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Homozygosity for HLA-B27 in psoriatic arthritis and spondylitis.

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vestigated. Four of 82 patients developed ANA while on
oral contraceptives. There were, however, no controls
( I ) . Rodnan found no convincing evidence of internal
involvement in the localized forms of scleroderma or of
overlap between these conditions and progressive systemic sclerosis (2). Dubois has reported 3 patients with
both localized scleroderma and SLE (3). Familial scleroderma is rare; only several documented instances have
been reported (4).
Grunow Memorial Medical Building
926 East McDowell Road
Phoenix, Arizona 85006
Dubois EL: Lupus Erythematosus. Second edition. Los
Angeles, University of Southern California Press, 1974, p
2. Rodnan G P Progressive systemic sclerosis (scleroderma),
Arthritis and Allied Conditions. Eighth edition. Edited by
J L Hollander, DJ McCarty Jr. Philadelphia, Lea & Febiger, 1972, pp 962-1005.
3. Dubois EL, Chandor S, Friou GJ, et al: Progressive systemic sclerosis (PSS) and localized scleroderma (morphea)
with positive LE cell test. Medicine 50199-222. 1900
4. Wuthrich RC, Roenigk HH Jr, Steck WD: Localized
scleroderma. Arch Dermatol 3:98-100, 1900
Homozygosity for HLA-B27 in Psoriatic
Arthritis and Spondylitis
To the Editor:
The Journal published a recent report on homozygosity at the B locus for allele B27 and impact on
rheumatic disease expression ( I ) . We would like to present the case of a young man with severe psoriatic arthritis and spondylitis who is homozygous for HLA-B27.
A 29-year-old French Canadian male developed
psoriasis at age 22 beginning with his elbows, knees, and
scalp and was treated successfully with coal tars. When
he was 25, asymmetric polyarthritis began in his knees
and ankles associated with low back and neck pain and
stiffness. The arthritis progressed, causing him to stop
working at age 26. He was hospitalized in 1974 and his
arthritis was treated with salicylates, with mild improvement. His arthritis and psoriasis both flared in 1976 and
he was again hospitalized. There was no history of
urethritis, uveitis, or conjunctivitis.
Physical examination revealed generalized psoriasis with the exception of his hands. Pitting of his nails
was noted. The complete fusion of his cervical and lumbar spine resulted in marked disability. Little active
synovitis was present, but there were flexion deformities
of both shoulders, elbows, hips, and right knee. In addition there was a severe valgus deformity of his right knee
and left foot.
Laboratory studies showed a hematocrit of 38%.
white blood count of 11,800, and Wintrobe sedimentation rate of 52 mm/hour. His urinalysis, latex fixation,
and antinuclear antibody were negative. HLA typing
revealed the phenotype of AW 31, B27. X-rays of the
axial skeleton demonstrated fusion of the cervical and
lumbar spine and both sacroiliac joints. Asymmetric
erosions were evident in the PIP joints, wrists, shoulders, knees, ankles, and feet. There was narrowing of the
right hip joint, collapse of the right medial tibia1 plateau,
and dislocation of the M P joints of the left foot.
The patient was started on methotrexate 2.5 mg
4 1 2 hr for three doses weekly. This has produced considerable improvement in both his rash and his functional ability.
The patient’s mother, father, and sister were interviewed and examined. None had a history of psoriasis, musculoskeletal complaints, or any abnormalities
on examination. HLA typing revealed his mother to be
AW 25, A W 3 I , B 18, B27: the father and sister were AW
24, AW 31, B7, B27. it is of interest that the patient’s
grandmothers were second cousins. Two way mixed
lymphocyte cultures were performed. There was no reaction between the patient and either of his parents.
This patient had severe axial and peripheral psoriatic arthritis. Of 50 patients with psoriatic arthritis
seen in the past 3 years at our institution he has the most
severe case. He is also the only patient found to be
homozygous for B27. I n addition this patient also demonstrated homozygosity at the A and D loci. The influence of homozygosity for A locus antigens on disease
is unknown. I n fact, disease associations with HLAAW31 antigen have not been described, and none of
our other patients with psoriatic arthritis had this allele.
The possibility of disease associated with D locus antigens exists. A recent paper by Chused showed that Sjo
grens syndrome is primarily associated with HLA-DWj
and only secondarily with HLA B8 (2). However in
anklylosing spondylitis, D locus antigen association has
not been found (3). Similar studies in psoriatic arthritis
have not been done.
Thus definite consideration must be given to our
patient’s homozygosity at the B locus as being significant. HLA-B27 has been reported in 60% of patients
with psoriatic spondylitis but only in 11% of those with
peripheral arthritis alone (4).
A study on British Columbian Coastal Indians
has documented a remarkably high prevalence of anky-
losing spondylitis. Over 50% of the total community had
HLA-B27 (5). A considerable number of these patients
were thought to be homozygous. As in our patient the
homozygosity was thought to result from previous consan gui n i t y.
I t appears that homozygosity for major histocompatibility complex (M HC) antigens, particularly
827, may be associated with more severe psoriatic arthritis and spondylitis. Further family studies on clinical
rheumatic diseases relating severity to the M HC antigens will clarify this point.
Royal Victoria Hospital
687 Pine West
Montreal, Canada H3A I A l
1. Arnett FC, Schacter BZ, Hochberg MC, et al: Homo-
zygosity for HLA-B27: impact on rheumatic disease expression in two families. Arthritis Rheum 20797-804, 1977
2. Chused T M , Kassan SS, Opelz G, et al: Sjogrens syndrome
associated with HLA DW3. N Engl J Med 296:895-897,
3. Sachs JA, Sterioff S, Robinette M, et al: Ankylosing
spondylitis and the major histocompatibility system. Tissue
Antigens 5:120-127, 1975
4. Lambert J R , Wright V, Rajah SM, et al: Histocompatibility antigens in psoriatic arthritis. Ann Rheum Dis
35:526-550. 1976
5 . Gofton JP, Chalmers A, Price GE, et al: HL-A 27 and
ankylosing spondylitis i n B.C. Indians. J Rheurnatol
2:314-318. 1975
Congress on Pediatric Advances
The Associaion Medica del DIF will hold its first Congress on Pediatric Advances on February
9, 10 and 11, 1978 in Mexico City. For information contact: Hospital del Nino DIF, Congress Office,
lnsurgentes Sur 3700, Mexico 22, D.F. Telephone: (905) 573-5348.
Satellite Symposia on the Inflammatory Process
Two symposia concerning the inflammatory process and its treatment will be held in Paris,
France (July 2, 1978) and in Brussels, Belgium (July 24, 1978). Abstracts must be submitted by
March 30, 1978. The symposia are organized in connection with the 7th International Congress of
Pharmacology to be held in Paris.
For information on the symposium on “Anti-inflammatory and Anti-rheumatic Drugs” in Paris,
contact: Professor J. P. Giroud, Department of Pharmacology, 27 rue du Faubourg Saint-Jacques,
C.H.U. Cochin, Paris XIV France.
For the symposium on “The Inflammatory Process” in Brussels, contact: Dr. J. P. Famaey,
Service de Rheumatologie et Physiotherapie, Hopital Universitaire Saint-Pierre, 322 rue Haute,
1000 Bruxelles, Beligique.
Psoriasis Therapy: An International Symposium
This symposium will be held in Israel from February 19-26, 1978, sponsored jointly by the
Departments of Dermatology at Stanford University School of Medicine and Hadassah University
For information, contact: Travelthon Ltd., 1359 Broadway, New York, N.Y. 10018; or Mr. N. D.
Yahalom, 44 Ibn Gviral Street, Tel Aviv, Israel.
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hla, arthritis, psoriatic, b27, spondylitis, homozygosity
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