243
CLINICAL ROUNDS
DEFORMING ARTHRITIS OF THE HANDS IN POLYMYOSITIS THOMAS W. BUNCH, J. DESMOND O’DUFFY, and RICHARD A . McLEOD Arthritis of the hands with erosions, periosteal calcification, and interphalangeal thumb joint instability was seen in 6 patients with polymyositis. “Overlap” features such as Raynaud’s phenomenon, positive LE clot test, and positive antinuclear antibody test were present, but clinically the primary disease was clearly polymyositis. This rather unusual constellation of roentgenographic findings strongly suggests the possibility of polymyositis.
to the hands. The diagnosis of polymyositis was based on proximal muscle weakness with elevated levels of serum creatine phosphokinase; electromyographic findings showing typical low-amplitude, polyphasic potentials with normal nerve conduction time; and muscle biopsy showing mononuclear cell infiltration with degenerating and regenerating fibers. Other clinical, laboratory, and roentgenographic data were obtained to clarify further the extent and type of arthritis (Tables 1 and 2).
Arthralgias and arthritis have previously been reported to occur in polymyositis; however reports of destructive changes have been uncommon. This report describes 6 patients who had deforming arthritis confined to the hands and emphasizes that this feature can occur in polymyositis.
RESULTS
MATERIALS AND METHODS From January 1971 t o July 1974, the authors saw 6 patients with polymyositis who had unusual arthritis confined From the Division of Rheumatology and Internal Medicine and the Department of Diagnostieoentgenology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Thomas W . Bunch, M.D.: Consultant, Division of Rheumatology and Internal Medicine, and Instructor in Medicine, Mayo Medical School; J . Desmond O’DufTy, M.B., B. Ch.: Consultant. Division of Rheumatology and Internal Medicine, and Assistant Professor of Medicine, Mayo Medical School; Richard A. McLeod, M.D.: Consultant, Department of Diagnostic Roentgenology. and lnstructor in Radiology, Mayo Medical School. Address reprint requests to Thomas W. Bunch, M.D., Rheumatology, Mayo Clinic, 200 First Street, S. W., Rochester, Minnesota 55901, Accepted for publication November 12, 1975. Arthritis and Rheumatism, Vol. 19, No. 2 (March-April 1976)
All patients had their disease for at least 1 year (range: 1-12 years). The diagnosis of polymyositis was confirmed in the first 5 cases; all 5 patients had characteristic proximal muscle weakness, elevated levels of serum creatine phosphokinase, and abnormal electromyographic and muscle biopsy findings. The sixth patient had erythema of the knuckles, extensive calcification within the muscles of the arms, proximal muscle weakness, and abnormal findings on an electromyogram; she refused to undergo a muscle biopsy. N o patient had arthritis in joints other than the hands, although 3 had knee arthralgia. Three patients had roentgenograms of the foot but these showed no erosions. Only 1 patient had demonstrable rheumatoid factor in the serum. One patient (Case 4) fulfilled three criteria for rheumatoid arthritis ( 1 ) but had biopsy evidence of polymyositis; no other joints besides digital ones were involved. I n 5 of the 6 patients a characteristic finding was a “floppy thumb sign” consisting of extreme lateral instability of the interphalangeal joint.
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BUNCH ET AL
Table 1. Clinical and Laboratory Features of 6 Paiients wiih Polymyositis
Case Age/Sex
Weakness
No. of ARA* Duration Criteria of MYOPathy (yrs) RA( I ) SLE(2)
I
69/M
Moderate
8
2
2
2
52/F
Moderate
2
2
2
3
54/F
Moderate
2
2
2
4
54/F
Mild
3
3
2
5
40/F
Moderate
I1
2
I
6
74/F
Mild
I2
0
1
* American
Electromyogram Inflammatory niyopathy Myopathy Inflammatory myopathy Inflammatory myopathy Inflammatory myopathy Myopathy
Muscle Biopsy
Highest Creatine Phosphokinase U/1+
Inflammatory myopathy Mild inflammatory myopathy Inflammatory myopathy Plasma cells around vessels; myopathy Inflammatory myopathy ND
Lupus RheumaAnti- Erythematoid nuclear tows Factor Antibody Clot
440
Neg
ND$
ND
536
Neg
Neg
ND
484
1:160
1:256
Pos
715
Neg
1:32
Pos
536
Neg
1:64
Neg
44
Neg
1:512
ND
Rheumatism Association.
t Normal levels in males < 71 U/I, in females
CLINICAL ROUNDS
DEFORMING ARTHRITIS OF THE HANDS IN POLYMYOSITIS THOMAS W. BUNCH, J. DESMOND O’DUFFY, and RICHARD A . McLEOD Arthritis of the hands with erosions, periosteal calcification, and interphalangeal thumb joint instability was seen in 6 patients with polymyositis. “Overlap” features such as Raynaud’s phenomenon, positive LE clot test, and positive antinuclear antibody test were present, but clinically the primary disease was clearly polymyositis. This rather unusual constellation of roentgenographic findings strongly suggests the possibility of polymyositis.
to the hands. The diagnosis of polymyositis was based on proximal muscle weakness with elevated levels of serum creatine phosphokinase; electromyographic findings showing typical low-amplitude, polyphasic potentials with normal nerve conduction time; and muscle biopsy showing mononuclear cell infiltration with degenerating and regenerating fibers. Other clinical, laboratory, and roentgenographic data were obtained to clarify further the extent and type of arthritis (Tables 1 and 2).
Arthralgias and arthritis have previously been reported to occur in polymyositis; however reports of destructive changes have been uncommon. This report describes 6 patients who had deforming arthritis confined to the hands and emphasizes that this feature can occur in polymyositis.
RESULTS
MATERIALS AND METHODS From January 1971 t o July 1974, the authors saw 6 patients with polymyositis who had unusual arthritis confined From the Division of Rheumatology and Internal Medicine and the Department of Diagnostieoentgenology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota. Thomas W . Bunch, M.D.: Consultant, Division of Rheumatology and Internal Medicine, and Instructor in Medicine, Mayo Medical School; J . Desmond O’DufTy, M.B., B. Ch.: Consultant. Division of Rheumatology and Internal Medicine, and Assistant Professor of Medicine, Mayo Medical School; Richard A. McLeod, M.D.: Consultant, Department of Diagnostic Roentgenology. and lnstructor in Radiology, Mayo Medical School. Address reprint requests to Thomas W. Bunch, M.D., Rheumatology, Mayo Clinic, 200 First Street, S. W., Rochester, Minnesota 55901, Accepted for publication November 12, 1975. Arthritis and Rheumatism, Vol. 19, No. 2 (March-April 1976)
All patients had their disease for at least 1 year (range: 1-12 years). The diagnosis of polymyositis was confirmed in the first 5 cases; all 5 patients had characteristic proximal muscle weakness, elevated levels of serum creatine phosphokinase, and abnormal electromyographic and muscle biopsy findings. The sixth patient had erythema of the knuckles, extensive calcification within the muscles of the arms, proximal muscle weakness, and abnormal findings on an electromyogram; she refused to undergo a muscle biopsy. N o patient had arthritis in joints other than the hands, although 3 had knee arthralgia. Three patients had roentgenograms of the foot but these showed no erosions. Only 1 patient had demonstrable rheumatoid factor in the serum. One patient (Case 4) fulfilled three criteria for rheumatoid arthritis ( 1 ) but had biopsy evidence of polymyositis; no other joints besides digital ones were involved. I n 5 of the 6 patients a characteristic finding was a “floppy thumb sign” consisting of extreme lateral instability of the interphalangeal joint.
244
BUNCH ET AL
Table 1. Clinical and Laboratory Features of 6 Paiients wiih Polymyositis
Case Age/Sex
Weakness
No. of ARA* Duration Criteria of MYOPathy (yrs) RA( I ) SLE(2)
I
69/M
Moderate
8
2
2
2
52/F
Moderate
2
2
2
3
54/F
Moderate
2
2
2
4
54/F
Mild
3
3
2
5
40/F
Moderate
I1
2
I
6
74/F
Mild
I2
0
1
* American
Electromyogram Inflammatory niyopathy Myopathy Inflammatory myopathy Inflammatory myopathy Inflammatory myopathy Myopathy
Muscle Biopsy
Highest Creatine Phosphokinase U/1+
Inflammatory myopathy Mild inflammatory myopathy Inflammatory myopathy Plasma cells around vessels; myopathy Inflammatory myopathy ND
Lupus RheumaAnti- Erythematoid nuclear tows Factor Antibody Clot
440
Neg
ND$
ND
536
Neg
Neg
ND
484
1:160
1:256
Pos
715
Neg
1:32
Pos
536
Neg
1:64
Neg
44
Neg
1:512
ND
Rheumatism Association.
t Normal levels in males < 71 U/I, in females
