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Mutilans-like arthropathy in mixed connective tissue disease.

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1013
CASE REPORTS
MUTILANS-LIKE ARTHROPATHY IN MIXED CONNECTIVE
TISSUE DISEASE
DONATO ALARCON-SEGOVIA and OSCAR URIBE-URIBE
Patients who combine clinical features of various
connective tissue disorders have been found t o have
antibodies to ribonucleoprotein. This antigen is included as the ribonuclease-sensitive portion of an extractable nuclear antigen (ENA) (1). These patients
have been grouped under the term mixed connective tissue disease (MCTD), and there is increasing evidence
that they constitute a distinct disease group. Articular
manifestations in patients with MCTD are prominent
and may be destructive t o the point of closely mimicking rheumatoid arthritis (2-4). Herein we describe a
unique patient with MCTD who developed severe arthritis, as well as middle and distant phalangeal bone
resorption which resulted in opera-glass hands akin t o
those found in arthritis mutilans.
CASE REPORT
This 32-year-old female had a 10-year history of arthralgia with swelling which began at the wrists and later involved elbows, shoulders, hips, knees, and ankles. She also
noticed the appearance of marked swelling of all fingers. Limitation of motion as well as brief morning stiffness was present. Shortly thereafter she began to notice the appearance of
subcutaneous nodules on the dorsum of the hands over the extensor tendons of the fingers. At that time low grade fever also
occurred and pain and swelling on the left parotid gland developed. Weight loss and generalized muscle weakness developed. Three years after the onset of her illness, she noticed
tightening of the facial skin with loss of elasticity and diminished buccal opening. At that time three-color-phase Raynaud's phenomenon, sensitivity of the skin to sun exposure,
and abundant hair loss were also noted. Three years before
admission she developed dryness of the mouth, decreased
From the Department of Immunology and Rheumatology,
lnstituto Nacional de la Nutricion, Mexico City, Mexico.
Donato Alarcon-Segovia, MD: Professor and Chairman, Department of Immunology and Rheumatology; Oscar Uribe-Uribe,
MD: Fellow in Rheumatology.
Submitted for publication March 16, 1979; accepted in revised form April 4, 1979.
Address reprint requests to Donato Alarcon-Segovia, MD,
lnstituto Nacional de la Nutrition, Avenida San Fernando y Viaducto Tlalpan, Mexico 22, DF, Mexico.
Arthritis and Rheumatism, Vol. 22. No. 9 (September 1979)
lacrimation with stinging of the eyes, and a tendency to ulnar
deviation at the metacarpophalangeal (MCP) joints, as well as
shortening of the fingers, particularly at the portions distal to
the proximal interphalangeal joints.
Two years before admission she developed shortness
of breath and retrosternal pain which increased upon deep
breathing. She was placed on dexamethasone 2 mg per day
with some improvement. One year later a slight trauma to her
feet resulted in bilateral malleolar ulcerations.
On physical examination the face was typical of
scleroderma patients with smooth, taut, shiny, hardened skin.
There was sharpening of the nose with small telangiectasia.
Thickening of the skin involved the neck, trunk, arms, and
thighs. The hair was scant, short at the forehead, and lusterless. There was marked conjunctival irritation with minute
corneal ulcerations. The submandibular glands were enlarged
and slightly painful. The buccal opening was decreased, the
mouth was dry with scant thickened saliva, and the tongue
was smooth and shiny. She had bilateral, small firm nodes on
the neck and left supraclavicular area. Axillary and pubic hair
was scant. The liver was palpable 4 cm below the right costal
margin, as was the spleen at 6 cm below the left costal margin.
Both temporomandibular joints were tender. She had
a grade I synovitis of both elbows with a 15 degree limitation
of extension. The wrists were swollen with synovitis and a
grade 111 limitation of motion. The hands showed interosseous muscle atrophy and ulnar deviation at the metacarpophalangeal joints. The fingers were shortened (Figure 1) but
the excess skin permitted elongation of all of them with an opera-glass hand effect when pulled by their tips (Figure 2).
Shortening of the fingers was apparently due to loss of the second phalanges. The skin of the hands was normal thus permitting stretching of the fingers. Synovitis was also found at
the knees, where there was normal joint motion and n o effusion. The ankles were swollen but could reflect the presence of
deep, sharply limited ulcerations in both internal malleoli
which were secondarily infected and had a diameter of 5 to 6
cm. On the right side, the ulceration extended to the back of
the ankle reaching the Achilles tendon. There was tenderness
of the metacarpophalangeal joints and the toes had hammer
deformities. Peripheral pulses were normal.
There was no family history of similar illness. She had
had the main childhood diseases including mumps and a tonsillectomy at age 2 1 because of repeated sore throats.
Laboratory studies revealed a blood hemoglobin of
9.6 gm/dl with hematocrit of 32 mm. She had 4000 leukocytes/pl with 22% lymphocytes, 7% monocytes, 71% neutrophils. Blood urea nitrogen, serum creatinine, serum uric acid,
ALARCON-SEGOVIA A N D URIBE-URIBE
1014
Figure 1. The patient’s hands.
serum transaminases, blood glucose, serum calcium and phosphate, and serum alkaline phosphatase levels were all normal.
Serum protein electrophoresis showed a total protein of 9.2
gm with albumin of 4.42 gm,alpha- 1 globulins of 0.28 gm, alpha-2 globulins of 0.66 gm, beta globulins of 0.77 gm, and
gamma globulins of 3.07 gm per dl.
Antinuclear antibodies studied by immunofluorescence on frozen rat stomach and kidney sections gave a
4+ speckled pattern to a titer of I : 1280 and a faint peripheral
pattern. Testing of antinuclear antibodies by complement fixation revealed the serum to be anticomplementary to a titer of
I :4. Rheumatoid factor as determined by the Singer-Plotz
method (5) was positive at a titer of 1:20,480. Complement
studies revealed a CH50 of 13.15 units/ml (normal 30-65
units/ml), a C3 level of 36 mg/dl (normal 80-120 mg/dl), and
a C4 level of 18 mg/dl (normal 10-15 mg/dl). Serum immunoglobulins were determined on commercial antibody-agar
plates (Boehringwerke A. G. Marburg, West Germany). IgG
Figure 2. Elongation of a finger by pulling it by the tip; a, resting, b, pulled out.
MUTILANS-LIKE ARTHROPATHY
1015
Figure 3. Roentgenograms of the hands in a patient with MCTD and mutilans-like arthropathy. Notice erosive changes at the
styloids, wrists, and MCP joints, as well as the resorption of the second and third phalanges.
Figure 4. Various degrees of resorption of the distal phalanx in 3 fingers. The second phalanx is completely resorbed while the distal end of the first
phalanx shows only small erosions.
1016
ALARCON-SEGOVIA AND URIBE-URIBE
tarsophalangeal joints. Interestingly, the distal end of the second phalanx of the first toes was resorbed.
Pulmonary function tests showed a moderate restrictive pattern. Scintillation scanning of salivary glands revealed
diminished uptake by all major salivary glands as compared
to the thyroid.
The patient remained hospitalized for 47 days. She
initially received only salicylates and antibiotics for the secondary infection of the malleolar ulcerations. During hospitalization she developed bilateral parotid inflammation and
pleuritic pain. Because of this she was started on 15 mg of
prednisone daily; she has had no arthralgia, and the liver and
spleen diminished in size to become barely palpable on this
regimen. Attempts to decrease the dose of corticosteroids have
been unsuccessful because of recurrence of systemic symptoms.
DISCUSSION
Figure 5. The loss of the proximal end of the distal phalanx of the
thumbs permitted the wrinkled, “rolled-up’’ effect.
was 3,948 mg. IgA 439 mg, and IgM 859 mg/dl. The serum
relative viscosity determined in an Ostwald viscometer was of
2.4 units (normal less than 1.9 units). Antibodies to extractable nuclear antigen were determined by microhemagglutination with calf thymus antigen (ENA) freshly
prepared in our laboratory as described earlier (4). The initial
serum gave a hemagglutination titer of 1 :400, while with the
ribonuclease-treated ENA the titer became negative. The test
was repeated after the patient was placed on corticosteroids.
The titer had then risen to 1 :200,000 with untreated ENA, but
remained negative with ribonuclease-treated ENA.
Esophageal manometric studies done as previously
described (6) showed hypomotility of the lower third of the
esophagus. An electrocardiogram was normal. Biopsy of the
skin of the thigh revealed changes compatible with scleroderma. Biopsy of the lip showed lymphocytic infiltration of
minor salivary glands. HLA typing was B18- and B2I-positive.
Roentgenogram of the thorax showed changes compatible with slight interstitial fibrosis of both lungs and a
parotid sialogram revealed grade I1 sialectasia. Roentgenograms of the hands and wrists (Figure 3) revealed marked
osteoporosis and erosive changes at the styloid processes,
wrists, and MCP joints. The second phalanges were practically completely resorbed with remnants found only in those
of the thumbs. The proximal end of the distal phalanges was
also resorbed, leaving distal remnants of various sizes (Figure
4). Soft tissues of the fingers revealed the wrinkling of the
overlying skin characteristic of the “lorgnette” effect (Figure
5). Roentgenograms of the feet showed erosive changes in the
calcaneocuboid, talonavicular, and tarsometatarsal and meta-
The patient fulfills our previously proposed criteria for MCTD (4,7,8). She has had arthritis with palpable synovitis, scleroderma, three-color-phase Raynaud’s phenomenon, edema of the hands, and may have
had myopathy as evidenced by her history of muscle
weakness. Besides having had 3 or 4 of the 5 cardinal
symptoms of MCTD, she had evidence of at least three
connective tissue diseases: rheumatoid arthritis-synovitis, joint deformity, bone erosions, rheumatoid factor;
systemic lupus erythematosus-alopecia, frontal hair
shortening (9), photosensitivity, leukopenia, oral ulcerations; and progressive systemic sclerosis-scleroderma,
telangectasia, pulmonary fibrosis, hypoperistalsis of the
lower esophagus. She also had symptomatic Sjogren’s
syndrome, a frequent occurrence in our patients with
MCTD(7).
Antibodies to ENA were positive, first at low titers and, following the onset of corticosteroid treatment,
at the high titers required by the postulated criteria for
MCTD (4,7,8). Appearance of antibodies to ENA following the initiation of corticosteroid treatment has
been described in MCTD (10). Studies with ribonuclease-treated ENA showed that the antibodies present
in the serum of our patient were directed to the ribonuclease-sensitive portion of this antigen: nuclear ribonucleoprotein.
Patients with psoriatic arthritis (1 1) and an occasional patient with rheumatoid arthritis (12) may have
marked destruction of joint surfaces, which instead of
causing ankylosis may cause finger shortening and hypermobility of the joints. This has been termed arthritis
mutilans. When finger shortening is extreme, the over-
MUTILANS-LIKE ARTHROPATHY
lying skin may wrinkle and allow the fingers to be
stretched out by pulling their distal portion. This effect
of arthritis mutilans is called opera-glass hand or mainen-lorgnette.
Because the typical opera-glass hand effect in
our patient was due to total resorption of the middle
and partial resorption of the distal phalanges, we prefer
to call our patient’s hand findings “mutilans-like.” Also,
despite the presence of an arthritis (synovitis), we could
not be certain that this synovitis caused the phalangeal
resorption; therefore we preferred to call the mutilans
component of it arthropathy rather than arthritis. Damage to the joints was present, but whether it was caused
by the synovitis was impossible to judge. Indeed, we
consider it rather unlikely that such damage was due to
the synovitis, in view of the paucity of damage to the
distal end of the proximal phalanges of the hands. Bone
resorption is part of the picture of scleroderma, and although it is more often distal (13) it may occur at other
sites such as the angle of the mandible (14) and the ribs
(1 5). Interestingly, the lorgnette effect was allowed by
the fact that the scleroderma which affected the face,
neck, trunk, and proximal portions of the extremities
spared the hands, causing no loss of elasticity of the skin
of the fingers.
There was no evidence of psoriasis in our patient. There were no nail changes which are practically
uniformly present when arthritis mutilans is due to psoriasis (16). Furthermore, our patient was HLA-B13,
Bw17, and Bw37 negative, HLA phenotypes which associate with psoriatic arthritis (17). The loss of entire
phalanges in our patient resembles what occurs in
idiopathic multicentric osteolysis ( 18). This could, however, be ruled out by the absence of carpal or tarsal
osteolysis, nephropathy, or family history (18).
The course of the disease indicates that the hand
changes were part of the picture of MCTD. There has
been a case of probable arthritis mutilans in a patient
with MCTD described recently (3). The patient seemed
to be different from ours in that there was pencil and
cup destruction of 2 distal interphalangeal joints of each
hand that resulted in finger shortening, more closely resembling. psoriatic arthritis than did our patient. However, it is possible that the patient described by Bennett
and O’Connell (3) represents an early stage of the joint
destruction due to bone resorption that we found in our
patient.
Patients with MCTD tend to have more severe
joint involvement than patients with the other con-
1017
nective tissue diseases, with the exception of rheumatoid
arthritis. Indeed, if exclusions are not considered, up to
86% of patients with MCTD may fulfill criteria for classic or definite rheumatoid arthritis (4). This is compounded by the frequent finding of rheumatoid factor
and the occasional finding of rheumatoid nodules in patients with mixed connective tissue disease (4).
REFERENCES
1. Sharp GC,
Irvin WS, Tan EM, Gould RG, Holman HR:
Mixed connective tissue disease-an apparently distinct
rheumatic disease syndrome associated with a specific
antibody to an extractable nuclear antigen (ENA). Am J
Med 52:148-158, 1972
2. Halla JM, Hardin JG: Clinical features of the arthritis of
mixed connective tissue disease. Arthritis Rheum 2 1:497503, 1978
3. Bennett RM, OConnell DJ: The arthritis of the mixed
connective tissue disease. Ann Rheum Dis 37:397-403,
I978
4. Ramos-Niembro F, Alarcon-Segovia D, Hernandez-Ortiz
J: Articular manifestations of mixed connective tissue disease. Arthritis Rheum 22:43-5 1, 1979
5. Singer JM, Plotz CM: Latex fixation test: application to
the serologic diagnosis of rheumatoid arthritis. Am J Med
2 1 :888-892, 1956
6. Ramirez-Mata M, Ibaliez G, Alarcon-Segovia D: Stimulatory effect of metoclopramide on the esophagus and
lower esophageal sphincter of patients with PSS. Arthritis
Rheum 20:30-34, 1977
7. Alarcon-Segovia D: Symptomatic Sjogren’s syndrome in
mixed connective tissue disease. J Rheumatol 3: 191-195,
1976
8. Diaz-Jouanen E, Llorente L, Ramos-Niembro F, Alarcon-Segovia D: Cold-reactive lymphocytotoxic antibodies
in mixed connective tissue disease. J Rheumatol 4:4- 10,
1977
9. Alarcon-Segovia D, Cetina JA: Lupus hair. Am J Med Sci
267~241-242,1974
10. Ramos-Niembro F, Alarcon-Segovia D: Familial aspects
of mixed connective tissue disease (MCTD). I. Occurrence of systemic lupus erythematosus in another member
in two families and aggregation of MCTD in another
family. J Rheumatol5:433-440, 1978
1 I . Avila R, Pugh DG, Slocumb CH, Winkelmann RK: Psoriatic arthritis: a roentgenologic study. Radiology 75:69 1702, 1960
12. Buchanan WW: Clinical features of rheumatoid arthritis,
Copeman’s Textbook of the Rheumatic Diseases. Fifth
edition. Edited by JT Scott. Edinburgh, Churchill Livingstone, 1978, pp 329-33 1
ALARCON-SEGOVIA AND URIBE-URIBE
1018
13. Rabinowitz JG, Tweresky J, Guttadauria M: Similar bone
manifestations of scleroderma and rheumatoid arthritis.
Radiology 121:35-44, 1974
14. Seifert MH, Steigerwald JC, Clif MM: Bone resorption of
the mandible in progressive systemic sclerosis. Arthritis
Rheum 18:507-512, 1975
15. Steigerwald JC, Seifert MH, Cliff MM, Neff TA: Bone resorption of ribs and pulmonary function in progressive
systemic sclerosis. Chest 68:838-840, 1975
16. Leonard DG, O'Duffy JD, Rogers RS 111: Prospective
analysis of psoriatic arthritis in patients hospitalized for
psoriasis. Mayo Clin Proc 535 I 1-5 18, 1978
17. Sasazuki T, McDevitt HO, Grumet FC: The association
between genes in the major histocompatibility complex
and disease susceptibility. Ann Rev Med 28~425452,1977
18. Whyte MP, Murphy WA, Kleerekoper M, Teitelbaum
SL, Avioli LV: Idiopathic multicentric osteolysis. Arthritis
Rheum 21:367-376, 1978
GLOMERULONEPHRITIS IN RHEUMATOID ARTHRITIS
JAMES A. DAVIS, ARTHUR H. COHEN, RICHARD WEISBART, and HAROLD E. PAULUS
Glomerulonephritis is rarely a complication of
rheumatoid arthritis (RA) despite the observation of
significant amounts of circulating immune complexes in
both glomerulonephritis and RA (1). Among 5,232 patients with a diagnosis of RA, hospitalized at the UCLA
Center for the Health Sciences from 1955 to 1977, glomerulonephritis was diagnosed in only 4 patients, all of
whom also met diagnostic criteria for systemic lupus
erythematosus or mixed connective tissue disease. We
present a well documented case of proliferative glomerulonephritis in association with RA in the absence of
another immunologically mediated disease.
CASE REPORT
A 57-year-old white male customs inspector first developed RA in 1969. The RA was well controlled with nonsteroidal antiinflammatory drugs until August 1974, when he
was admitted to a community hospital with a flare of RA associated with fever, marked malaise, minimal proteinuria, and
microscopic hematuria. Laboratory results (Table 1) revealed
no source for fever other than RA. He started prednisone 40
From the Department of Medicine, UCLA School of Medicine, Los Angeles, California, and Department of Pathology, Harbor
General Hospital, Torrance, California.
Supported in part by the Thomas and Dorothy Leavey Trust.
James A. Davis, MD; Arthur H. Cohen, MD; Richard Weisbart, MD; Harold E. Paulus, MD.
Address reprint requests to James A. Davis, MD, loo0 Veteran Avenue, Los Angeles, California 90024.
Submitted for publication February 23, 1979; accepted May
9, 1979.
Arthritis and Rheumatism, Vol. 22, No. 9 (September 1979)
mg daily and received 200 mg of aurothiomalate with no
change in clinical status and was then admitted to Sepulveda
Veterans Administration Hospital in October 1974. Physical
examination revealed temperature 101.4'F, generalized moderately active RA, nodules over both elbows, small left pleural
effusion, and mild hepatomegaly without splenomegaly. Examination included a liver-spleen scan that showed mild
hepatomegaly, normal liver biopsy, and a bone marrow examination that showed mild granulocytic hyperplasia. He was
treated with prednisone 15 mg per day and aspirin 5.4 gm per
day, with improvement of the RA and resolution of the hematuna and proteinuria. Aurothiomalate was resumed in November 1974. From November 1974 to November 1976 he received 1985 mg of aurothiomalate but then was again noted to
have hematuria and proteinuria which were attributed to gold
induced nephropathy. An IVP and cystography showed normal results. In November 1976 he was first noted to have the
murmur of aortic insufficiency. Numerous blood cultures revealed negative results and the murmur was attributed to RA.
Although gold therapy was stopped, hematuria and
proteinuria persisted during the next year and serum creatinine increased from 1.3 to 2.1 mg/dl. Therefore, he was admitted to Sepulveda Veterans Administration Hospital in January 1978 for a kidney biopsy.
The patient had no signs or symptoms of Sjogren's
syndrome, cutaneous vasculitis, butterfly rash, alopecia,
photosensitivity, or Raynaud's phenomenon.
Physical examination. Temperature was 97.8'F, blood
pressure 130/55, pulse 90. No rash was evident; numerous
rheumatoid nodules were found over the extensor surface of
the elbows, achilles tendons, and fingers. Swan neck deformities of several fingers were observed and there was decreased range of motion of the wrists, elbows, shoulders, cervical spine, and ankles. Minimal swelling was present in
almost all the small joints of the hands and feet, wrists, el-
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