases which include the species of Klebsiella, Pseudomonas, and Yersinia [5]. Exposure to Isotretinoin is reported to be related to the occurrence of vasculitis [6]. PAN is also found to occur at an increased frequency with ulcerative colitis which is probably due to exposure to immune complexes or altered permeability of the gut. A study reports increased incidence of this vasculitis with familial Mediterranean fever [7].

Epidemiology

Reports of incidence of this disease varies in different countries. It is relatively uncommon, with about 1.6 cases in a million in Sweden. In England the estimated incidence rate is 4.6 cases in one million, while in U.S it is 3-4.5 cases in comparable populations. Among adults, about 30.7 cases in a million are reported in Paris [8]. It occurs in all age groups, from children to adults. The incidence of PAN is more among 10 year old children, while among adults the most commonly affected age group is 40 to 65 years [9]. All ethnic groups are affected by this condition but is more prevalent in regions where hepatitis B infection is endemic. Studies report that PAN is found to be more prevalent among men when compared to women.

Sex distribution
Age distribution

Pathophysiology

Vascular lesions characteristic of PAN are more commonly found in the bifurcation point and branching regions of arteries. Inflammation affects the entire wall with the laminae often destroyed and replaced with a fibrin-like, amorphous eosinophilic material. Inflammation of the vessel wall is characterized by necrosis of media with cellular infiltration, particularly by neutrophils, lymphocytes and eosinophils. Aneurysms and thrombi may occur at the site of lesion as walls are weak. Aneurysms may lead to hemorrhage. With the progression of lesions on vessels, intimal may proliferate into the lesion leading to ischemia or infarction [10]. Necrotizing vasculitis may co-exist with a healed lesion in different tissues. This co-existence may also be noted in different parts of the same tissue. Lesions are found in medium-sized arteries and are rare in large and small blood vessels.

Prevention

A definitive method to prevent PAN is not known yet. Prompt treatment and close monitoring are the best ways to avoid complications and help remission.

Summary

Polyarteritis nodosa or PAN, is a rare form of vasculitis that affects small and medium-sized arteries. Inflammation of the blood vessels may affect skin and many other organs including kidney, gut and nerves [1]. This condition, first described by Kussumaul and Maier, may develop in any age group. It is found to occur in association with infections like hepatitis B and streptococcal infections [2]. If the condition affects internal organs significantly, it may be life threatening and immunosuppression may be needed. Cases where inflammation is restricted to skin are mild and need only minimal therapy. Based on the size of the blood vessel affected, PAN is subdivided into two groups: 1) Classical PAN, when it affects medium-sized blood vessels and 2) Microscopic polyangiitis, when inflammation affects the small-sized vessels [3]. PAN is a progressive disease and may be sub-acute, acute or chronic.

Patient Information

Polyarteritis nodosa or PAN is a rare form of autoimmune disease characterized by inflammation of small and medium-sized arteries. Inflammation of the blood vessels may affect different organs including intestine, heart, kidney and nerves, and may be life threatening in certain cases. It is found to occur in all age groups but it is more common among men than in women. The actual cause of PAN is not yet known. It may occur as a part of many viral and bacterial infections, malignancies and other diseases. It is more commonly seen in people with hepatitis B infection.
Since many organs are affected by the condition, a variety of symptoms may be presented in PAN. Some of the common nonspecific symptoms include fever, muscle pain, tiredness, and loss of appetite. Symptom depend on the organ affected. Skin sores, ulcers, and nodules are seen when skin is affected. When the condition affects gastrointestinal tract, abdominal pain and blood in stools are common symptoms. Shortness of breath and chest pain is common when inflammation affects heart. Inflammation of arteries in kidneys may cause high blood pressure. When nerves are affected, it may result in stroke, seizure, and loss of strength.
A variety of sources are used for definitive diagnosis of PAN. A complete medical history helps in checking for presence of symptoms of PAN. Physical examination helps to identify the organ affected by the disease, and also to rule out the possibility of other diseases that mimic symptoms of PAN. Blood tests are also used to identify the affected organ or site of inflammation. Imaging techniques like CT scan and MRI are useful in identifying the site of vasculitis. A definitive diagnosis can be done with angiogram and biopsy of affected tissue sample.
The cornerstone for the treatment of PAN is corticosteroids and medications that suppress immune system. This helps to improve the symptoms caused by inflammation of the blood vessel. Once the symptoms improve, dosage of corticosteroids can be reduced gradually and then stopped completely. The treatment duration and dose varies with the severity of the condition. PAN related to hepatitis and other infections are treated similarly but with an addition of antiviral medications. Outcome of this condition depends on the severity and the organ involved. But studies show that over 80% of the people survive the effects of PAN with early treatment. Prompt treatment and close monitoring help in remission.

References

Article

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Last updated: 2018-06-22 02:25