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This case illustrates that giant cell arteritis can occur in nonwhites. Hence, this disease should definitely be considered in any person, regardless of race, when the clinical symptoms and physical findings suggest it. DONALD T. KING,MD JAMESF. FREED,MD Harbor General Hospital Torrance, Calfornia 90509 REFERENCES 1. Healey LA, Wilske KR: Manifestations of giant cell arteri-
tis. Med Clin North Am 61:261-270, 1977 2. Terasaki PI, Healey LA, Wilske KR: Distribution of HLA haplotypes in polymyalgia rheumatica. N Engl J Med 295:905, 1976 3. Goodman LJ, Layfer LF, Banner BF: Polymyalgia rheumatic and giant-cell arteritis in a black woman. N Engl J Med 298:113, 1978 4. Dirnant J, Farmer PM, Sobol N: Giant cell arteritis in a black person. Arthritis Rheum 21:391-393, 1978 5 . Ballou SP, Khan MA, Kushner I: Giant-cell arteritis in a black patient. Ann Intern Med 88:659-660, 1978 6. Jacobs RC: Polyrnyalgia rheumatica in an American Indian. Arthritis Rheum 20: 1429, 1977
Coexistence of ankylosing spondyli tis and rheumatoid arthritis in a single family To the Editor: In the past several years increasing attention has been paid to the occurrence of ankylosing spondylitis (AS) and rheumatoid arthritis (RA) in the same individual (1-4). In none of the cases reported in the literature so far has either condition been observed to be present in other family members. We have observed a family in which one member was found to have AS and RA, and both conditions were seen separately in other members of the family. HLA typing studies revealed positivity for B27 in all members affected with a rheumatic condition and in 50% of the first degree relatives. The index patient is a 53-year-old white male painter followed at the Rheumatic Disease Unit (RDU) of the Royal Victoria Hospital. He was first seen at the RDU in September 1973 because of pain and stiffness of neck and lower back. Examination revealed a decreased chest expansion, tenderness, pain on motion, and decreased range of motion of the entire spine. Tenderness was also elicited on pressure of the sacroi-
liac joints. The diagnosis of ankylosing spondylitis was made clinically. X-ray examination showed bilateral sacroiliitis and spondylitic involvement of the entire spine consistent with the diagnosis of AS. HLA typing revealed positivity for B27. Butazolidin 100 mg three times daily was begun. Because of the extraordinary family history of AS, the pedigree was included in the familial study of ankylosing spondylitis that we were conducting at the time (Family JO, Ref. 5). However, shortly after we first saw this patient in our clinic, he developed morning stiffness and a peripheral symmetrical polyarthritis which involved small and large joints. By the end of 1974 he had developed typical manifestations of classic rheumatoid arthritis. Rheumatoid nodules were present, and synovial thickening of wrists, metacarpophalangeal, and proximal interphalangeal joints with bilateral ulnar deviation deformities developed. Rheumatoid factor became positive shortly after the onset of polyarthritis in both synovial fluid and serum. Antinuclear factor was negative. No other significant serologic abnormalities were noted. Biopsy of one of the subcutaneous nodules was consistent with the diagnosis of rheumatoid nodule. X-ray revealed erosive changes on proximal interphalangeal and wrist joints. From the time the patient developed polyarthritis, therapeutic management became very difficult. Aspirin in full doses was given in conjunction with Butazolidin and physical therapy. Because of poor response, gold therapy was begun but had to be discontinued after the patient had received 1 gm because of skin rash and proteinuria. At the beginning of 1975, prednisone 10 mg and azathioprine 100 mg daily were begun. In spite of the medication, the patient’s condition continued to deteriorate. On 200 mg azathioprine he showed improvement, but active synovitis persists. Twenty members were studied. Ankylosing spondylitis was found in one of his siblings (a 46-yearold female) and RA in two others (a 56-year-old female and a 36-year-old male). By history it was concluded that the proband’s deceased maternal grandfather had also had AS. All affected members were B27 positive, as well as 50% of his first degree relatives. This patient illustrates some of the clinical aspects of the concomitant presentation of AS and RA, already reviewed by Good et a1 (4). From the clinical presentation of our patient and the reports in the literature, a pattern is emerging regarding the characteristics of the clinical association of AS and RA. The patient is usually male with a long history of back pain. The ankylos-
204
ing spondylitis seems to occur first in a mild to severe form. Rheumatoid arthritis usually develops during the third or fourth decade of life, and its clinical course is independent of the AS. As far as is known, the presence of B27 does not modify the manifestations of RA on these patients with AS or in the relatives.
L. R. ESPINOZA, MD Section of Rheumatology College of Medicine University of South Florida Tampa, Florida 33612 F. B. DOVE,MD C. KIRKOSTERLAND, MD Rheumatic Disease Unit Royal Victoria Hospital Montreal, P.Q. H3A-IAI Canada REFERENCES I . Rosenthal SH, Lidsky MD, Sharp JT: Arthritis with nodules following ankylosing spondylitis. JAMA 206:28932894, 1968 2. Luthra HS, Ferguson RH, Conn DL: Coexistence of ankylosing spondylitis and rheumatoid arthritis. Arthritis Rheum 19:lII-1l4, 1976 3. Fallet GH, Mason M, Berry H, et al: Rheumatoid arthritis and ankylosing spondylitis occurring together. Br Med J 1:804-807. 1976 4. Good AE, Hyla JF, Rapp R: Ankylosing spondylitis with rheumatoid arthritis and subcutaneous nodules. Arthritis Rheum 20:1434-1437, 1977 5. Espinoza LR, Oh JH, Kinsella, TD, Stacey CH, Osterland CK, Dove FB: Ankylosing spondylitis: familial studies and HLA-27 antigen distribution. J Rheumatol 1:254-258, 1974
Relapsing polychondritis To the Editor: Current recommendations in rheumatology texts for treatment of relapsing polychondritis include systemic corticosteroids (1-3). We wish to report a case of relapsing polychondritis in which systemic corticosteroids were ineffective and the response to Avlosulfon (dapsone) appeared unequivocal. Mrs. C. O., a 37-year-old white female, was seen in the dermatology clinic on June 5, 1977 with a swollen, painful, and red right ear. Initially she had been treated with 2 intramuscular 80 mg doses of Depo-
Medrol 1 week apart and later with Velosef for 1 week with no improvement. Her medical history was of interest because in 1976 she had developed a red, swollen, and tender nose which resolved spontaneously. In February of 1977 a diagnosis of “inflammation of the cartilage of the ribs” was made because of bilateral parasternal pain and tenderness; resolution was spontaneous. The patient denied any history of fever, symptoms of rheumatoid or collagen vascular disease, or cardiovascular symptoms. Results of a physical examination were normal other than marked redness, swelling, and tenderness of the right ear except for the lobe. Laboratory evaluation showed a white blood cell count of 14,700 with 72% polymorphonuclear leukocytes. The erythrocyte sedimentation rate was 43, and fluorescent antinuclear antibody test was negative. Rheumatoid factor was not done. The patient was given Avlosulfon 100 mg twice daily. When seen again on July 18, 1977, the patient reported improvement in the ear after 4 days and no symptoms after 1 week of dapsone. She was seen again on August 15 at which time her ear appeared almost normal, and she had a normal erythrocyte sedimentation rate. The dapsone was discontinued on October 5 with no recurrence of symptoms since. One other patient with relapsing polychondritis has been reported as responding to dapsone (4). The mechanism of response to dapsone in an inflammatory state such as relapsing polychondritis may be inhibition of lysosomal enzymes (5). Considering the significant undesirable side effects of steroids, the relatively infrequent side effects of dapsone, and the frequent ineffectiveness of steroids in relapsing polychondritis, we think a trial of dapsone is warranted, either initially or when systemic corticosteroids have failed to establish control of the inflammatory symptoms. Because polychondritis is rare and unpredictable in its course, the response to dapsone may be difficult to establish, but perhaps further individual case reports will allow assessment of its effectiveness in this disease.
JAMESR. CARPENTER, MD W. L. MACAULAY, MD Departments of Rheumatology and Dermatology Fargo Clinic Fargo, North Dakota
This case illustrates that giant cell arteritis can occur in nonwhites. Hence, this disease should definitely be considered in any person, regardless of race, when the clinical symptoms and physical findings suggest it. DONALD T. KING,MD JAMESF. FREED,MD Harbor General Hospital Torrance, Calfornia 90509 REFERENCES 1. Healey LA, Wilske KR: Manifestations of giant cell arteri-
tis. Med Clin North Am 61:261-270, 1977 2. Terasaki PI, Healey LA, Wilske KR: Distribution of HLA haplotypes in polymyalgia rheumatica. N Engl J Med 295:905, 1976 3. Goodman LJ, Layfer LF, Banner BF: Polymyalgia rheumatic and giant-cell arteritis in a black woman. N Engl J Med 298:113, 1978 4. Dirnant J, Farmer PM, Sobol N: Giant cell arteritis in a black person. Arthritis Rheum 21:391-393, 1978 5 . Ballou SP, Khan MA, Kushner I: Giant-cell arteritis in a black patient. Ann Intern Med 88:659-660, 1978 6. Jacobs RC: Polyrnyalgia rheumatica in an American Indian. Arthritis Rheum 20: 1429, 1977
Coexistence of ankylosing spondyli tis and rheumatoid arthritis in a single family To the Editor: In the past several years increasing attention has been paid to the occurrence of ankylosing spondylitis (AS) and rheumatoid arthritis (RA) in the same individual (1-4). In none of the cases reported in the literature so far has either condition been observed to be present in other family members. We have observed a family in which one member was found to have AS and RA, and both conditions were seen separately in other members of the family. HLA typing studies revealed positivity for B27 in all members affected with a rheumatic condition and in 50% of the first degree relatives. The index patient is a 53-year-old white male painter followed at the Rheumatic Disease Unit (RDU) of the Royal Victoria Hospital. He was first seen at the RDU in September 1973 because of pain and stiffness of neck and lower back. Examination revealed a decreased chest expansion, tenderness, pain on motion, and decreased range of motion of the entire spine. Tenderness was also elicited on pressure of the sacroi-
liac joints. The diagnosis of ankylosing spondylitis was made clinically. X-ray examination showed bilateral sacroiliitis and spondylitic involvement of the entire spine consistent with the diagnosis of AS. HLA typing revealed positivity for B27. Butazolidin 100 mg three times daily was begun. Because of the extraordinary family history of AS, the pedigree was included in the familial study of ankylosing spondylitis that we were conducting at the time (Family JO, Ref. 5). However, shortly after we first saw this patient in our clinic, he developed morning stiffness and a peripheral symmetrical polyarthritis which involved small and large joints. By the end of 1974 he had developed typical manifestations of classic rheumatoid arthritis. Rheumatoid nodules were present, and synovial thickening of wrists, metacarpophalangeal, and proximal interphalangeal joints with bilateral ulnar deviation deformities developed. Rheumatoid factor became positive shortly after the onset of polyarthritis in both synovial fluid and serum. Antinuclear factor was negative. No other significant serologic abnormalities were noted. Biopsy of one of the subcutaneous nodules was consistent with the diagnosis of rheumatoid nodule. X-ray revealed erosive changes on proximal interphalangeal and wrist joints. From the time the patient developed polyarthritis, therapeutic management became very difficult. Aspirin in full doses was given in conjunction with Butazolidin and physical therapy. Because of poor response, gold therapy was begun but had to be discontinued after the patient had received 1 gm because of skin rash and proteinuria. At the beginning of 1975, prednisone 10 mg and azathioprine 100 mg daily were begun. In spite of the medication, the patient’s condition continued to deteriorate. On 200 mg azathioprine he showed improvement, but active synovitis persists. Twenty members were studied. Ankylosing spondylitis was found in one of his siblings (a 46-yearold female) and RA in two others (a 56-year-old female and a 36-year-old male). By history it was concluded that the proband’s deceased maternal grandfather had also had AS. All affected members were B27 positive, as well as 50% of his first degree relatives. This patient illustrates some of the clinical aspects of the concomitant presentation of AS and RA, already reviewed by Good et a1 (4). From the clinical presentation of our patient and the reports in the literature, a pattern is emerging regarding the characteristics of the clinical association of AS and RA. The patient is usually male with a long history of back pain. The ankylos-
204
ing spondylitis seems to occur first in a mild to severe form. Rheumatoid arthritis usually develops during the third or fourth decade of life, and its clinical course is independent of the AS. As far as is known, the presence of B27 does not modify the manifestations of RA on these patients with AS or in the relatives.
L. R. ESPINOZA, MD Section of Rheumatology College of Medicine University of South Florida Tampa, Florida 33612 F. B. DOVE,MD C. KIRKOSTERLAND, MD Rheumatic Disease Unit Royal Victoria Hospital Montreal, P.Q. H3A-IAI Canada REFERENCES I . Rosenthal SH, Lidsky MD, Sharp JT: Arthritis with nodules following ankylosing spondylitis. JAMA 206:28932894, 1968 2. Luthra HS, Ferguson RH, Conn DL: Coexistence of ankylosing spondylitis and rheumatoid arthritis. Arthritis Rheum 19:lII-1l4, 1976 3. Fallet GH, Mason M, Berry H, et al: Rheumatoid arthritis and ankylosing spondylitis occurring together. Br Med J 1:804-807. 1976 4. Good AE, Hyla JF, Rapp R: Ankylosing spondylitis with rheumatoid arthritis and subcutaneous nodules. Arthritis Rheum 20:1434-1437, 1977 5. Espinoza LR, Oh JH, Kinsella, TD, Stacey CH, Osterland CK, Dove FB: Ankylosing spondylitis: familial studies and HLA-27 antigen distribution. J Rheumatol 1:254-258, 1974
Relapsing polychondritis To the Editor: Current recommendations in rheumatology texts for treatment of relapsing polychondritis include systemic corticosteroids (1-3). We wish to report a case of relapsing polychondritis in which systemic corticosteroids were ineffective and the response to Avlosulfon (dapsone) appeared unequivocal. Mrs. C. O., a 37-year-old white female, was seen in the dermatology clinic on June 5, 1977 with a swollen, painful, and red right ear. Initially she had been treated with 2 intramuscular 80 mg doses of Depo-
Medrol 1 week apart and later with Velosef for 1 week with no improvement. Her medical history was of interest because in 1976 she had developed a red, swollen, and tender nose which resolved spontaneously. In February of 1977 a diagnosis of “inflammation of the cartilage of the ribs” was made because of bilateral parasternal pain and tenderness; resolution was spontaneous. The patient denied any history of fever, symptoms of rheumatoid or collagen vascular disease, or cardiovascular symptoms. Results of a physical examination were normal other than marked redness, swelling, and tenderness of the right ear except for the lobe. Laboratory evaluation showed a white blood cell count of 14,700 with 72% polymorphonuclear leukocytes. The erythrocyte sedimentation rate was 43, and fluorescent antinuclear antibody test was negative. Rheumatoid factor was not done. The patient was given Avlosulfon 100 mg twice daily. When seen again on July 18, 1977, the patient reported improvement in the ear after 4 days and no symptoms after 1 week of dapsone. She was seen again on August 15 at which time her ear appeared almost normal, and she had a normal erythrocyte sedimentation rate. The dapsone was discontinued on October 5 with no recurrence of symptoms since. One other patient with relapsing polychondritis has been reported as responding to dapsone (4). The mechanism of response to dapsone in an inflammatory state such as relapsing polychondritis may be inhibition of lysosomal enzymes (5). Considering the significant undesirable side effects of steroids, the relatively infrequent side effects of dapsone, and the frequent ineffectiveness of steroids in relapsing polychondritis, we think a trial of dapsone is warranted, either initially or when systemic corticosteroids have failed to establish control of the inflammatory symptoms. Because polychondritis is rare and unpredictable in its course, the response to dapsone may be difficult to establish, but perhaps further individual case reports will allow assessment of its effectiveness in this disease.
JAMESR. CARPENTER, MD W. L. MACAULAY, MD Departments of Rheumatology and Dermatology Fargo Clinic Fargo, North Dakota
