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Kitakanto Med J 2003 : 53 : 289•`291 289 Rheumatoid Arthritis Preceded by Interstitial Pneumonia Associated with Myelodysplastic Syndrome : A Case Report Yoshinori Mita,1 Kunio Dobashi,1 Yasuo Shimizu,1 Tsugio Nakazawa,2 and Masatomo Mori1 A 75-year-old male was admitted because of dyspnea. A chest X-ray showed interstitial shadows in both lung fields, and hematology studies revealed a normochronic anemia. Examination of bone marrow smears led to a diagnosis of myelodysplastic syndrome. The rheumatoid factor test was positive, but the patient did not have arthritis. Twenty-one months later, he began to experience joint pain and swelling, and he fulfilled the diagnostic criteria for rheumatoid arthritis. This is the first case ever reported of rheumatoid arthritis preceded by interstitial pneumonia associated with myelodysplastic syndrome. (Kitakanto Med J 2003 ; 53 : 289•`291) Key words rheumatoid arthritis, myelodysplastic syndrome, interstitial pneumonia Introduction Diagnostic criteria for the various types of myelodysplastic syndrome (MDS) have been proposed, and a detailed description of features that may help define MDS has been published.1 MDS refers to disorders in which the abnormal findings are thought to be confined to the myeloid cell series.2 Raskind et al. hypothesized that at least two events are involved in the pathogenesis of the myelodysplasia : one causing proliferation of a clone of genetically unstable plur- ipotent stem cells, and the other inducing chromosomal abnormalities in its descendants.3 Vari- ous rheumatic manifestations have been described in patients with MDS.4•`6 However, there have been no reports rheumatoid arthritis preceded by interstitial pneumonia associated with MDS. We report the first case of rheumatoid arthritis preceded by interstitial pneumonia associated with MDS. Case Report A 75-year-old male was admitted to a hospital because of dyspnea. He had no history of morning stiffness, pain, or swelling of the joints. On physical examination, blood pressure was 128/82mmHg, pulse rate 83/min, and body temperature 36.1•Ž, and his conjunctivae were pale. Chest examination revealed Velcro rales in the inferior lung fields bilaterally. Laboratory studies showed a hemoglobin level of 9.2g/ dL, hematocrit 28.8%, WBC count 4900/ƒÊL, platelet count 109,000/ƒÊL, LDH 240IU/L, RAPA 2560X. CRP, the erythrocyte sedimentation rate (ESR), the ANA test, and direct and indirect Coombs' tests were normal. A bone marrow biopsy revealed normocel- lularity with erythroid hyperplasia and dysplasia, a myeloid to erythroid (M : E) ratio 4 : 1, and pseudo- Pelger anomalies of neutrophils and micro- megakaryocytes. A diagnosis of myelodysplastic syn- drome (MDS) was made based on these findings. A chest x-ray showed basal-predominant reticular abnor- mality in both lung fields (Fig. 1). Computed tomogra- phy (CT) of the chest showed reticular honeycombing in the peripheral subpleural base of both lung fields (Fig. 2). Pulmonary function testing yielded a % DLCO of 38%. Despite the positive rheumatoid fac- tor test, the patient did not have arthritis. Twenty-one months later, he began to experience pain and swelling of the small joints of the hands and feet, and of the wrists, elbows, knees, and ankles. The patient then met the diagnostic criteria for rheumatoid arthritis, and 1 Department of Medicine and Molecular Science (First Department of Internal Medicine), Gunma University Graduate School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma 371-8511, Japan 2 Gunma University School of Health Sciences, 3-39-15, Showa-machi, Maebashi, Gunma 371-8511, Japan Received : May 6, 2003 Address : YOSHINORI MITA Department of Medicine and Molecular Science (First Department of Internal Medicine), Gunma University Graduate School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma 371-8511, Japan

Rheumatoid Arthritis Preceded by Interstitial Pneumonia

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Kitakanto Med J

2003 : 53 : 289•`291

289

Rheumatoid Arthritis Preceded by Interstitial Pneumonia

Associated with Myelodysplastic Syndrome : A Case Report

Yoshinori Mita,1 Kunio Dobashi,1 Yasuo Shimizu,1

Tsugio Nakazawa,2 and Masatomo Mori1

A 75-year-old male was admitted because of dyspnea. A chest X-ray showed interstitial shadows in

both lung fields, and hematology studies revealed a normochronic anemia. Examination of bone

marrow smears led to a diagnosis of myelodysplastic syndrome. The rheumatoid factor test was positive,

but the patient did not have arthritis. Twenty-one months later, he began to experience joint pain and

swelling, and he fulfilled the diagnostic criteria for rheumatoid arthritis. This is the first case ever

reported of rheumatoid arthritis preceded by interstitial pneumonia associated with myelodysplastic

syndrome. (Kitakanto Med J 2003 ; 53 : 289•`291)

Key words rheumatoid arthritis, myelodysplastic syndrome, interstitial pneumonia

Introduction

Diagnostic criteria for the various types of

myelodysplastic syndrome (MDS) have been proposed,

and a detailed description of features that may help

define MDS has been published.1 MDS refers to

disorders in which the abnormal findings are thought

to be confined to the myeloid cell series.2 Raskind et

al. hypothesized that at least two events are involved in

the pathogenesis of the myelodysplasia : one causing

proliferation of a clone of genetically unstable plur-

ipotent stem cells, and the other inducing

chromosomal abnormalities in its descendants.3 Vari-

ous rheumatic manifestations have been described in

patients with MDS.4•`6 However, there have been no

reports rheumatoid arthritis preceded by interstitial

pneumonia associated with MDS. We report the first

case of rheumatoid arthritis preceded by interstitial

pneumonia associated with MDS.

Case Report

A 75-year-old male was admitted to a hospital

because of dyspnea. He had no history of morning

stiffness, pain, or swelling of the joints. On physical

examination, blood pressure was 128/82mmHg, pulse

rate 83/min, and body temperature 36.1•Ž, and his

conjunctivae were pale. Chest examination revealed

Velcro rales in the inferior lung fields bilaterally.

Laboratory studies showed a hemoglobin level of 9.2g/

dL, hematocrit 28.8%, WBC count 4900/ƒÊL, platelet

count 109,000/ƒÊL, LDH 240IU/L, RAPA 2560X.

CRP, the erythrocyte sedimentation rate (ESR), the

ANA test, and direct and indirect Coombs' tests were

normal. A bone marrow biopsy revealed normocel-

lularity with erythroid hyperplasia and dysplasia, a

myeloid to erythroid (M : E) ratio 4 : 1, and pseudo-

Pelger anomalies of neutrophils and micro-

megakaryocytes. A diagnosis of myelodysplastic syn-

drome (MDS) was made based on these findings. A

chest x-ray showed basal-predominant reticular abnor-

mality in both lung fields (Fig. 1). Computed tomogra-

phy (CT) of the chest showed reticular honeycombing

in the peripheral subpleural base of both lung fields

(Fig. 2). Pulmonary function testing yielded a %

DLCO of 38%. Despite the positive rheumatoid fac-

tor test, the patient did not have arthritis. Twenty-one

months later, he began to experience pain and swelling

of the small joints of the hands and feet, and of the

wrists, elbows, knees, and ankles. The patient then

met the diagnostic criteria for rheumatoid arthritis, and

1 Department of Medicine and Molecular Science (First Department of Internal Medicine), Gunma University Graduate School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma 371-8511, Japan

2 Gunma University School of Health Sciences, 3-39-15, Showa-machi, Maebashi, Gunma 371-8511, Japan

Received : May 6, 2003 Address : YOSHINORI MITA Department of Medicine and Molecular Science (First Department of Internal Medicine), Gunma

University Graduate School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma 371-8511, Japan

290 RA with IP and MDS

a diagnosis of rheumatoid arthritis preceded by inter-

stitial pneumonia associated with MDS was made.

Discussion

Homma et al. prospectively followed 68 patients

diagnosed with idiopathic interstitial pneumonia (IIP)

for periods of 1 to 11 years, and 13 of them (19%)

subsequently developed systemic manifestations of

collagen vascular diseases CVD) and were diagnosed

as having had interstitial pneumonia as the sole

presenting manifestation of collagen vascular disease

(CVD-IP).7 Comparism with the CVD-IP patients revealed that the IIP patients were more likely to be

male, to be of advanced age, to have a past history of

hypertension, and to have a cough, exertional dyspnea,

and digital clubbing.7'8 The CVD-IP patients had a

higher ESR and a higher incidence of x-ray evidence of

discoid atelectasis in the lower lung fields.7 However,

since our patient was an older male with no past

history of hypertension, no cough, no digital clubbing,

had a normal ESR, and had no discoid atelectasis in

the lower lung fields, the features of our patient were

different from those of CVD-IP.7 These difference

may be attributable to our patient having MDS, which

are contributed to the long-term immunodysfunction

found in CVD.9

There is a report that the rate of complication of

rheumatoid arthritis by interstitial pneumonia is

1.1%,10 and there is also a report that the rate of

complication of rheumatoid arthritis associated with

MDS by interstitial pneumonia is 11.8% (2 of the 17

patients) in patients.6,11,12 These reports suggest that MDS may contribute to complication by interstitial

pneumonia. This is the first report of a first case of rheumatoid

arthritis preceded by interstitial pneumonia associated

with MDS.

References

1. Bennett JM, Catovsky D, Daniel MT, et al:

Proposals for the classification of the myelodys-

plastic syndromes. Br J Haematol 1982 ; 51 : 189-199.

2. Bynoe AG, Scott CS, Ford P, et al : Decreased T

helper cells in the myelodysplastic syndromes. Br

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3. Raskind WH, Tirumali N, Jacobson R, et al :

Evidence for a multistep pathogenesis of a

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Fig. 1 Chest x-ray showing basal-predominant reticular abnor-

mality in both lung fields.

Fig. 2 Chest computed tomogram showed reticular, honeycomb-ing in the peripheral subpleural base of both lung fields

(A and B).

291

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