Postgraduate Medicine
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Adult-Onset Still's Disease Ira Jeffry Strumpf MD & Jeffrey D. Lazar MD To cite this article: Ira Jeffry Strumpf MD & Jeffrey D. Lazar MD (1975) Adult-Onset Still's Disease, Postgraduate Medicine, 58:4, 174-181, DOI: 10.1080/00325481.1975.11714179 To link to this article: http://dx.doi.org/10.1080/00325481.1975.11714179
Published online: 07 Jul 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=ipgm20 Download by: [Flinders University of South Australia]
Date: 04 September 2016, At: 06:15
case report ADULT-ON SET STILL'S DISEASE Variant of Rheumatoid Arthritis A 26-year-old man was admitted to University Hospital, Ann Arbor, Michigan, in February 1975 for evaluation of polyarthritis and fever of unknown origin. The patient had been weil until early August 1974, when he had bad severe pharyngitis, with chills, night sweats, and arthralgia in the shoulders and right elbow. In mid August, he had entered another hospital because of a ten-day history of sore throat; chills; fever spiking as high as 105 F daily, accompanied by a transient nonpruritic rash on the trunk and extremities; and increased arthralgia in the shoulders, elbows, and hips. He denied having had chorea. Findings on physical examination at that time bad been reported to be normal, except for nonexudative pharyngitis. Results of the joint examination bad not been recorded. A battery of laboratory tests• bad ali been negative or within normal limits except for an elevated white blood celi count (21,000 /cu mm with a shift to the left), elevated antistreptolysin-0 titer (333 Todd units), elevated cold agglutinin titer (returned to normal after antibiotic therapy), and two (of five) positive tests (weakly homogeneous patterns) for antinuclear antibodies. The liver-spleen scan, gallium scan, intravenous cholangiogram, intravenous pyelogram, lymphangiogram, and lumbar puncture had ali given normal results. On the 13th hospital day, the patient bad bad acute onset of pleuritic pain, with pericardial friction rub and atrial Butter-fibrillation. A chest x-ray film showed an enlarged cardiac shadow, bilateral pulmonary infiltrates, and a pleuropericardial effusion. The patient was digitalized and treated with heparin for what the attending physicians believed to be pulmonary emboli. Subsequent pericardiocentesis yielded nonclotting blood.
IRA JEFFRY STRUMPF, MD JEFFREY D. LAZAR, MD University of Michigan Medical Center Ann Arbor
•Latex fixation test for rheumatoid factor, lupus ery· thematosus (LE) cell test, VDRL test for syphilis, ag· glutination tests for Salmonella, Bruce/la, and tularemia, Weil-Felix reaction, numerous blood and urine cultures, mononucleosis spot test, test for hepatitis·associated antigen.
The patient had remained seriously ill for severa! weeks, and his weight dropped from 168 to 118 lb. With hyperalimentation he gained 16 lb by the time of discharge. The chest x-ray film and electrocardiogram returned to normal, but the patient continued to have a daily spiking fever, intermittent migratory joint pain and swelling, and leukocytosis. The serum glutamic oxalacetic transaminase leve! was slightly elevated, although the results of other liver function tests and a percutaneous liver biopsy were normal. After a thorough workup to determine the cause of the persistent fever, an exploratory laparotomy was recommended. The patient refused the operation, choosing instead to seek further evaluation. On admission to University Hospital, the patient appeared to be chronicaliy ill but in no acute distress. There was diffuse thinning of scalp haie. A solitary node was found in the right axilla, and the left wrist was swollen and tender. No other joint abnormalities were found. Laboratory values were: hemoglobin 11.3 gm/100 ml, hematocrit 38 vol %, and white celis 17,000/cu mm, with a normal differentiai. The streptococcal exoenzyme (Streptozyme) titer was 1: 100. Serum glutamic oxalacetic and glutamic pyruvic transaminase levels were minimally elevated, the bilirubin value was normal, and the lactic dehydrogenase value was 1Y2 times normal. Tests for hepatitis-associated antigen and antibody were negative. Arthrocentesis of the left wrist yielded 3 cc of viscous yellow to amber fluid, with 89,000 red celis and 73,000 white celis (94% polymorphonuclear neutrophils). On the second hospital day, single fever
174
POSTGRADUATE MEDICINE
o
October 1975 • Vol. 58
o
No. 4
~~--------------------------------------------right shoulder that lasted three or four days, but
spikes to as high as 103 F began to occur daily. They were associated with a salmon-colored, erythematous, maculopapular, discrete-to-confluent rash on the trunk and upper extremities. The rash always resolved within a few hours after defervescence and was nonpruritic. During the second hospital week, the patient's right knee became painful and swollen, and aspiration yielded 30 cc of viscous clear to light yellow fluid. Analysis of the fluid showed it to be noninflammatory, with no red cells and 600 white cells per cubic millimeter (40% polymorphonuclear neutrophils and 60% monocytes). The mucin clot was fair and no inclusions or crystals were present. The joint fluid hemolytic complement value was 84 units; on a simultaneous determination, the serum complement leve! was 214 units 3 SD above laboratory mean). The peripheral blood white cell count was 20,000/ cu mm. The bane marrow was hypercellular, with eosinophilia and normal iron stores. The sedimentation rate, reported to have been as high as 108 mm/br when the patient was not hospitalized, was 51 mm. The C-reactive protein was 3 +. One of three tests for the presence of antinuclear antibody was positive, with a speckled pattern. Two LE cell tests were negative. A latex fixation test and a test for anti-DNA antibody were negative. Serum protein electrophoresis showed elevated alpha-2 globulin and gamma globulin levels. On immunodiffusion, serum concentration of IgG was increased. On a repeat determination, streptococcal exoenzyme ti ter was elevated to 1: 300, but a throat culture was negative for group A beta hemolytic streptococci. The ophthalmologic evaluation showed no evidence of iridocyclitis but suggested the possibility of a cataract. Therapy with acetylsalicylic acid (6 gm daily) was started. After one week, the patient became afebrile, bad no joint complaints, and was discharged from the hospital. At follow-up after two weeks, he bad gained 15 lb and showed no evidence of joint effusion, and the alopecia was resolving: The sedimentation rate was 84 mm/br. When seen after six weeks, he bad continued to gain weight. He bad bad one episode of weakness and soreness of the
Still's disease, the explosive, systemic, and seronegative form of juvenile rheumatoid artbritis, is a condition familiar to pediatricians. When the disease presents in the older patient, however, the diagnosis is frequently overlooked. Instead, an extensive evaluation is undertaken for fever of unknown origin, which as in this patient, is frequendy the presenting symptom.1.2 Diagnosis and institution of appropriate therapy are thus delayed unnecessarily. In the adult, arthritis is a sine qua non for the diagnosis of rheumatoid arthritis. In adultonset Still's disease, fever and constitutional symptoms (fatigue, malaise, weight loss, myalgia, and arthralgia) may precede the development of arthritis by months or even years. 1 After extensive studies have shawn that an infectious agent or a hematologie disorder is not responsible for the fever, the differenciation of adult-onset Still's disease from rheumatic fever and systemic lupus erythematosus (SLE) remains to be made. The initial clue to the diagnosis of adultonset Still's disease is the fever pattern. McMinn and Bywaters8 and others have described it as quotidian, an intermittent fever that occurs daily. In SLE, the course of the fever is less chaotic; in rheumatic fever, the temperature tends to remain elevated. A second clue to the diagnosis is the evanescent, salmon-colored, maculopapular, nonpruritic rash. 1 •2 Kôbner's phenomenon (isomorphic lesions at the site of trauma) often may be elicited when the rash is present. The rash is quite distinct from the erythema marginatum of rheumatic fever and from the malar butterfly erythema of SLE. Other clinical findings are common to aU three of these diseases. Pulmonary infiltrates, pleural effusions, and pleuritic chest pain may occur. An acute process not unlike pulmonary emboli
176
POSTGRADUATE MEDICINE
he did not remember having omitted his medication. A physical examination showed sorne bogginess of the left wrist. Sedimentation rate was 36 mm/hr, white cell count 20,950/cu mm, and hemoglobin leve! 12.5 gm/100 ml. Discussion
o
October 1975
o
Vol. 58
o
No. 4
case report-----------or infective pneumonitis is occasionally seen. Cardiac murmurs, suggesting endocarditis, and a pericardial friction rub, indicating pericarditis, are most often associated with acute rheumatic fever, but they may also be present in adultonset Still's disease. Although arrhythmias and valvular incompetence have been reported in juvenile rheumatoid arthritis in children and in rheumatoid arthritis in adults, a specifie type or presentation of carditis has not been identified in adult-onset Still's disease per se. As in the case presented, pharyngitis may precede the development of adult-onset Still's disease. It may be associated with an elevated antistreptolysin-0 titer, however, and thus may be confused with the streptococcal pharyngitis associated with rheumatic fever. Alopecia, another clinical characteristic of Still's disease, is also seen in SLE. Unfortunately, studies of joint aspirate may not permit differentiation of adult-onset Still's disease from rheumatic fever or SLE. Ali three conditions are inflammatory processes characterized by leukocytosis, normal joint fluid glucose levels, absence of crystals, normal mucin clot, and negative cultures. Although certain tests assist in differentiating among the three diseases, the findings may be merely supportive rather than diagnostic. Usually, normocytic-normochromic anemia is present, without evidence of hemolysis. Hematocrit readings range from 25 % to 35 % and hemoglobin levels from 8 to 10 gm/100 ml. The white blood cell count tends to be increased in adultonset Still's disease and rheumatic fever and decreased in SLE. The sedimentation rate is elevated in all three conditions, but in rheumatic fever the rate tends to rise during the fever spikes, and in Still's disease it is occasionally only borderline and may even be normaP The presence of LE cells, a positive antinuclear antibody test, and a high titer of anti-DNA antibodies virtually establishes the diagnosis of SLE. In reported cases of adult-onset Still's disease, the LE cell test has been negative. 1 •2 Positive tests for antinuclear antibody and associated iridocyclitis have been reported in children with Still's disease. 4 •5 However, Schaller and associates5 have reported specifie anti-DNA and
178
~~:------------------
Singlet® decongestant - analgesie 40 mg. phenylephrlne hydrochloride 8 mg. chlorpheniramine maleate 500 mg. acetaminophen
INDICATIONS: Singlet is indicated for the symptomatic treatment of colds, sinusitis, allergie rhinitis, and influenza complicated by congestion, rhinorrhea, fever, headache, myalgia and ether painful states.
CONTRAINDICAnONS: Hypersensltivity to any of the formula Ingredients.
PRECAUnONS: Singlet contains a syinpathomimetic agent and should be used cautiously in individuals wlth severe hypertension, diabetes mellitus, hyperthyroidism or urinary retention. The antihistaminic agent may cause drowsiness, and ambulatory patients who operate machinery or motor vehicles should be cautlcined accordingly.
anti-RNA antibody factors to be absent. The depressed serum hemolytic complement level is another factor that differentiates SLE from adult-onset Still's disease and rheumatic fever. In Still's disease and rheumatic fever the level is elevated.6 •7 Synovial fluid complement level bas been reported to be depressed in rheumatie fever and SLE but normal in children with Still's disease. 8 Levels of synovial fluid complement in adults with Still's disease have not yet been reported. Finally, an important characteristic of adultonset Still's disease is that the rheumatoid factor is reported to be negative or present only in very low titer.1 •2 Initial therapy of adult-onset Still's disease consists of the use of salicylates to tolerance. If this regimen fails, steroid therapy, usually with prednisone, is used. Despite the severity of the constitutional symptoms, the long-term prognosis for these patients is good and joint deformity is uncommon.1 •2 Address reprint requests to Ira ]effry Strumpf, MD, Department of Internai Medicine, University Hospital, 1405 E Ann St, Ann Arbor, MI 48104.
REFERENCES 1. Bujak JS, Aptekar RG, Decker JL, et al: Juvenile
2. 3.
DOSAGE AND ADMINISTRAnON: Adults, one Singlet tablet three or four times daily. The interval between doses, however, should not be less than six hours, and not more th an one Singlet should be taken at one ti me. To assure continuous release of medication do not break or crush tablets.
4. 5.
6. 7. 8.
4•8;• DOW PHARMACEUTICALS The Dow Chemical Company Indianapolis, IN 46268
rheumatoid arthritis in the adult as fever of unknown origin. Medicine 52:431, 1973 Bywaters EG: Still's disease in the adult. Ann Rheum Dis 30:121, 1971 McMinn FJ, Bywaters EG: Differences between fever of Still's disease and that of rheumatic fever. Ann Rheum Dis 18:293, 1959 Petty RE, Cassidy JT, Sullivan DB: Oinical correlates of antinuclear antibodies in juvenile rheurnatoid arthritis. J Pediatr 83:386, 1973 &haller JG, Johnson GD, Holborow EJ, et al: The association of antinuclear antibodies with chronic iridocyclitis of juvenile rheumatoid arthritis (Still' s disease). Arthritis Rheum 17:409, 1974 Cooper NR, Fogel BJ: Complement in normal and disease processes. J Pediatr 70:982, 1967 Hoyeraal HM, Mellbye 0 J: High levels of serum complement factors in juvenile rheumatoid arthritis. Ann Rheum Dis 33:243, 1974 Bunch TW, Hunder GG, McDuffie FC, et al: Synovial fluid complement determination as a diagnostic aid in inflarnmatory joint disease. Mayo Oin Proc 49:715, 1974
POSTGRADUATB MllDICINB invites submission of brief case reports for early publication. Illustrations and references should be included only when essential.
181
ISSN: 0032-5481 (Print) 1941-9260 (Online) Journal homepage: http://www.tandfonline.com/loi/ipgm20
Adult-Onset Still's Disease Ira Jeffry Strumpf MD & Jeffrey D. Lazar MD To cite this article: Ira Jeffry Strumpf MD & Jeffrey D. Lazar MD (1975) Adult-Onset Still's Disease, Postgraduate Medicine, 58:4, 174-181, DOI: 10.1080/00325481.1975.11714179 To link to this article: http://dx.doi.org/10.1080/00325481.1975.11714179
Published online: 07 Jul 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=ipgm20 Download by: [Flinders University of South Australia]
Date: 04 September 2016, At: 06:15
case report ADULT-ON SET STILL'S DISEASE Variant of Rheumatoid Arthritis A 26-year-old man was admitted to University Hospital, Ann Arbor, Michigan, in February 1975 for evaluation of polyarthritis and fever of unknown origin. The patient had been weil until early August 1974, when he had bad severe pharyngitis, with chills, night sweats, and arthralgia in the shoulders and right elbow. In mid August, he had entered another hospital because of a ten-day history of sore throat; chills; fever spiking as high as 105 F daily, accompanied by a transient nonpruritic rash on the trunk and extremities; and increased arthralgia in the shoulders, elbows, and hips. He denied having had chorea. Findings on physical examination at that time bad been reported to be normal, except for nonexudative pharyngitis. Results of the joint examination bad not been recorded. A battery of laboratory tests• bad ali been negative or within normal limits except for an elevated white blood celi count (21,000 /cu mm with a shift to the left), elevated antistreptolysin-0 titer (333 Todd units), elevated cold agglutinin titer (returned to normal after antibiotic therapy), and two (of five) positive tests (weakly homogeneous patterns) for antinuclear antibodies. The liver-spleen scan, gallium scan, intravenous cholangiogram, intravenous pyelogram, lymphangiogram, and lumbar puncture had ali given normal results. On the 13th hospital day, the patient bad bad acute onset of pleuritic pain, with pericardial friction rub and atrial Butter-fibrillation. A chest x-ray film showed an enlarged cardiac shadow, bilateral pulmonary infiltrates, and a pleuropericardial effusion. The patient was digitalized and treated with heparin for what the attending physicians believed to be pulmonary emboli. Subsequent pericardiocentesis yielded nonclotting blood.
IRA JEFFRY STRUMPF, MD JEFFREY D. LAZAR, MD University of Michigan Medical Center Ann Arbor
•Latex fixation test for rheumatoid factor, lupus ery· thematosus (LE) cell test, VDRL test for syphilis, ag· glutination tests for Salmonella, Bruce/la, and tularemia, Weil-Felix reaction, numerous blood and urine cultures, mononucleosis spot test, test for hepatitis·associated antigen.
The patient had remained seriously ill for severa! weeks, and his weight dropped from 168 to 118 lb. With hyperalimentation he gained 16 lb by the time of discharge. The chest x-ray film and electrocardiogram returned to normal, but the patient continued to have a daily spiking fever, intermittent migratory joint pain and swelling, and leukocytosis. The serum glutamic oxalacetic transaminase leve! was slightly elevated, although the results of other liver function tests and a percutaneous liver biopsy were normal. After a thorough workup to determine the cause of the persistent fever, an exploratory laparotomy was recommended. The patient refused the operation, choosing instead to seek further evaluation. On admission to University Hospital, the patient appeared to be chronicaliy ill but in no acute distress. There was diffuse thinning of scalp haie. A solitary node was found in the right axilla, and the left wrist was swollen and tender. No other joint abnormalities were found. Laboratory values were: hemoglobin 11.3 gm/100 ml, hematocrit 38 vol %, and white celis 17,000/cu mm, with a normal differentiai. The streptococcal exoenzyme (Streptozyme) titer was 1: 100. Serum glutamic oxalacetic and glutamic pyruvic transaminase levels were minimally elevated, the bilirubin value was normal, and the lactic dehydrogenase value was 1Y2 times normal. Tests for hepatitis-associated antigen and antibody were negative. Arthrocentesis of the left wrist yielded 3 cc of viscous yellow to amber fluid, with 89,000 red celis and 73,000 white celis (94% polymorphonuclear neutrophils). On the second hospital day, single fever
174
POSTGRADUATE MEDICINE
o
October 1975 • Vol. 58
o
No. 4
~~--------------------------------------------right shoulder that lasted three or four days, but
spikes to as high as 103 F began to occur daily. They were associated with a salmon-colored, erythematous, maculopapular, discrete-to-confluent rash on the trunk and upper extremities. The rash always resolved within a few hours after defervescence and was nonpruritic. During the second hospital week, the patient's right knee became painful and swollen, and aspiration yielded 30 cc of viscous clear to light yellow fluid. Analysis of the fluid showed it to be noninflammatory, with no red cells and 600 white cells per cubic millimeter (40% polymorphonuclear neutrophils and 60% monocytes). The mucin clot was fair and no inclusions or crystals were present. The joint fluid hemolytic complement value was 84 units; on a simultaneous determination, the serum complement leve! was 214 units 3 SD above laboratory mean). The peripheral blood white cell count was 20,000/ cu mm. The bane marrow was hypercellular, with eosinophilia and normal iron stores. The sedimentation rate, reported to have been as high as 108 mm/br when the patient was not hospitalized, was 51 mm. The C-reactive protein was 3 +. One of three tests for the presence of antinuclear antibody was positive, with a speckled pattern. Two LE cell tests were negative. A latex fixation test and a test for anti-DNA antibody were negative. Serum protein electrophoresis showed elevated alpha-2 globulin and gamma globulin levels. On immunodiffusion, serum concentration of IgG was increased. On a repeat determination, streptococcal exoenzyme ti ter was elevated to 1: 300, but a throat culture was negative for group A beta hemolytic streptococci. The ophthalmologic evaluation showed no evidence of iridocyclitis but suggested the possibility of a cataract. Therapy with acetylsalicylic acid (6 gm daily) was started. After one week, the patient became afebrile, bad no joint complaints, and was discharged from the hospital. At follow-up after two weeks, he bad gained 15 lb and showed no evidence of joint effusion, and the alopecia was resolving: The sedimentation rate was 84 mm/br. When seen after six weeks, he bad continued to gain weight. He bad bad one episode of weakness and soreness of the
Still's disease, the explosive, systemic, and seronegative form of juvenile rheumatoid artbritis, is a condition familiar to pediatricians. When the disease presents in the older patient, however, the diagnosis is frequently overlooked. Instead, an extensive evaluation is undertaken for fever of unknown origin, which as in this patient, is frequendy the presenting symptom.1.2 Diagnosis and institution of appropriate therapy are thus delayed unnecessarily. In the adult, arthritis is a sine qua non for the diagnosis of rheumatoid arthritis. In adultonset Still's disease, fever and constitutional symptoms (fatigue, malaise, weight loss, myalgia, and arthralgia) may precede the development of arthritis by months or even years. 1 After extensive studies have shawn that an infectious agent or a hematologie disorder is not responsible for the fever, the differenciation of adult-onset Still's disease from rheumatic fever and systemic lupus erythematosus (SLE) remains to be made. The initial clue to the diagnosis of adultonset Still's disease is the fever pattern. McMinn and Bywaters8 and others have described it as quotidian, an intermittent fever that occurs daily. In SLE, the course of the fever is less chaotic; in rheumatic fever, the temperature tends to remain elevated. A second clue to the diagnosis is the evanescent, salmon-colored, maculopapular, nonpruritic rash. 1 •2 Kôbner's phenomenon (isomorphic lesions at the site of trauma) often may be elicited when the rash is present. The rash is quite distinct from the erythema marginatum of rheumatic fever and from the malar butterfly erythema of SLE. Other clinical findings are common to aU three of these diseases. Pulmonary infiltrates, pleural effusions, and pleuritic chest pain may occur. An acute process not unlike pulmonary emboli
176
POSTGRADUATE MEDICINE
he did not remember having omitted his medication. A physical examination showed sorne bogginess of the left wrist. Sedimentation rate was 36 mm/hr, white cell count 20,950/cu mm, and hemoglobin leve! 12.5 gm/100 ml. Discussion
o
October 1975
o
Vol. 58
o
No. 4
case report-----------or infective pneumonitis is occasionally seen. Cardiac murmurs, suggesting endocarditis, and a pericardial friction rub, indicating pericarditis, are most often associated with acute rheumatic fever, but they may also be present in adultonset Still's disease. Although arrhythmias and valvular incompetence have been reported in juvenile rheumatoid arthritis in children and in rheumatoid arthritis in adults, a specifie type or presentation of carditis has not been identified in adult-onset Still's disease per se. As in the case presented, pharyngitis may precede the development of adult-onset Still's disease. It may be associated with an elevated antistreptolysin-0 titer, however, and thus may be confused with the streptococcal pharyngitis associated with rheumatic fever. Alopecia, another clinical characteristic of Still's disease, is also seen in SLE. Unfortunately, studies of joint aspirate may not permit differentiation of adult-onset Still's disease from rheumatic fever or SLE. Ali three conditions are inflammatory processes characterized by leukocytosis, normal joint fluid glucose levels, absence of crystals, normal mucin clot, and negative cultures. Although certain tests assist in differentiating among the three diseases, the findings may be merely supportive rather than diagnostic. Usually, normocytic-normochromic anemia is present, without evidence of hemolysis. Hematocrit readings range from 25 % to 35 % and hemoglobin levels from 8 to 10 gm/100 ml. The white blood cell count tends to be increased in adultonset Still's disease and rheumatic fever and decreased in SLE. The sedimentation rate is elevated in all three conditions, but in rheumatic fever the rate tends to rise during the fever spikes, and in Still's disease it is occasionally only borderline and may even be normaP The presence of LE cells, a positive antinuclear antibody test, and a high titer of anti-DNA antibodies virtually establishes the diagnosis of SLE. In reported cases of adult-onset Still's disease, the LE cell test has been negative. 1 •2 Positive tests for antinuclear antibody and associated iridocyclitis have been reported in children with Still's disease. 4 •5 However, Schaller and associates5 have reported specifie anti-DNA and
178
~~:------------------
Singlet® decongestant - analgesie 40 mg. phenylephrlne hydrochloride 8 mg. chlorpheniramine maleate 500 mg. acetaminophen
INDICATIONS: Singlet is indicated for the symptomatic treatment of colds, sinusitis, allergie rhinitis, and influenza complicated by congestion, rhinorrhea, fever, headache, myalgia and ether painful states.
CONTRAINDICAnONS: Hypersensltivity to any of the formula Ingredients.
PRECAUnONS: Singlet contains a syinpathomimetic agent and should be used cautiously in individuals wlth severe hypertension, diabetes mellitus, hyperthyroidism or urinary retention. The antihistaminic agent may cause drowsiness, and ambulatory patients who operate machinery or motor vehicles should be cautlcined accordingly.
anti-RNA antibody factors to be absent. The depressed serum hemolytic complement level is another factor that differentiates SLE from adult-onset Still's disease and rheumatic fever. In Still's disease and rheumatic fever the level is elevated.6 •7 Synovial fluid complement level bas been reported to be depressed in rheumatie fever and SLE but normal in children with Still's disease. 8 Levels of synovial fluid complement in adults with Still's disease have not yet been reported. Finally, an important characteristic of adultonset Still's disease is that the rheumatoid factor is reported to be negative or present only in very low titer.1 •2 Initial therapy of adult-onset Still's disease consists of the use of salicylates to tolerance. If this regimen fails, steroid therapy, usually with prednisone, is used. Despite the severity of the constitutional symptoms, the long-term prognosis for these patients is good and joint deformity is uncommon.1 •2 Address reprint requests to Ira ]effry Strumpf, MD, Department of Internai Medicine, University Hospital, 1405 E Ann St, Ann Arbor, MI 48104.
REFERENCES 1. Bujak JS, Aptekar RG, Decker JL, et al: Juvenile
2. 3.
DOSAGE AND ADMINISTRAnON: Adults, one Singlet tablet three or four times daily. The interval between doses, however, should not be less than six hours, and not more th an one Singlet should be taken at one ti me. To assure continuous release of medication do not break or crush tablets.
4. 5.
6. 7. 8.
4•8;• DOW PHARMACEUTICALS The Dow Chemical Company Indianapolis, IN 46268
rheumatoid arthritis in the adult as fever of unknown origin. Medicine 52:431, 1973 Bywaters EG: Still's disease in the adult. Ann Rheum Dis 30:121, 1971 McMinn FJ, Bywaters EG: Differences between fever of Still's disease and that of rheumatic fever. Ann Rheum Dis 18:293, 1959 Petty RE, Cassidy JT, Sullivan DB: Oinical correlates of antinuclear antibodies in juvenile rheurnatoid arthritis. J Pediatr 83:386, 1973 &haller JG, Johnson GD, Holborow EJ, et al: The association of antinuclear antibodies with chronic iridocyclitis of juvenile rheumatoid arthritis (Still' s disease). Arthritis Rheum 17:409, 1974 Cooper NR, Fogel BJ: Complement in normal and disease processes. J Pediatr 70:982, 1967 Hoyeraal HM, Mellbye 0 J: High levels of serum complement factors in juvenile rheumatoid arthritis. Ann Rheum Dis 33:243, 1974 Bunch TW, Hunder GG, McDuffie FC, et al: Synovial fluid complement determination as a diagnostic aid in inflarnmatory joint disease. Mayo Oin Proc 49:715, 1974
POSTGRADUATB MllDICINB invites submission of brief case reports for early publication. Illustrations and references should be included only when essential.
181
