Hospital Practice
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
A Man with Persistent Fever and Diffuse Abdominal Pain Arun Adlakha & Henry J. Schultz To cite this article: Arun Adlakha & Henry J. Schultz (1992) A Man with Persistent Fever and Diffuse Abdominal Pain, Hospital Practice, 27:12, 143-150, DOI: 10.1080/21548331.1992.11705541 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705541
Published online: 17 May 2016.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [Monash University Library]
Date: 03 July 2016, At: 01:54
The Problem Patient
A Man with Persistent Fever and Diffuse Abdon1inal Pain ARUN ADLAKHA
and HENRY J. SCHULTZ
Downloaded by [Monash University Library] at 01:54 03 July 2016
Case Presentation A 43-year-old man was admitted from the emergency room with a history of fever and chills, drainage from the right ear, and generalized abdominal pain. About two weeks before admission, he had had a sore throat, pain in the side of his neck, dysphagia, headache, myalgias, and fever (37.7" to 38.3 •) with chills. Over the next few days, all of his symptoms, except fever, resolved spontaneously. Six days before coming to the emergency room, he noted pain in the right ear. A day later the ear began to drain. He saw his doctor, who started him on oral amoxicillin. The fever persisted, however, and two days prior to admission, he had nausea and vomiting, as well as generalized abdominal pain. He denied any history of anorexia, weight loss, hematemesis, melena or hematochezia, obstipation, urinary symptoms, altered sQIJ.sortum, cough, shortness of breath, chest pain, joint pain, skin lesions, muscle weakness, or paresthesia. His medical history was significant for stable asthma and hypertension. Medications included oral methyldopa, 250 mg twice daily, Dr. Adlakha Is Clinical Instructor In Internal Medicine and Dr. Schultz Is Associate Professor of lntemal MedIcine, Mayo Graduate School of MedIcine, Rochester, Minn.
Mayo Medical School
and albuterol aerosol. two puffs four times daily when needed. Review of systems was positive for night sweats for six months before hospitalization. He had not traveled recently and denied using tobacco or alcohol. Family history was noncontributory. Physical examination showed a moderately sick man with a temperature of 38.3", blood pressure of 110/72, pulse rate of 100, and respiratory rate of 22. Examination of the throat was negative, and there was a healed perforation of the right tympanic membrane. There was no stgniftcant lymphadenopathy, goiter, clubbing, ankle edema, or skin lesions. Examination of the abdomen revealed generalized tenderness (without any peritoneal signs), normal bowel sounds, and no organomegaly, masses, or ascites. Rectal examination was negative. The rest of the examination was normal. Initial laboratory values were hemoglobin 11, hematocrit 32% with normal red cell volumes, white blood cell count 15,200 (90% segmented neutrophils), platelet count 520,000, and erythrocyte sedimentation rate 38 mm/hr. Urinalysis and serum amylase, lipase, electrolytes, and chemistry panel were normal. A 12-lead ECG showed sinus tachycardia with mild left ventricular hypertrophy. X-ray of the chest was normal. Plain film of
the abdomen revealed no evidence of perforation or obstruction. Urine, sputum, and blood cultures were sterile. The patient was admitted with a diagnosis of acute abdomen of unknown etiology and was managed with nasogastric suction and intravenous fluids. Abdominal pain and fever persisted, and leukocytosis with neutrophilia worsened. After surgical consultation, an exploratory laparotomy was performed. Preoperative diagnostic possibilities included atypical appendicitis, Meckel's diverticulum, omental or bowel infarction, or diverticulitis. The abdominal examination was negative. An appendectomy was performed, and histopathology of the appendix was normal. After surgery, the patient's overall condition was unimproved. He continued to have fever and generalized abdominal pain. The leukocytosis persisted, and the erythrocyte sedimentation rate increased to 62. On infectious disease consultation, his case was evaluated as fever of undetermined origin with leukocytosis, normochromic normocytic anemia, and elevated erythrocyte sedimentation rate. Repeat urine, sputum, and blood cultures were negative. Peripheral smears were negative for atypical cells and for para(conttnues)
Hospital Practice December 15. 1992
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FEvER
(continued)
sites. Serology for Legionella, Mycoplasma, cytomegalovirus, Epstein-Barr virus, Coxiella burnettt, Brucella, Toxoplasma, and fungi was negative. Assays for antinuclear antibody, rheumatoid factor, and cryoglobulins also were negative. Serum protein electrophoresis revealed a polyclonal hypergammaglobulinemia. Serum complement components were in the normal range. HIV, PPD, and HBsAg tests were negative. A 24-hour urinary creatinine clearancewas 110ml/min. X-rays of the paranasal sinuses, chest, and abdomen were negative. Sputum and nasogastric aspirates were negative for acid-fast bacilli and fungi; stool studies were negative; a twodimensional transthoracic echocardiogram was normal; CSF study and bone marrow aspiration and biopsy were inconclusive; and HEENT examination was normal. During this time, the patient's fever, malaise, and generalized abdominal pain persisted, and he began to experience arthralgias and myalgias. Leukocytes increased to 22,000, with 92% neutrophils, and the erythrocyte sedimentation rate rose to 112. Chemistry panel now showed serum AST 86 (normal, 9-29), ALT 52 (normal, 9-31), GGTP 156 (normal, 9-29), and alkaline phosphatase 464 U/L (normal, 199-310). CT scan of the abdomen was negative. Selective renal and superior mesenteric angiography was then performed after nearly one month of hospitalization. The renal angiograms revealed severe vasculitis in small vessels of both kidneys, with aneurysms suggestive of periarteritis nodosa(Figure 1).
The superior mesenteric angiogram was essentially normal. Celiac axis angiography was not performed. The patient was then started on oral prednisone, 60 mgdaily. Over the next few weeks his symptoms improved. The erythrocyte sedimentation rate fell to 2, and the liver function values normalized. Prednisone was
then reduced by 5 to 10 mg every two weeks, and once the dosage was down to 15 mgtday, it was further reduced by 1 mg every several weeks. After six months, the glucocorticoid was withdrawn. A follow-up angiogram was normal, with complete resolution of the aneurysms. The patient has not experienced any relapse.
Figure 1. Anglograms of left (above) and right kidneys showed severe small-vessel anomalies throughout both organs, Including mlcroaneurysms and multiple areas of tortuosity with vascular occlusion suggestive of perlarterHis nodosa.
Hospital Practice December 15. 1992
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The Case in Context
148
In 1866, Adolf Kussmaul and Rudolf Maier first reported a case of widespread necrotizing inflammation of small and medium arteries in a patient With palpable nodules along the course of the arteries. They called it periarteritis nodosa. The disease (commonly called polyarteritis nodosa), has an annual incidence of 0. 7 per 100,000 and a prevalence of 6.3. It occurs between 40 and 60 years of age, more often in males (2: 1 ). The most common etiologies postulated are 1) immune complexes, 2) viruses (hepatitis B, HIV, CMV, parvovirus), 3) antibasement membrane antibodies, and 4) hairy cell leukemia (hairy cells have been found in vasculitlc lesions; antibodies directed toward certain determinants on hairy cells can cross-
react with endothelial cells). Whatever the cause, the effects are protean. They include elevation of von Willebrand factor and factor VIII; release of platelet activating factor, interleukin-1, colony stimulating factors, and growth factors; induction of class I and II antigens, C3b and Fe receptors, y-interferon, and tumor necrosis factor; increased production of thromboxane B 2 and decreased levels of prostacyclin; hyperfibrinogenemia and hypofibrinolytic activity; and diminished endothelium-derived relaxation factor and release of endothelin. The pathophysiologic pathway involves initiation and maintenance of an immune response, with vasospasm, clot formation, and cellular proliferation. The classic pathology of periarteritis nodosa is generalized necrotizing inflammation of
small and medium arteries with predilection for the bifurcation of vessels. Acute changes consist of fibrinoid necrosis and pleomorphic cellular infiltration, With chronic changes that include endothelial cell and fibrous tissue proliferation. Thrombosis, aneurysmal dilation, and vessel occlusion are the end result of the inflammatory and healing processes. Lesions at all stages may occur simultaneously. Kidneys, intestines, skin, peripheral nerves, and joints are the organs most commonly affected; pulmonary and splenic arteries are the least involved. Periarteritis nodosa may be mild and ~imited or progressive and fatal. Classically, the patient presents with constitutional symptoms (fever, malaise, weight loss) and coincidental or subsequent multisystem and
Downloaded by [Monash University Library] at 01:54 03 July 2016
visceral involvement. Isolated single-organ involvement, however, can occur in some cases and may remain limited. Peripheral neuropathy (e.g., mononeuritis multiplex, mononeuropathy, symmetric polyneuropathy, distal sensory neuropathy, or brachial plexus neuropathy) occurs in 50% to 70% of cases. Central nervous system involvement, such as seizures or hemiparesis, occurs in approximately 10%. In nearly 70% of cases, there is frank renal involvement (e.g., proteinuria, nephrotic syndrome, renal failure, or hypertension). Gastrointestinal involvement occurs in about 50% of cases. Cutaneous lesions (e.g., ulceration, livedo, palpable purpura, infarctions, ischemia, or nodules) occur in up to 50%. Arthritis or arthralgtas are present in about 50%; and an asym-
metric, episodic, nondeforming polyarthritis (especially of the lower extremities) in 20% of patients. Cardiac involvement is less common. Skeletal muscle involvement, such as pain or intermittent claudication, is seen in 30% of cases. Organs uncommonly involved are testes (although autopsy studies show vasculitis in about 86% of patients), lungs, spleen, eyes, and temporal arteries. Normochromic normocytic anemia, thrombocytosis, elevated erythrocyte sedimentation rate, decreased serum albumin and complement, rheumatoid factor, and cryoglobulinemia are all nonspecific findings. Hepatitis B surface antigen has been found in 10% to 54% of cases, and immune complexes may be found in the sera of some patients. HBsAg-associated periarteritis nodosa usually devel-
ops in patients who are chronic carriers of the virus. The best diagnostic tests are biopsy of the clinically abnormal tissue (e.g., skin, muscle, testis, sural nerve, kidney, or lung) and visceral angiography. Blind muscle biopsies may show arteritis in 30% to 50% of cases. Angiography is called for when there is intra-abdominal involvement or there is no tissue available for biopsy. Aneurysms are most commonly found in the kidneys and liver and usually suggest severe and extensive disease. In patients with limited or nonprogressive disease. prednisone alone may be sufficient. Clinical status and erythrocyte sedimentation rate are followed closely to assess treatment response. Tapering should be done gradually and cautiously. The patient may have to be maintained on a low-dose schedule indefi-
149
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nitely. Alternate-day dosage, however, is not advised because of the risk of disease flare-ups. Occasionally, glucocorticoids may promote vascular occlusion because of their incomplete inhibition of thromboxane and significant inhibition of prostacyclin production. Addition of vasodilator or antiplatelet drugs may modify this effect and thereby reduce vascular complications (early and late). The presence of extensive visceral involvement or evidence of disease progression should warrant the addition of cytotoxic drugs. Cyclophosphamide is commonly used, but azathioprine and methotrexate also have been effective, as has been plasma exchange combined with glucocorticoids and cytotoxic drugs. Renal transplantation can be performed once arteritis becomes inactive. Five-year survival associated with untreated disease is 15% and is improved to 50% to 60% by the addition of steroids. Early death (within a year of diagnosis) is due to diagnostic delay leading to uncontrolled vasculitis or to infectious complications of treatment. Death one year or more after diagnosis usually is from stroke, myocardial infarction, renal failure, hypertension, or complications of treatment. This case demonstrates that a patient with limited and subtle periarteritis nodosa may be hos-
pitalized for weeks of intensive investigation for septicemia, infective endocarditis, malignancy, atrial myxoma, connective tissue disease, and so on, without a diagnosis. Lack of cutaneous, musculoskeletal, and systemic involvement and the absence of any clinically involved tissue for biopsy made the diagnosis more difficult in our patient. Presence of fever, constitutional symptoms, diffuse abdominal pain, normochromic normocytic anemia, leukocytosis with neutrophilia, thrombocytosis, polyclonal hypergammaglo bulinemia, high erythrocyte sedimentation rate, and abnormal liver function finally suggested the need for visceral angiography, with which the diagnosis was made. The response to prednisone confirmed the diagnosis. Surprisingly, despite the renal vessel involvement seen on the angiogram, the patient had normal renal function. The superior mesenteric angiogram was grossly normal, and a celiac axis angiogram (which might have shown hepatic artery aneurysms) was not obtained. We assume that he had small-vessel mesenteric disease (not evident on angiography), giving rise to generalized and persistent abdominal pain and tenderness. In retrospect, it is possible that his right-sided otitis media and perforation of the tympanic membrane was also a part of the periarteritis nodosa. D
Selected Reading Albert DA, Rimon D, Silverstein MC: The diagnosis of polyarteritis nodosa. I. A literature-based decision analysis approach. Arthritis Rheum 31:1117,1988 Conn DL et al: Immunologic mechanisms in systemic vasculitis. Mayo ClinProc 51:511, 1976 Conn DL: Polyarteritis. Rheum Dis Clin North Am 16: 341, 1990 Conn DL, Thmpkins RB, Nichols WL: Glucocortlcoids in the management of vasculitis-a double-edged sword? J Rheumatoll5: 1181, 1988 Guillevin L et al: Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: A study in 165 patients. Br J Rheumatol 27:258,1988
150
ISSN: 2154-8331 (Print) 2377-1003 (Online) Journal homepage: http://www.tandfonline.com/loi/ihop20
A Man with Persistent Fever and Diffuse Abdominal Pain Arun Adlakha & Henry J. Schultz To cite this article: Arun Adlakha & Henry J. Schultz (1992) A Man with Persistent Fever and Diffuse Abdominal Pain, Hospital Practice, 27:12, 143-150, DOI: 10.1080/21548331.1992.11705541 To link to this article: http://dx.doi.org/10.1080/21548331.1992.11705541
Published online: 17 May 2016.
Submit your article to this journal
View related articles
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ihop20 Download by: [Monash University Library]
Date: 03 July 2016, At: 01:54
The Problem Patient
A Man with Persistent Fever and Diffuse Abdon1inal Pain ARUN ADLAKHA
and HENRY J. SCHULTZ
Downloaded by [Monash University Library] at 01:54 03 July 2016
Case Presentation A 43-year-old man was admitted from the emergency room with a history of fever and chills, drainage from the right ear, and generalized abdominal pain. About two weeks before admission, he had had a sore throat, pain in the side of his neck, dysphagia, headache, myalgias, and fever (37.7" to 38.3 •) with chills. Over the next few days, all of his symptoms, except fever, resolved spontaneously. Six days before coming to the emergency room, he noted pain in the right ear. A day later the ear began to drain. He saw his doctor, who started him on oral amoxicillin. The fever persisted, however, and two days prior to admission, he had nausea and vomiting, as well as generalized abdominal pain. He denied any history of anorexia, weight loss, hematemesis, melena or hematochezia, obstipation, urinary symptoms, altered sQIJ.sortum, cough, shortness of breath, chest pain, joint pain, skin lesions, muscle weakness, or paresthesia. His medical history was significant for stable asthma and hypertension. Medications included oral methyldopa, 250 mg twice daily, Dr. Adlakha Is Clinical Instructor In Internal Medicine and Dr. Schultz Is Associate Professor of lntemal MedIcine, Mayo Graduate School of MedIcine, Rochester, Minn.
Mayo Medical School
and albuterol aerosol. two puffs four times daily when needed. Review of systems was positive for night sweats for six months before hospitalization. He had not traveled recently and denied using tobacco or alcohol. Family history was noncontributory. Physical examination showed a moderately sick man with a temperature of 38.3", blood pressure of 110/72, pulse rate of 100, and respiratory rate of 22. Examination of the throat was negative, and there was a healed perforation of the right tympanic membrane. There was no stgniftcant lymphadenopathy, goiter, clubbing, ankle edema, or skin lesions. Examination of the abdomen revealed generalized tenderness (without any peritoneal signs), normal bowel sounds, and no organomegaly, masses, or ascites. Rectal examination was negative. The rest of the examination was normal. Initial laboratory values were hemoglobin 11, hematocrit 32% with normal red cell volumes, white blood cell count 15,200 (90% segmented neutrophils), platelet count 520,000, and erythrocyte sedimentation rate 38 mm/hr. Urinalysis and serum amylase, lipase, electrolytes, and chemistry panel were normal. A 12-lead ECG showed sinus tachycardia with mild left ventricular hypertrophy. X-ray of the chest was normal. Plain film of
the abdomen revealed no evidence of perforation or obstruction. Urine, sputum, and blood cultures were sterile. The patient was admitted with a diagnosis of acute abdomen of unknown etiology and was managed with nasogastric suction and intravenous fluids. Abdominal pain and fever persisted, and leukocytosis with neutrophilia worsened. After surgical consultation, an exploratory laparotomy was performed. Preoperative diagnostic possibilities included atypical appendicitis, Meckel's diverticulum, omental or bowel infarction, or diverticulitis. The abdominal examination was negative. An appendectomy was performed, and histopathology of the appendix was normal. After surgery, the patient's overall condition was unimproved. He continued to have fever and generalized abdominal pain. The leukocytosis persisted, and the erythrocyte sedimentation rate increased to 62. On infectious disease consultation, his case was evaluated as fever of undetermined origin with leukocytosis, normochromic normocytic anemia, and elevated erythrocyte sedimentation rate. Repeat urine, sputum, and blood cultures were negative. Peripheral smears were negative for atypical cells and for para(conttnues)
Hospital Practice December 15. 1992
143
Downloaded by [Monash University Library] at 01:54 03 July 2016
FEvER
(continued)
sites. Serology for Legionella, Mycoplasma, cytomegalovirus, Epstein-Barr virus, Coxiella burnettt, Brucella, Toxoplasma, and fungi was negative. Assays for antinuclear antibody, rheumatoid factor, and cryoglobulins also were negative. Serum protein electrophoresis revealed a polyclonal hypergammaglobulinemia. Serum complement components were in the normal range. HIV, PPD, and HBsAg tests were negative. A 24-hour urinary creatinine clearancewas 110ml/min. X-rays of the paranasal sinuses, chest, and abdomen were negative. Sputum and nasogastric aspirates were negative for acid-fast bacilli and fungi; stool studies were negative; a twodimensional transthoracic echocardiogram was normal; CSF study and bone marrow aspiration and biopsy were inconclusive; and HEENT examination was normal. During this time, the patient's fever, malaise, and generalized abdominal pain persisted, and he began to experience arthralgias and myalgias. Leukocytes increased to 22,000, with 92% neutrophils, and the erythrocyte sedimentation rate rose to 112. Chemistry panel now showed serum AST 86 (normal, 9-29), ALT 52 (normal, 9-31), GGTP 156 (normal, 9-29), and alkaline phosphatase 464 U/L (normal, 199-310). CT scan of the abdomen was negative. Selective renal and superior mesenteric angiography was then performed after nearly one month of hospitalization. The renal angiograms revealed severe vasculitis in small vessels of both kidneys, with aneurysms suggestive of periarteritis nodosa(Figure 1).
The superior mesenteric angiogram was essentially normal. Celiac axis angiography was not performed. The patient was then started on oral prednisone, 60 mgdaily. Over the next few weeks his symptoms improved. The erythrocyte sedimentation rate fell to 2, and the liver function values normalized. Prednisone was
then reduced by 5 to 10 mg every two weeks, and once the dosage was down to 15 mgtday, it was further reduced by 1 mg every several weeks. After six months, the glucocorticoid was withdrawn. A follow-up angiogram was normal, with complete resolution of the aneurysms. The patient has not experienced any relapse.
Figure 1. Anglograms of left (above) and right kidneys showed severe small-vessel anomalies throughout both organs, Including mlcroaneurysms and multiple areas of tortuosity with vascular occlusion suggestive of perlarterHis nodosa.
Hospital Practice December 15. 1992
147
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The Case in Context
148
In 1866, Adolf Kussmaul and Rudolf Maier first reported a case of widespread necrotizing inflammation of small and medium arteries in a patient With palpable nodules along the course of the arteries. They called it periarteritis nodosa. The disease (commonly called polyarteritis nodosa), has an annual incidence of 0. 7 per 100,000 and a prevalence of 6.3. It occurs between 40 and 60 years of age, more often in males (2: 1 ). The most common etiologies postulated are 1) immune complexes, 2) viruses (hepatitis B, HIV, CMV, parvovirus), 3) antibasement membrane antibodies, and 4) hairy cell leukemia (hairy cells have been found in vasculitlc lesions; antibodies directed toward certain determinants on hairy cells can cross-
react with endothelial cells). Whatever the cause, the effects are protean. They include elevation of von Willebrand factor and factor VIII; release of platelet activating factor, interleukin-1, colony stimulating factors, and growth factors; induction of class I and II antigens, C3b and Fe receptors, y-interferon, and tumor necrosis factor; increased production of thromboxane B 2 and decreased levels of prostacyclin; hyperfibrinogenemia and hypofibrinolytic activity; and diminished endothelium-derived relaxation factor and release of endothelin. The pathophysiologic pathway involves initiation and maintenance of an immune response, with vasospasm, clot formation, and cellular proliferation. The classic pathology of periarteritis nodosa is generalized necrotizing inflammation of
small and medium arteries with predilection for the bifurcation of vessels. Acute changes consist of fibrinoid necrosis and pleomorphic cellular infiltration, With chronic changes that include endothelial cell and fibrous tissue proliferation. Thrombosis, aneurysmal dilation, and vessel occlusion are the end result of the inflammatory and healing processes. Lesions at all stages may occur simultaneously. Kidneys, intestines, skin, peripheral nerves, and joints are the organs most commonly affected; pulmonary and splenic arteries are the least involved. Periarteritis nodosa may be mild and ~imited or progressive and fatal. Classically, the patient presents with constitutional symptoms (fever, malaise, weight loss) and coincidental or subsequent multisystem and
Downloaded by [Monash University Library] at 01:54 03 July 2016
visceral involvement. Isolated single-organ involvement, however, can occur in some cases and may remain limited. Peripheral neuropathy (e.g., mononeuritis multiplex, mononeuropathy, symmetric polyneuropathy, distal sensory neuropathy, or brachial plexus neuropathy) occurs in 50% to 70% of cases. Central nervous system involvement, such as seizures or hemiparesis, occurs in approximately 10%. In nearly 70% of cases, there is frank renal involvement (e.g., proteinuria, nephrotic syndrome, renal failure, or hypertension). Gastrointestinal involvement occurs in about 50% of cases. Cutaneous lesions (e.g., ulceration, livedo, palpable purpura, infarctions, ischemia, or nodules) occur in up to 50%. Arthritis or arthralgtas are present in about 50%; and an asym-
metric, episodic, nondeforming polyarthritis (especially of the lower extremities) in 20% of patients. Cardiac involvement is less common. Skeletal muscle involvement, such as pain or intermittent claudication, is seen in 30% of cases. Organs uncommonly involved are testes (although autopsy studies show vasculitis in about 86% of patients), lungs, spleen, eyes, and temporal arteries. Normochromic normocytic anemia, thrombocytosis, elevated erythrocyte sedimentation rate, decreased serum albumin and complement, rheumatoid factor, and cryoglobulinemia are all nonspecific findings. Hepatitis B surface antigen has been found in 10% to 54% of cases, and immune complexes may be found in the sera of some patients. HBsAg-associated periarteritis nodosa usually devel-
ops in patients who are chronic carriers of the virus. The best diagnostic tests are biopsy of the clinically abnormal tissue (e.g., skin, muscle, testis, sural nerve, kidney, or lung) and visceral angiography. Blind muscle biopsies may show arteritis in 30% to 50% of cases. Angiography is called for when there is intra-abdominal involvement or there is no tissue available for biopsy. Aneurysms are most commonly found in the kidneys and liver and usually suggest severe and extensive disease. In patients with limited or nonprogressive disease. prednisone alone may be sufficient. Clinical status and erythrocyte sedimentation rate are followed closely to assess treatment response. Tapering should be done gradually and cautiously. The patient may have to be maintained on a low-dose schedule indefi-
149
Downloaded by [Monash University Library] at 01:54 03 July 2016
nitely. Alternate-day dosage, however, is not advised because of the risk of disease flare-ups. Occasionally, glucocorticoids may promote vascular occlusion because of their incomplete inhibition of thromboxane and significant inhibition of prostacyclin production. Addition of vasodilator or antiplatelet drugs may modify this effect and thereby reduce vascular complications (early and late). The presence of extensive visceral involvement or evidence of disease progression should warrant the addition of cytotoxic drugs. Cyclophosphamide is commonly used, but azathioprine and methotrexate also have been effective, as has been plasma exchange combined with glucocorticoids and cytotoxic drugs. Renal transplantation can be performed once arteritis becomes inactive. Five-year survival associated with untreated disease is 15% and is improved to 50% to 60% by the addition of steroids. Early death (within a year of diagnosis) is due to diagnostic delay leading to uncontrolled vasculitis or to infectious complications of treatment. Death one year or more after diagnosis usually is from stroke, myocardial infarction, renal failure, hypertension, or complications of treatment. This case demonstrates that a patient with limited and subtle periarteritis nodosa may be hos-
pitalized for weeks of intensive investigation for septicemia, infective endocarditis, malignancy, atrial myxoma, connective tissue disease, and so on, without a diagnosis. Lack of cutaneous, musculoskeletal, and systemic involvement and the absence of any clinically involved tissue for biopsy made the diagnosis more difficult in our patient. Presence of fever, constitutional symptoms, diffuse abdominal pain, normochromic normocytic anemia, leukocytosis with neutrophilia, thrombocytosis, polyclonal hypergammaglo bulinemia, high erythrocyte sedimentation rate, and abnormal liver function finally suggested the need for visceral angiography, with which the diagnosis was made. The response to prednisone confirmed the diagnosis. Surprisingly, despite the renal vessel involvement seen on the angiogram, the patient had normal renal function. The superior mesenteric angiogram was grossly normal, and a celiac axis angiogram (which might have shown hepatic artery aneurysms) was not obtained. We assume that he had small-vessel mesenteric disease (not evident on angiography), giving rise to generalized and persistent abdominal pain and tenderness. In retrospect, it is possible that his right-sided otitis media and perforation of the tympanic membrane was also a part of the periarteritis nodosa. D
Selected Reading Albert DA, Rimon D, Silverstein MC: The diagnosis of polyarteritis nodosa. I. A literature-based decision analysis approach. Arthritis Rheum 31:1117,1988 Conn DL et al: Immunologic mechanisms in systemic vasculitis. Mayo ClinProc 51:511, 1976 Conn DL: Polyarteritis. Rheum Dis Clin North Am 16: 341, 1990 Conn DL, Thmpkins RB, Nichols WL: Glucocortlcoids in the management of vasculitis-a double-edged sword? J Rheumatoll5: 1181, 1988 Guillevin L et al: Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: A study in 165 patients. Br J Rheumatol 27:258,1988
150
