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Polyarteritis nodosa

Polyarteritis nodosa (or periarteritis nodosa) is a serious blood vessel disease. Small and medium-sized arteries become swollen and damaged when they are attacked by rogue immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier disease. more...

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Causes and risk factors

Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that carry oxygenated blood to organs and tissues. It occurs when certain immune cells attack the affected arteries.


The condition affects adults more frequently than children. It damages the tissues supplied by the affected arteries because they don't receive enough oxygen and nourishment without a proper blood supply.


In this disease, symptoms result from damage to affected organs, often the skin, heart, kidneys, and nervous system.

Generalized symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps.

Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness. Central nervous system involvement may cause strokes or seizures. Kidney involvement can produce varying degrees of renal failure.

Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation of the sack around the heart (pericarditis).

  • Fatigue
  • Weakness
  • Fever
  • Abdominal pain
  • Decreased appetite
  • Unintentional weight loss
  • Muscle aches
  • Joint aches

Signs and tests

There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is generally based upon the physical examination and a few laboratory studies that help to confirm the diagnosis:

  • CBC (may demonstrate an elevated white blood count)
  • ESR (often elevated)
  • Tissue biopsy (reveals inflammation in small arteries, called arteritis)
  • Immunoglobulins (may be increased)


Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide.

Expectations (prognosis)

Current treatments using steroids and other drugs that suppress the immune system (such as cyclophosphamide) can improve symptoms and the chance of long-term survival. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. Without treatment, the outlook is poor.


  • Stroke
  • Kidney failure
  • Heart attack
  • Intestinal necrosis and perforation


This disease cannot currently prevented, but early treatment can prevent some damage and symptoms.


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Bronchiolitis obliterans associated with polyarteritis nodosa
From CHEST, 7/1/92 by Bruce W.S. Robinson

Bronchiolitis obliterans with organizing pneumonia (BOOP) has not previously been described in association with polyarteritis nodosa (PAN). This report describes a patient in whom fulminant systemic PAN followed subacute onset of BOOP, with associated pulmonary arteritis. (Chest 1992; 102:309-11) BOOP = bronchiolitis obliterans with organizing pneumonia; PAN = polyarteritis nodosa.

Bronchiolitis obliterans with organizing pneumonia (BOOP) is usually not associated with any apparent cause of underlying disorder, although some cases occur after inhalation of noxious gases, recent infection, or one of the connective tissue disorders.[1] It has not previously been described in association with polyarteritis nodosa (PAN). This report describes a patient who presented with a systemic illness and BOOP and whose underlying PAN did not become manifest until some months after the onset of his pulmonary disease. This report adds PAN to the list of associated disease processes to consider in a patient presenting with BOOP.

Case Report

A 71-year-old nonsmoking retired carpenter presented with a four-week history of fever, sweating, anorexia, malaise, weight loss (3.2 kg), dry cough, and muscle aches without chest pain, hemoptysis, or previous pulmonary disease. He was febrile (38 [degrees] C) with signs collapse/consolidation in the left upper zone only plus paninspiratory crackles at both bases.

A chest roentgenogram revealed patchy left upper zone consolidation.

The white cell count was 15,600/cu mm (80 percent neutrophils with left shift; no eosinophilia). The hemoglobin level was 11.1 g/L (normal, 13 to 18 g/L); erythrocyte sedimentation rate, 90 mm/h; platelet count, 832,000/cu mm. The serum albumin concentration was 29 g/L (normal, 35 to 45 g/L); alkaline phosphatase, 287 U/L (normal, 35 to 135 U/L); aspartate aminotransferase, 43 U/L (normal, 6 to 42 U/L); creatinine, 87 [Mu] mol/L (normal, <120 [Mu] mol/L); urea nitrogen, 6.1 mmol/L (normal, 3 to 8 mmol/L); electrolytes, normal. Urine microscopic findings were normal. Levels of serum immunoglobulins G, M, A, and E were all within normal limits, and the antinuclear factor and rhematoid factors were both normal. Hepatitis B serologic testing was negative.

The patient was treated with systemic antibiotics (erythromycin, clindamycin, and cefotaxime) but remained well. All investigations for infective agents were negative, specifically, sputum examination (including examination for acid-fast bacilli) and serologic testing for respiratory viruses (influenza A and B; adenovirus; parainfluenza I, II, and III; respiratory syncytial virus; cytomegalovirus; varicella) as well as for psittacosis, Q fever, Mycoplasma, and Legionella (all performed on two separate occasions) plus Toxoplasma, Leptospira, and Brucella.

The patient remained unwell, and the cough persisted. A chest roentgenogram showed progression of the left upper zone changes, with infiltration of the right middle and upper zones. Bronchoscopy revealed no endobronchial lesion, and bronchial washings, brushings, and biopsy revealed no infective organisms. Bronchoalveolar lavage was markedly abnormal with elevated neutrophil proportions (50 percent; normal, <4 percent) with some elevation in eosinophil and lymphocyte proportions (2 percent and 17 percent, respectively [normal, respectively] <1 percent and <16 percent).

Open lung biopsy of the right upper lobe was performed four weeks after admission. Biopsy of the right upper lobe showed severe patchy alteration of lung architecture by loose intra-alveolar fibroblastic tissue and associated nonspecific chronic interstitial inflammation. Many bronchioles contained polypoid projections of fibroblastic and inflammatory tissue, and the numbers of identifiable bronchioles were reduced (Fig 1). Small, thick-walled muscular vessels were present within damaged lung tissue. One medium-sized artery demonstrated fibrinoid necrosis of the wall and infiltration by lymphocytes, histiocytes, and neutrophils (Fig 2). This vessel was considered to be a pulmonary artery, rather than a bronchial artery, because of its large size in relation to the adjacent bronchiolar structure; the vessel was similar in size to other pulmonary arteries in nearby lung, whereas identifiable bronchial arteries were of smaller diameter.

Postoperatively, the patient's renal function deteriorated, the creatinine level rising to 355 [Mu] mol/L. Urinalysis demonstrated hematuria, proteinuria, and occasional hyaline and granular casts. A renal biopsy was performed. One segmental area of glomerular fibrinoid change was evident, together with a mild focal and segmental increase in glomerular mesangial cellularity, and patchy interstitial infiltration. A large arcuate artery showed severe fibrinoid necrosis of the wall. Ultrastructurally, there were no electrondense deposits.

Despite treatment with systemic methylprednisolone and cyclophosphamide, the patient developed ARDS one week postoperatively and died in respiratory and renal failure.

At autopsy, the lungs exhibited alveolar exudation of neutrophils and macrophages and foci of hemorrhage and necrosis. Areas of fibrosis and organizing pneumonia were also present, although changes of bronchiolitis were difficult to find. No vasculitis could be identified in multiple lung sections, and no organisms were seen with special stains.

The kidneys had mottled external surfaces with hemorrhagic and infarcted areas with aneurysmally dilated blood vessels. Multiple foci of segmental or circumferential fibrinoid necrosis were observed microscopically in medium-sized arteries (Fig 3), as well as patchy interstitial hemorrhage and numerous foci of ischemic necrosis.

Vasculitis was confirmed microscopically in the testes, deltoid muscle, tongue, sciatic nerve, prostate gland, and adrenal glands. Fibrinous pericarditis was noted.


The features of a systemic illness associated with patchy pulmonary consolidation, after exclusion of pulmonary infection, and the open-lung biopsy findings confirmed a diagnosis of BOOP. This condition has a distinctive histologic pattern of subacute lung reaction to injury characterized by patchy lung involvement, intra-alveolar fibroblastic tissue, polypoid projections of fibroblastic and inflammatory tissue within bronchioles, bronchiolar destruction and disappearance, and a variable degree of interstitial inflammation and fibrosis.[3-7,11,12] In most cases, there is no known precipitating event. The cryptogenic form of BOOP usually presents clinically as a severe subacute illness over several months with dyspnea, fever, and patchy or interstitial bilateral lung shadowing.[1,3] Diagnosis is usually made at open-lung biopsy. Recognition of the pattern histologically and distinction from other fibrosing lung processes, in particular usual interstitial pneumonitis, is important, since most cases of cryptogenic BOOP will respond to corticosteroid therapy and then resolve completely. Some patients suffer progressive lung impairment and death.

High-dose corticosteroids and cyclophosphamide failed to prevent progression of this patient's pulmonary or renal disease, suggesting that despite the subacute onset of his condition, the disease process in both organs accelerated rapidly at the end of his clinical course.

The sequence of histologic changes was that of BOOP followed by the development of florid PAN of the classic type, apart from one focus of necrotizing vasculitis in a medium-sized pulmonary artery. Although the lung exhibited one focus of necrotizing vasculitis, further vasculitis was not evident in lung at autopsy despite florid vasculitis elsewhere.

The pathogenetic processes underlying the various forms of bronchiolitis

obliterans are unknown. It has been suggested that, when associated with connective tissue diseases, heart-lung transplantation, and perhaps viral infection, the disease represents an autoimmune process whereby the host immune system responds to airway epithelial cell class II antigen expression induced by gamma interferon.[13,14] Spontaneous gamma interferon production by cells obtained at bronchoalveolar lavage was measured in this patient as part of another study and was very high (320 units/[10.sup.6] cells in 24 h [normal individuals release [is less than or equal to] 10 units/[10.sup.6] cells in 24h]);[14,15] this finding is consistent with the above hypothesis.

The failure to respond to treatment is a little unusual for BOOP, although death from progressive disease has been well described.[1,2] This patient clearly had a very aggressive form of the disease.

This report describes an association between BOOP and PAN, which suggests that similar pathogenetic mechanisms may be present in some patients with these disorders. Also, since BOOP can clearly precede the clinical manifestations of PAN, awareness of these facts may lead to careful monitoring for PAN and therefore to earlier therapy.


[1]Epler GR, Colby TV, McLoud TC, Carrington CB, Gaensler EA. Bronchiolitis obliterans organizing pneumonia. N Engl J Med 1985; 312:152-58 [2]Guerry-Force ML, Muller NL, Wright JL, Wiggs B, Coppin C, Pare PD, et al. A comparison of bronchiolitis obliterans with organizing pneumonia, usual interstitial pneumonia, and small airways disease. Am Rev Respir Dis 1987; 135:705-12 [3]Muller NL, Guerry-Force ML, Staples CA, Wright JL, Wiggs B, Coppin C, et al. Differential diagnosis of bronchiolitis obliterans with organizing pneumonia and usual interstitial pneumonia: clinical, functional and radiologic findings. Radiology 1987; 162:151-56 [4]Katzenstein AL, Myers JL, Prophet WD, Corley LS, Shin MS. Bronchiolitis obliterans and usual interstitial pneumonia. Am J Surg Pathol 1986; 10::373-81 [5]Herzog CA, Miller RR, Hoidal JR. Bronchiolitis and rheumatoid arthritis. Am Rev Respir Dis 1981; 124:636-39 [6]Kinney WW, Angelillo VA. Bronchiolitis in systemic lupus erythematosus. Chest 1982; 82:646-49 [7]Geddes DM, Corrin B, Brewerton DA, Davies RJ, Turner-Warwick M. Progressive airway obliteration in adults and its association with rheumatoid disease. Q J Med 1977; 46:427-44 [8]Epler GR, Snider GL, Gaensler EA, Cathcart ES, FitzGerald MX, Carrington CB. Bronchiolitis and bronchitis in connective tissue disease: a possible relationship to the use of penicillamine. JAMA 1979; 242:528-32 [9]Murphy KC, Atkins CJ, Offer RC, Hogg JC, Stein HB. Obliterative bronchiolitis in two rheumatoid arthritis patients treated with penicillamine. Arthritis Rheum 1981; 24:557-60 [10]Homma H, Yamanaka A, Tanimoto S. Tamura M, Chijimatsu Y, Kira S, et al. Diffuse panbronchiolitis: a disease of the transitional zone of the lung. Chest 1983; 83:63-9 [11]Schwarz MI, Matthay RA, Sahn SA, Stanford RE, Marmorstein RL, Scheinhorn DJ. Interstitial lung disease in polymyositis and dermatomyositis: analysis of six cases and review of the literature. Medicine (Baltimore) 1976; 55:89-104 [12]Theodore J, Lemiston N. Lung transplantation comes of age. N Engl J Med 1990; 322:772-74 [13]Burke CM, Glanville AR, Theodore J. Robin ED. Lung immunogenicity, rejection, and obliterative bronchiolitis. Chest 1987; 92:547-49 [14]Halloran PF, Wadgymar A, Autenried P. The regulation of expression of major histocompatibility complex products. Transplantation 1986; 41:413-20 [15]Robinson BWS, McLemore TL, Crystal RG. Gamma interferon is spontaneously produced by lung T-lymphocytes and alveolar macrophages in patients with pulmonary sarcoidosis. J Clin Invest 1985; 75:1488-95 Bruce W. S. Robinson, M. D., F.C.C.P.; and Gregory Sterrett, M.D. From the University Department of Medicine, University of Western Australia (Dr Robinson), and Hospital and University Pathology Services, Sir Charles Gairdner Hospital (Dr Sterrett) Queen Elizabeth II Medical Centre, Nedlands, Western Australia.

COPYRIGHT 1992 American College of Chest Physicians
COPYRIGHT 2004 Gale Group

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