å¡ CASE REPORT å¡ Periodic Fever Compatible with Familial Mediterranean Fever Mayumi Takahashi, Tsukasa Ebe, Tomoo Kohara, Masayoshi Inagaki, Hiroshi Isonuma, Ichiroh Hibiya, Takeshi Mori, Kazuyoshi Watanabe* and Hideo Ikemoto A 55-year-old male presented with a recurrent fever of over 38°C, occurring at irregular intervals 1-6 times a month with chest, back or abdominal pain. After admission to our hospital, we found the following characteristics: 1) the febrile attacks were accompanied by obvious inflammatory findings and pleuritis or peritonitis; 2) the patient's elder sister had a similar periodic fever; and 3) there were no apparent causative factors responsible for his symptoms. Therefore, we diagnosed this as a case compatible with familial Mediterranean fever. The febrile attacks have been completely suppressed by daily colchicine. This is the seventh case of familial Mediterranean (Internal Medicine 31: 893-898, 1992) fever reported in Japan. Key words: recurrent polyserositis, catecholamine, colchicine. pleuritis, peritonitis, hereditary disease
Intr oduction Familial Mediterranean fever (FMF) is a hereditary disease of unknown etiology with recurrent febrile attacks accompanied by polyserositis , including arthritis , occurring at irregular intervals. This disease is almost specific to the Mediterranean area or individuals of Mediterranean ancestry, and it is therefore very rare in Japan. Recently, however, several cases of FMF have been reported in Japan. We also encountered a case of FMF showing urine catecholamine elevation during the febrile phase which was completely suppressed by daily colchicine.Case Report A 55-year-old male, who had a seven-year history of periodic fever accompanied by chest, back or abdominal pain, entered our hospital on August 10, 1987. He had been a cattle breeder before and had never lived or travelled abroad. He had no case history of serious illness, and his family had no history of consanguineous marriage. His elder sister has diabetus mellitus and
He had experienced febrile attacks of over 38°C, occurring at irregular intervals 1-6 times a month, since the age of 48. He had noted that chest, back or abdominal pain simultaneously appeared with the fever since he was 49. He consulted several physicians, but the cause of his symptoms remained unclear. He entered a While in twice the hospital, a fever of 38-39°Cinwith shivering hospital for general examinations 1983 and 1986. developed suddenly once or twice a week. Most febrile attacks were accompanied by chest, back or abdominal pain. Untreated, the fever and painCRP lasted 1-2recognized days. Obvious leucocytosis and positive were in the febrile phase, but there were no remarkable findings in the afebrile phase. Despite systemic investi gations, there was no evidence of infectious diseases, including brucellosis, malignant diseases or collagen diseases. Antibiotics and non-steroidal analgestic agents were not effective. He was treated with three tuberculo statics for 4 weeks because of a positive tuberculin skin test, but spike fever continued. Corticosteroids were also ineffective. At the time of admission to our hospital, his physical condition was within normal limits. But on the 4th day, a fever of 38.8°C appeared with abdominal distension. The laboratory findings at that time (Table 1) showed remarkable inflammatory changes: leukocytosis, an in-
similar periodic fever accompanied by abdominal pain. His younger brother has paroxysmal nocturnal From the Department of Internal Medicine, Juntendo University School of Medicine and the *Juntendo Medical College of Nursing, Tokyo hemoglobinuria. Received for publication August 19, 1991; Accepted for publication March 13, 1992 Reprint requests should be addressed to Dr. Mayumi Takahashi, the Department of Internal Medicine, Juntendo University School of Medi 2-1-1 Hongo, Bunkyo-ku, Tokyo 113, Japan Internal Medicine
Vol. 31, No. 7 (July
1992)
893
Takahashi Table
1.
Laboratory
A fe b rile p h a se W B C E o sin o . R B C H b H t PLT E SR CR P C H 5( Ig G Ig A Ig M Ig E A LP G O T G PT L D H y- G T P T -B il D -B il B U N C re a t.
Findings F e b rile p h as e
4 4 0 0 /w l 1% 5 19 x lO 4 / u l
1 0 1 0 0 //il 2 % 5 3 0 x 10 4 M
1 4 .4 g /d l 4 3 .7 % 3 4 .0 x 1 04 /m l
14 .9 g /d l 4 4 .9 % 2 8 .1 x lO 4 M
1 9 /4 1 m m 0 .3 m g /d l 4 4 .7 15 0 8 m g /d l 3 9 0 m g /d l 16 1 m g /d l 2 2 TU /m l 7 .7 15 10 291 25 0 .6 0 .3 ll 0 .9
K -A U IU /1 IU /1 IU /1 U /1 m g /d l m g /d l m g /d l m g /d l
7 2 /10 7 m m 1 5 .4 m g /d l 6 1 .8 1467 401 157 25
m g /d l m g /d l m g /d l IU /m l
10 .8 17 13 273 47 1 .3 0 .6 15 0 .9
K -A U IU /1 I U /1 I U /1 U /1 m g /d l m g /d l m g /d l m g /d l
creased erythrocyte sedimentation rate and positive CRP. However, these symptoms promptly disappeared in less than 24 hours without any treatment. Thereafter, the patient had frequent febrile attacks accompanied by inflammatory findings and abdominal, chest and back
Fig.
894
1.
Clinical
et al pain (Fig. 1). Table 2 shows the findings of the major laboratory tests conducted during the patient's stay in the hospital. There was no evidence of infectious disease or collagen disease. Abdominal echography, computed tomographic scans of chest, abdomen and brain, gastro intestinal roentgenography, barium enema, intravenous pyelography and systemic gallium scintigraphy were also performed. Buta we could notoffind malignant diseases. Table 3 shows comparison theany serum and urine data for corticosteroids, catecholamines and etiocholanolone between a febrile phase and an afebrile phase. The febrile phase showed elevation of urine catecholamines, especially noradrenaline, but etiocholanolone remained within normal limits. At first, we could not define the cause of the fever, but finally noted that the attacks appeared simultaneously with pleuritis on chest X-ray films. Figure 2 is a chest X ray film taken on the day of admission. On the 7th day, a fever of 38.8°C and right chest pain appeared. The picture on the left of Fig. 3 is an X-ray film taken at that time. An abnormal shadow suggestive of a pleural lesion, was seen in the right lower field. On the 13th day, he had a fever of 38.7°C and back pain. The picture on the right of Fig. 3 was taken on the 15th day. The previous shadow has clearly improved, but a new, different shadow, due to the latest attack, was found in the right lower field. The picture on the left of Fig. 4 is an X-ray taken We noted pleural effusion in the right costophrenic on the 19th day when he reported fever and back pain. angle. The fever and back pain lasted less than 24 hours. The abnormal chest shadow disappeared from the film
course after
admission.
Internal
Medicine
Vol. 31, No. 7 (July
1992)
A Case of Familial Table 2. c o ld a g g lu tin in < 4 C o o m b s' te st d ir ec t ( - ) in d ire c t (- ) R A te st (- ) L E te st (- ) A NA
Intr oduction Familial Mediterranean fever (FMF) is a hereditary disease of unknown etiology with recurrent febrile attacks accompanied by polyserositis , including arthritis , occurring at irregular intervals. This disease is almost specific to the Mediterranean area or individuals of Mediterranean ancestry, and it is therefore very rare in Japan. Recently, however, several cases of FMF have been reported in Japan. We also encountered a case of FMF showing urine catecholamine elevation during the febrile phase which was completely suppressed by daily colchicine.Case Report A 55-year-old male, who had a seven-year history of periodic fever accompanied by chest, back or abdominal pain, entered our hospital on August 10, 1987. He had been a cattle breeder before and had never lived or travelled abroad. He had no case history of serious illness, and his family had no history of consanguineous marriage. His elder sister has diabetus mellitus and
He had experienced febrile attacks of over 38°C, occurring at irregular intervals 1-6 times a month, since the age of 48. He had noted that chest, back or abdominal pain simultaneously appeared with the fever since he was 49. He consulted several physicians, but the cause of his symptoms remained unclear. He entered a While in twice the hospital, a fever of 38-39°Cinwith shivering hospital for general examinations 1983 and 1986. developed suddenly once or twice a week. Most febrile attacks were accompanied by chest, back or abdominal pain. Untreated, the fever and painCRP lasted 1-2recognized days. Obvious leucocytosis and positive were in the febrile phase, but there were no remarkable findings in the afebrile phase. Despite systemic investi gations, there was no evidence of infectious diseases, including brucellosis, malignant diseases or collagen diseases. Antibiotics and non-steroidal analgestic agents were not effective. He was treated with three tuberculo statics for 4 weeks because of a positive tuberculin skin test, but spike fever continued. Corticosteroids were also ineffective. At the time of admission to our hospital, his physical condition was within normal limits. But on the 4th day, a fever of 38.8°C appeared with abdominal distension. The laboratory findings at that time (Table 1) showed remarkable inflammatory changes: leukocytosis, an in-
similar periodic fever accompanied by abdominal pain. His younger brother has paroxysmal nocturnal From the Department of Internal Medicine, Juntendo University School of Medicine and the *Juntendo Medical College of Nursing, Tokyo hemoglobinuria. Received for publication August 19, 1991; Accepted for publication March 13, 1992 Reprint requests should be addressed to Dr. Mayumi Takahashi, the Department of Internal Medicine, Juntendo University School of Medi 2-1-1 Hongo, Bunkyo-ku, Tokyo 113, Japan Internal Medicine
Vol. 31, No. 7 (July
1992)
893
Takahashi Table
1.
Laboratory
A fe b rile p h a se W B C E o sin o . R B C H b H t PLT E SR CR P C H 5( Ig G Ig A Ig M Ig E A LP G O T G PT L D H y- G T P T -B il D -B il B U N C re a t.
Findings F e b rile p h as e
4 4 0 0 /w l 1% 5 19 x lO 4 / u l
1 0 1 0 0 //il 2 % 5 3 0 x 10 4 M
1 4 .4 g /d l 4 3 .7 % 3 4 .0 x 1 04 /m l
14 .9 g /d l 4 4 .9 % 2 8 .1 x lO 4 M
1 9 /4 1 m m 0 .3 m g /d l 4 4 .7 15 0 8 m g /d l 3 9 0 m g /d l 16 1 m g /d l 2 2 TU /m l 7 .7 15 10 291 25 0 .6 0 .3 ll 0 .9
K -A U IU /1 IU /1 IU /1 U /1 m g /d l m g /d l m g /d l m g /d l
7 2 /10 7 m m 1 5 .4 m g /d l 6 1 .8 1467 401 157 25
m g /d l m g /d l m g /d l IU /m l
10 .8 17 13 273 47 1 .3 0 .6 15 0 .9
K -A U IU /1 I U /1 I U /1 U /1 m g /d l m g /d l m g /d l m g /d l
creased erythrocyte sedimentation rate and positive CRP. However, these symptoms promptly disappeared in less than 24 hours without any treatment. Thereafter, the patient had frequent febrile attacks accompanied by inflammatory findings and abdominal, chest and back
Fig.
894
1.
Clinical
et al pain (Fig. 1). Table 2 shows the findings of the major laboratory tests conducted during the patient's stay in the hospital. There was no evidence of infectious disease or collagen disease. Abdominal echography, computed tomographic scans of chest, abdomen and brain, gastro intestinal roentgenography, barium enema, intravenous pyelography and systemic gallium scintigraphy were also performed. Buta we could notoffind malignant diseases. Table 3 shows comparison theany serum and urine data for corticosteroids, catecholamines and etiocholanolone between a febrile phase and an afebrile phase. The febrile phase showed elevation of urine catecholamines, especially noradrenaline, but etiocholanolone remained within normal limits. At first, we could not define the cause of the fever, but finally noted that the attacks appeared simultaneously with pleuritis on chest X-ray films. Figure 2 is a chest X ray film taken on the day of admission. On the 7th day, a fever of 38.8°C and right chest pain appeared. The picture on the left of Fig. 3 is an X-ray film taken at that time. An abnormal shadow suggestive of a pleural lesion, was seen in the right lower field. On the 13th day, he had a fever of 38.7°C and back pain. The picture on the right of Fig. 3 was taken on the 15th day. The previous shadow has clearly improved, but a new, different shadow, due to the latest attack, was found in the right lower field. The picture on the left of Fig. 4 is an X-ray taken We noted pleural effusion in the right costophrenic on the 19th day when he reported fever and back pain. angle. The fever and back pain lasted less than 24 hours. The abnormal chest shadow disappeared from the film
course after
admission.
Internal
Medicine
Vol. 31, No. 7 (July
1992)
A Case of Familial Table 2. c o ld a g g lu tin in < 4 C o o m b s' te st d ir ec t ( - ) in d ire c t (- ) R A te st (- ) L E te st (- ) A NA
