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Multiple saccular aortic aneurysms in nonspecific aortitis
February
Multiple Saccular Aortic Aneurysms in Nonspecific Aortitis STUART I. HENOCHOWICZ, MD JOSEPH LINDSAY, Jr., MD MICHAEL J. FURLONG, MD ANN K. FULENWIDER, MD DAVID I. GREENFIELD, MD ELIZABETH M. ROSS, MD
M
ultiple saccular aortic aneurysmsare not characteristic of the common forms of aortic disease:atherosclerosis,cystic medial degeneration,luetic aortitis, or HLA B-27associatedaortopathy.This report describes the aortographicand necropsy findings in a patient with nonspecific aortitis who died from rupture of 1 of 8 saccular aortic aneurysms. A 65-year-old woman presented with fever, dyspnea and pleuritic chestpain for 1 week. At age51,14 years earlier, she had the onset of weight loss and
1, 1966
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume
57
377
numbnessin her hands and feet. At age 53, she was seenat the National Institutes of Health. The patient had lost 15poundsin 2 years.She was unableto climb stairs and used a wheelchair. On examination the blood pressurewas XXV90mm Hg; she had a sensorymotor neuropathy, marked ataxia, and thyroid enlargement. Laboratory studies revealed serum positive for cryogiobulins,an elevatedsedimentationrate, a positive rheumatoid factor (2:32dilution) and hypergammaglobulinemia.There was evidenceof vasculitis on musclebiopsy.She wastreatedwith prednisonefor 2 years without significant therapeutic effect. Her symptomswere essentiallyunchangeduntil the week before her final admissionwhen her presentingsymptoms began. Temperature now was 101.8~F;bibasilar pulmonary rales and marked sensory deficits in her lower extremities were present.Chestx-ray revealeda mass in the left upper lung field. Aortography revealedmultiple aneurysmsof the thoracic and abdominal aorta (Fig. I]. Serum cryoglobulins were absent.Multiple bloodcultureswere sterile. A serologictestfor syphilis and a fluorescent antinuclear antibody examination were negative.Hepatitis B core antibody waspresent. lust beforeplanned aortic surgery,shedied suddenly.
From the Departments of Medicine and Pathology, The Washington Hospital Center and The George Washington University School of Medicine, Washington, DC; and the Pathology Branch, National Heart, Lung, and Blood Institutes, Bethesda, Maryland. Manuscript received April 29, 1985; revised manuscript received June 7,1985, accepted June 10,1985.
FIGURE
1. Aortographic
film illustrating
4 of the 8 aneurysms.
FIGURE 2. Six of the 8 aneurysms, of the medial dlssectlon.
including
the site of rupture
and
378
BRIEF REPORTS
At necropsy,8 saccular aortic aneurysmsvarying in sizefrom 1 to 6 cm were present(Fig. 2).The largest arose distal to the left subclavian artery. It had ruptured into the left upper lobe of the left lung. A medial dissectionextendedfrom the site of rupture to the iliac artery [Fig. 2). The aortic intima throughoutwas normal or nearly so. The walls of the aneurysms contained dense chronic inflammatory cell infiltrates (plasma cells and lymphocytes] with accompanying medial destruction and adventitial fibrosis. Stains of aortic sections for microorganisms were negative. Random sections of uninvolved aorta showed scattered chronic inflammatory cells (primarily plasmacytes) within the adventitia. Additional findings included chronic lymphocytic thyroiditis and marked demyelination of the dorsal columns and spinal nerve roots, consistentwith an immunoglobulin-associated neuropathy and myelopathy. Occasionallyaortitis is recognizedappearingunrelated to a specific infectious agent having the clinical and anatomic characteristicsof neither giant cell arteritis nor HLA B-27 aortopathies.Reportsfrom the Orient, where such aortitis occurswith some frequency, emphasize the occurrence of this disorder in young women and discuss the syndrome under the rubric Takayasu’s aortitis.lp2The clinical features of many instances of aortitis observed in Europe and in the Westernhemisphere vary from thoseseenin the Orient. Patients may be older and stenosisof aortic segments not as prevalents3Such nonspecific aortitis is idiopathic and is unlikely to be homogeneousin either etiologyor pathogenesis.In somepatients,evidenceof
a disturbed immune systemis found and autoimmunity has often been suggested as a pathogenetic mechanism.lJ Our patient is an example of aortitis associatedwith evidence of an autoimmune disorder. She had cryoglobulinemia, hyperglobulinemia, positive rheumatoid factor, polyneuropathy,and biopsy-provedvasculititis together with the necropsy findings of lymphocytic thyroiditis, spinal cord degenerationand chronic aortitis. The presenceof hepatitis B core antibody was alsointriguing, becausehepatitis B infection has been associatedwith polyarteritis nodosa.4 Although constrictionof the lumen of the aortaor of its main branchesis the most widely appreciatedmanifestation of nonspecific aortitis, multiple saccularaneurysms have also been frequently reported.5teBecause multiple saccular aneurysms are unusual in other forms of aortic disease,their presencemay be useful as an indicator of aortitis.
References 1. Lupi-Herra E, Sanchez-Torres G, Marchushamer J, Mispireta J, Horwitz S. Vela JE. Tokayasu’s arteritis. Clinical study of 107 cases. Am Heart J 1977;93:94-103. 2. Ishikswa K. Natural history and classification of occlusive thromboaortopathy (Takayasu’s disease]. Circulation 1978;57:27-35. 3. Lande A. Be&men YM. Aortitis: pathologic. clinical and angiographic review. Radio1 Clin North Am 1976;14:219-240. 4. Fauci AS, Haynes BF, Katz P. The spectrum of vasculitis: clinical. pathologic, immunologic and therapeutic considerations. Ann Intern Med 1978; 89(part 1):880-678. 5. Restrego C, Tejeda C. Correa P. Nonsyphilitic aortitis. Arch Pathol 1989;87:1-12. 6. Teoh PC. Tan KLA, Chia BL, Chao TC, Tambyak JA. Feng PH. Non-specific aorto-arteritis in Singapore with special reference to hypertension. Am Heart \ 1978;95:883-890.
Multiple Saccular Aortic Aneurysms in Nonspecific Aortitis STUART I. HENOCHOWICZ, MD JOSEPH LINDSAY, Jr., MD MICHAEL J. FURLONG, MD ANN K. FULENWIDER, MD DAVID I. GREENFIELD, MD ELIZABETH M. ROSS, MD
M
ultiple saccular aortic aneurysmsare not characteristic of the common forms of aortic disease:atherosclerosis,cystic medial degeneration,luetic aortitis, or HLA B-27associatedaortopathy.This report describes the aortographicand necropsy findings in a patient with nonspecific aortitis who died from rupture of 1 of 8 saccular aortic aneurysms. A 65-year-old woman presented with fever, dyspnea and pleuritic chestpain for 1 week. At age51,14 years earlier, she had the onset of weight loss and
1, 1966
THE AMERICAN
JOURNAL
OF CARDIOLOGY
Volume
57
377
numbnessin her hands and feet. At age 53, she was seenat the National Institutes of Health. The patient had lost 15poundsin 2 years.She was unableto climb stairs and used a wheelchair. On examination the blood pressurewas XXV90mm Hg; she had a sensorymotor neuropathy, marked ataxia, and thyroid enlargement. Laboratory studies revealed serum positive for cryogiobulins,an elevatedsedimentationrate, a positive rheumatoid factor (2:32dilution) and hypergammaglobulinemia.There was evidenceof vasculitis on musclebiopsy.She wastreatedwith prednisonefor 2 years without significant therapeutic effect. Her symptomswere essentiallyunchangeduntil the week before her final admissionwhen her presentingsymptoms began. Temperature now was 101.8~F;bibasilar pulmonary rales and marked sensory deficits in her lower extremities were present.Chestx-ray revealeda mass in the left upper lung field. Aortography revealedmultiple aneurysmsof the thoracic and abdominal aorta (Fig. I]. Serum cryoglobulins were absent.Multiple bloodcultureswere sterile. A serologictestfor syphilis and a fluorescent antinuclear antibody examination were negative.Hepatitis B core antibody waspresent. lust beforeplanned aortic surgery,shedied suddenly.
From the Departments of Medicine and Pathology, The Washington Hospital Center and The George Washington University School of Medicine, Washington, DC; and the Pathology Branch, National Heart, Lung, and Blood Institutes, Bethesda, Maryland. Manuscript received April 29, 1985; revised manuscript received June 7,1985, accepted June 10,1985.
FIGURE
1. Aortographic
film illustrating
4 of the 8 aneurysms.
FIGURE 2. Six of the 8 aneurysms, of the medial dlssectlon.
including
the site of rupture
and
378
BRIEF REPORTS
At necropsy,8 saccular aortic aneurysmsvarying in sizefrom 1 to 6 cm were present(Fig. 2).The largest arose distal to the left subclavian artery. It had ruptured into the left upper lobe of the left lung. A medial dissectionextendedfrom the site of rupture to the iliac artery [Fig. 2). The aortic intima throughoutwas normal or nearly so. The walls of the aneurysms contained dense chronic inflammatory cell infiltrates (plasma cells and lymphocytes] with accompanying medial destruction and adventitial fibrosis. Stains of aortic sections for microorganisms were negative. Random sections of uninvolved aorta showed scattered chronic inflammatory cells (primarily plasmacytes) within the adventitia. Additional findings included chronic lymphocytic thyroiditis and marked demyelination of the dorsal columns and spinal nerve roots, consistentwith an immunoglobulin-associated neuropathy and myelopathy. Occasionallyaortitis is recognizedappearingunrelated to a specific infectious agent having the clinical and anatomic characteristicsof neither giant cell arteritis nor HLA B-27 aortopathies.Reportsfrom the Orient, where such aortitis occurswith some frequency, emphasize the occurrence of this disorder in young women and discuss the syndrome under the rubric Takayasu’s aortitis.lp2The clinical features of many instances of aortitis observed in Europe and in the Westernhemisphere vary from thoseseenin the Orient. Patients may be older and stenosisof aortic segments not as prevalents3Such nonspecific aortitis is idiopathic and is unlikely to be homogeneousin either etiologyor pathogenesis.In somepatients,evidenceof
a disturbed immune systemis found and autoimmunity has often been suggested as a pathogenetic mechanism.lJ Our patient is an example of aortitis associatedwith evidence of an autoimmune disorder. She had cryoglobulinemia, hyperglobulinemia, positive rheumatoid factor, polyneuropathy,and biopsy-provedvasculititis together with the necropsy findings of lymphocytic thyroiditis, spinal cord degenerationand chronic aortitis. The presenceof hepatitis B core antibody was alsointriguing, becausehepatitis B infection has been associatedwith polyarteritis nodosa.4 Although constrictionof the lumen of the aortaor of its main branchesis the most widely appreciatedmanifestation of nonspecific aortitis, multiple saccularaneurysms have also been frequently reported.5teBecause multiple saccular aneurysms are unusual in other forms of aortic disease,their presencemay be useful as an indicator of aortitis.
References 1. Lupi-Herra E, Sanchez-Torres G, Marchushamer J, Mispireta J, Horwitz S. Vela JE. Tokayasu’s arteritis. Clinical study of 107 cases. Am Heart J 1977;93:94-103. 2. Ishikswa K. Natural history and classification of occlusive thromboaortopathy (Takayasu’s disease]. Circulation 1978;57:27-35. 3. Lande A. Be&men YM. Aortitis: pathologic. clinical and angiographic review. Radio1 Clin North Am 1976;14:219-240. 4. Fauci AS, Haynes BF, Katz P. The spectrum of vasculitis: clinical. pathologic, immunologic and therapeutic considerations. Ann Intern Med 1978; 89(part 1):880-678. 5. Restrego C, Tejeda C. Correa P. Nonsyphilitic aortitis. Arch Pathol 1989;87:1-12. 6. Teoh PC. Tan KLA, Chia BL, Chao TC, Tambyak JA. Feng PH. Non-specific aorto-arteritis in Singapore with special reference to hypertension. Am Heart \ 1978;95:883-890.