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Rheumatoid nodulosisReport of a case with evidence of intraosseous rheumatoid granuloma.

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BRIEF REPORT
RHEUMATOID NODULOSIS: REPORT OF A CASE WITH EVIDENCE
OF INTRAOSSEOUS RHEUMATOID GRANULOMA
ANTONIO MORALES-PIGA, ANGEL ELENA-IBANEZ, ANTONIO c. ZEA-MENDOZA,
ANTONIO ROCAMORA-RIPOLL, and JUAN BELTRAN-GUTIERREZ
We describe a patient who had multiple subcutaneous rheumatoid nodules associated with episodes of
acute intermittent arthritis and subchondral cystic lesions of the small bones of the hands and feet; this
coadition is termed “rheumatoid nodulosis.” The patient had a cystic lesion in communication with the joint
cavity, rheumatoid granulomas, and evidence of a central zone of necrosis opening toward the joint space. His
case is compared with 8 previously reported cases, and
possible etiologies of the subchondral bone cyst formation in rheumatoid nodulosis are discussed.
Subcutaneous rheumatoid nodules occur in
20-25% of patients with seropositive rheumatoid arthritis (RA) (l), and histologically identical subcutaneous nodules are seen in other diseases (2). Ginsberg
et a1 (3) were the first to report a symptom complex,
which they termed “rheumatoid nodulosis,” in which
subcutaneous rheumatoid nodules are associated with
episodes of acute intermittent arthritis and subchondral cystic lesions of the small bones of the hands and
feet. There are few histopathologic descriptions of
these cysts ( 4 3 , and they are not always well docuFrom the Rheumatology Service and the Pathology Service, Hospital “Rambn y Cajal,” Madrid, Spain.
Antonio Morales-Piga, MD: Assistant Head, Rheumatology
Service; Angel Elena-IbaAez, MD: Resident, Rheumatology Service; Antonio C. Zea-Mendoza, MD: Assistant Head, Rheumatology Service; Antonio Rocamora-Ripoll, MD: Assistant Head, Pathology Service; Juan Beltrfin-GutiCrrez, MD: Chief, Rheumatology
Service.
Address reprint requests to Dr. Antonio C. Zea-Mendoza,
Servicio de Reumatologia, Hospital “Rambn y Cajal,” Carretera de
Colmenar Km.9, 28034-Madrid, Spain.
Submitted for publication June 19, 1985; accepted in revised form April 12, 1986.
Arthritis and Rheumatism, Vol. 29, No. 10 (October 1986)
mented. For these reasons, their etiology and diagnostic significance remain unclear.
The morphologic findings in the patient with
rheumatoid nodulosis that are presented in this report
might elucidate the origin and significance of subchondral bone cysts in this condition.
Case report. At the age of 35, the patient had
experienced episodes of intermittent arthritis in the
metacarpophalangeal (MCP) and metatarsophalangeal
(MTP) joints and in the knees. The episodes remitted
spontaneously without sequelae within 4-5 days and
recurred at variable intervals ranging from 2-6 weeks.
At that time he also experienced nontender nodular
lesions localized in the hands, elbows, and feet, with
no apparent changes in the overlying skin. Laboratory
findings at that time included positive rheumatoid
factor (RF), erythrocyte sedimentation rate (ESR) 25
mm/hour, and normal values for hemoglobin, white
blood cell (WBC) count, and serum biochemical values including uric acid. A diagnosis of seropositive RA
was made and the patient was begun on a regimen of
nonsteroidal antiinflammatory drugs and gold salts. He
discontinued the gold salts on his own initiative after 4
weeks. His condition remained the same for 12
months; thereafter, the joint symptoms disappeared,
but the nodular lesions persisted.
When he was referred to our unit in 1983, at the
age of 44, the patient presented with intermittent
arthralgia in the MCP and MTP joints and knees with
morning stiffness lasting 2 hours. He denied having
intestinal symptoms or renal lithiasis. He admitted to
drinking 80 gm of alcohol/day. His family history was
unremarkable.
Physical examination revealed 19 nontender
subcutaneous nodules, 0.5-2 cm in diameter, which
BRIEF REPORTS
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Figure 1. Anteroposterior radiograph, showing subchondral geodes in the fifth metatarsal heads of both feet
and at the base of several proximal phalanges. Bilateral augmentation of soft tissue around the first and the
fifth metatarsophalangealjoints, corresponding to subcutaneous nodules, is seen.
were elastic in consistency. These nodules were localized in the digital pads of the fingers and toes, over the
MCP and MTP joints, and at the extensor surface of
both elbows, the dorsal aspect of both wrists, the
anterior region of the ankles, and the tarsus. The
overlying skin was normal, and no signs of joint
swelling or deformity were observed. The liver was
palpable 2 cm below the costal margin. No splenomegaly was observed.
Laboratory studies showed the hemoglobin,
WBC, and platelet counts, and serum biochemistries
obtained by SMAC 20, to be within the normal ranges.
The ESR was 21 mm/hour. Total plasma protein was
7.3 gm/dl; the albumin level was 3.2 gm/dl. Immunoglobulin, lipid, VDRL, and serum complement
values were normal or negative. RF was positive at a
titer of 1 :640; antinuclear antibodies were negative.
Chest radiographic findings were normal.
Roentgenograms of joints revealed increased soft tissue at the sites of subcutaneous nodules and multiple
round, well-defined, juxtaarticular osteolytic lesions,
2-10 mm in diameter, in the bones of the hands and
feet (Figures 1 and 2). These occurred predominantly
in areas near the subcutaneous nodules. No juxtaarticular erosions or demineralization were observed,
and there was minimal joint space narrowing in some
MCP and MTP joints. The subpopulations of circulating lymphocytes were studied with OKT monoclonal
antibodies, revealing a decrease in both absolute and
relative values of OKT8-t cells, with an OKT4+/
OKT8+ ratio of 7.0 (normal 2.01 +- 0.73).
While the patient was in the hospital, the distal
head of the fifth metatarsal bone of his left foot and the
subcutaneous nodule localized over this joint were
resected. Biopsies of the adjacent synovial membrane
and the subcutaneous nodule localized over an MCP
joint were also performed. The surgeon observed that
the nodules were located between the extensor tendons and the joint capsule, infiltrating both structures
as well as the periosteum. Serial sections of the head
of the metatarsal bone showed an intraosseous cavity
communicating with the joint space by a narrow canal.
Several rheumatoid granulomas with a fibrinoid
necrotic center surrounded by histiocytes, occasional
multinucleated giant cells, and focal infiltrates of lymphocytes and plasma cells were observed in the cavity
(Figures 3 and 4). Some sections of this pseudocyst
revealed a necrotic center opening toward the joint
space (Figure 5). Microscopic examination of the
subcutaneous nodules demonstrated the presence of
1280
Figure 2. Detailed view of the patient’s fifth metatarsophalangeal
joint.
typical rheumatoid granulomas. Direct immunofluorescence disclosed intense deposits with fibrinogen in
the center of the granulomas. Examination of the
synovial membrane showed an acute and chronic
inflammatory infiltrate and multiple granulomas resembling those previously described.
Discussion. Patients with subcutaneous rheumatoid nodules range from those with seropositive RA to
those in whom these nodules are the sole clinical
manifestation (2). There are also a small number of
patients in whom the subcutaneous rheumatoid nodules are accompanied by isolated features of RA, such
as rheumatoid lung (6), nodular scleritis (7), or positive
serum RF in the absence of severe joint involvement
(8,9). Ginsberg et a1 proposed the term “rheumatoid
nodulosis” to characterize the clinical presentation of
multiple biopsy-proven subcutaneous rheumatoid
BRIEF REPORTS
nodules, recurrent joint symptoms in the absence of
marked synovitis or systemic illness, and multiple
cystlike intraosseous radiolucencies (3). However, diagnostic criteria have not been established, and the
cases that have been diagnosed as rheumatoid
nodulosis vary among investigators. For this reason,
we extensively reviewed the literature and included all
patients who had the following characteristics: 1)
multiple subcutaneous rheumatoid nodules identified
histologically; 2) recurrent joint symptoms generally
classifiable as palindromic rheumatism; 3) absent or
minimal rheumatoid joint involvement (clinical and
radiologic); 4) no systemic manifestations of RA; 5 )
positive serum RF; and 6 ) subchondral cystic lesions
of small bones of the hands and feet. We found 8
patients with these characteristics described in the
literature (3-5,10-14) (Table 1).
Rheumatoid nodulosis is most common in
middle-aged men with normal or slightly elevated ESR
and a positive serum RF. RF was positive in all
patients reported except in the patient described by
Bywaters in 1949 (4), in whom it was not tested. None
of the described patients seems to have developed
joint or systemic manifestations of RA, after a
followup of as long as 25 years (10,14). The presence
of subchondral cystic lesions without severe erosions
is a striking feature.
Subchondral cyst (geode) formation is not an
uncommon manifestation of RA (15). Pathologic examination of these geodes frequently reveals a communication with the joint space and granulation tissue
inside (16). This confirms the fundamental role of
synovial inflammation in the development of subchondral geodes in RA.
In rheumatoid nodulosis, however, the cause
and significance of the subchondral cystic lesions have
not been established. Thus, it is not clear whether they
represent actual intraosseous rheumatoid nodules or
are formed by synovial tissue infiltration (3,10,11).
Bywaters’ patient underwent bone biopsy, which
showed “a small cyst lying on top of the periosteum
with its neck towards the joint cavity; it was difficult to
be certain whether it communicated with the joint”; in
its interior, the presence of “synovial membrane with
marked proliferation and inflammatory changes” was
seen, but no mention of nodules was made (4). In a
more recent study, the presence of “rheumatoid involvement” was reported, but its characteristics were
not specified (5).
In our patient, the cystic lesion communicated
with the joint cavity. It was not possible to identify
BRIEF REPORTS
Figure 3. Biopsy specimen from subchondral bone of the fifth metatarsal joint, showing a granuloma with a
fibrinoid necrotic center (hematoxylin and eosin stained, original magnification x 360).
Figure 4. Closer view of the biopsy specimen shown in Figure 3, demonstrating the presence of histiocytes
and some multinucleated giant cells, which surround a fibrinoid necrotic center (hematoxylin and eosin
stained, original magnification x 450).
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1282
BRIEF REPORTS
Figure 5. Section of a subchondral pseudocyst with granulomatous tissue in its interior. The necrotic center
opens toward the joint space. Replacement, by pannus, of cartilage adjacent to the stoma can also be
observed (hematoxylin and eosin stained, original magnification x 300).
synovial tissue in this cyst; however, several rheumatoid granulomas were seen (Figures 3 and 4). The
presence of this communication raises the possibility
that the rheumatoid granuloma had been formed from
Table I .
infiltrating synovial tissue. However, the absence of
synovial tissue within the cyst, and the evidence of the
central zone of necrosis opening toward the joint space
(Figure 5 ) lead us to consider the hypothesis that these
Characteristics of patients with rheumatoid hodulosis*
Reference
(year)
Disease
Age1 duration,
sex
years
4 (1949)
511M
II
Acute migratory attacks
12 (1967)
32lM
21
3 (1975)
39lM
10
5 (I 977)
46/M
5
13 (1979)
36lM
14
10 (1981)
33lM
25
Acute migratory polyarthritis Periarticular thickening of
MCPs, ulnar deviation
No synovial thickening
Episodic tenderness and
swelling lasting 24-48
hours
No evidence of active syIntermittent acute arthritis
novitis
lasting 24-48 hours
Episodic migratory polyarth- Minimal synovial thickening
ritis
Occasional arthralgia, morn- No synovitis
ing stiffness
14 (1981)
60lF
10
1 I (1984)
35lF
25
Present case
35lM
11
* RF = rheumatoid factor; ESR
Joint symptoms
Arthralgia lasting 24-48
hours
Mild intermittent migratory
aches
Intermittent acute arthritis
lasting 4-5 days
=
Joint examination findings
Pain and swelling during
the attacks
No synovitis, ulnar deviation
No evidence of synovitis
RF
titer
ESR,
mml
hour
ND
62
Radiologic findings
Subchondral cystic lesions,
complete loss of joint
space
Small cystic lesions
1:80
24
15,120
25
Intraosseous cyst-like lesions, mild erosion
1:2,560
10
15,120
44
1:1,280
8
Large cyst-like erosion,
mild periarticular erosions
Cystic lesions, small joint
erosions
Multiple subcortical cysts,
absence of rheumatoid
changes
Subchondral geodes, mild
joint space narrowing
Multiple small cystic lesions
1:64
1:8,000
No synovitis or deformity 1:640
18
ND
21
Subchondral cystic lesions,
minimal joint space narrowing
erythrocyte sedimentation rate; ND = not done; MCPs = metacarpophalangealjoints.
BRIEF REPORTS
granulomas are primarily formed in the subchondral
bone.
In vitro studies have shown the capacity of
rheumatoid nodules to produce proteases and collagenases (17), which suggests that these enzymes could
be responsible for the breakdown of the extracellular
collagen matrix. According to these findings, the formation of bone cysts in rheumatoid nodulosis could be
accounted for by the breakdown of bone tissue that is
caused by proteolytic enzymes released by rheumatoid granuloma. Furthermore, as these granulomas
grow, a communication could be made with the joint
space. The identification of synovial tissue within the
subchondral cysts, reported by others (4),might be
explained by a secondary colonization of the cysts by
adjacent synovial tissue.
The histopathologic alterations of the synovial
membrane in our patient and in previously described
patients with rheumatoid nodulosis (43) are similar to
those of RA. This fact, as well as the presence of
subcutaneous rheumatoid nodules, positive serum RF,
and the improvement achieved in some patients after
therapy with gold ( 5 ) or D-penicillamine (3), have led
to the hypothesis that rheumatoid nodulosis constitutes a relatively benign variant of RA (3,5,9,10).
However, the relationship between the 2 processes
has not been clearly established.
Further studies are needed to elucidate why
patients with rheumatoid nodulosis do not develop
persistent or erosive joint disease despite the presence
of serologic and histologic markers of severity.
REFERENCES
1. Williams RC Jr, McCarty DJ: Clinical picture of rheumatoid arthritis, Arthritis and Allied Conditions. Tenth
edition. Edited by DJ McCarty. Philadelphia, Lea &
Febiger, 1985, pp 605-619
2. Kaye BR, Kaye RL, Bobrove A: Rheumatoid nodules:
review of the spectrum of associated conditions and
1283
proposal of a new classification, with a report of four
seronegative cases. Am J Med 76:279-292, 1984
3. Ginsberg MH, Genant HK, Yu TF, McCarty DJ: Rheumatoid nodulosis: an unusual variant of rheumatoid
disease. Arthritis Rheum 18:49-58, 1975
4. Bywaters EGL: A variant of rheumatoid arthritis characterized by recurrent digital pad nodules and palmar
fasciitis closely resembling palindromic rheumatism.
Ann Rheum Dis 8:2-30, 1949
5. Brower AC, NaPombejara C, Stechschulte DJ, Mantz
F, Ketchum L: Rheumatoid nodulosis: another cause of
juxta-articular nodules. Radiology 125:669-670, 1977
6. Burke GW, Carrington CB, Grinnan R: Pulmonary
nodules and rheumatoid factor in the absence of arthritis. Chest 72538-540, 1977
7. Ganda OP, Caplan HI: Rheumatoid disease without joint
involvement. JAMA 228:338-339, 1974
8. Askari A, Moskowitz RW, Goldberg VM: Subcutaneous
rheumatoid nodules and serum rheumatoid factor without arthritis. JAMA 229:319-320, 1974
9. Belin DC, Abeles M, Weinstein A: Rheumatoid markers
in the absence of arthritis. J Rheumatol6:293-299, 1979
10. Wisnieski JJ, Askari AD: Rheumatoid nodulosis: a relatively benign rheumatoid variant. Arch Intern Med
141:615-619, 1981
11. Fleischer A, McGrath MH: Rheumatoid nodulosis of the
hand. J Hand Surg [Am] 9:404411, 1984
12. Watt TL, Baumann RR: Pseudoxanthomatous rheumatoid nodules. Arch Dermatol 95:156-160, 1967
13. Snow C, Goldman JA, Casey HL, Karcioglu AZ, Heath
P, Spira TJ: Rheumatoid nodulosis: a continuum of
extra-articular rheumatoid disease. South Med J
72~1572-1577, 1979
14. Dreyfus P, Daupleix D: La nodulite rhumatoide. Rev
Rhum Ma1 Osteoartic 48:441446, 1981
15. Rennell C, Mainzer F, Multz CV, Genant HK: Subchondral pseudocysts in rheumatoid arthritis. AJR
129:1069-1072, 1977
16. Resnick D, Niwayama G, Coutts RD: Subchondral cysts
(geodes) in arthritic disorders: pathologic and radiographic appearance of the hip joint. AJR 128:799-806,
1977
17. Harris ED Jr: A collagenolytic system produced by
primary cultures of rheumatoid nodule tissue. J Clin
Invest 51:2973-2976, 1972
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