Caso 2

The n e w e ng l a n d j o u r na l of m e dic i n e
case records of the massachusetts general hospital
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Eric S. Rosenberg, m.d., Editor Nancy Lee Harris, m.d., Editor
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Case 26-2013: A 46-Year-Old Woman

with Muscle Pain and Swelling
Margaret Seton, M.D., Carol C. Wu, M.D., and Abner Louissaint, Jr., M.D., Ph.D.
Pr e sen tat ion of C a se
From the Department of Medicine, Dr. Luke A. Neilans (Medicine): A 46-year-old woman was seen in the emergency de-
Brigham and Women’s Hospital (M.S.), partment at this hospital because of muscle pain and swelling in her arms and
the Departments of Radiology (C.C.W.)
and Pathology (A.L.), Massachusetts lower legs.
General Hospital, and the Departments The patient had been well until approximately 3 weeks before admission, when
of Medicine (M.S.), Radiology (C.C.W.), a deep ache developed in her left triceps, in the absence of trauma. During the fol-
and Pathology (A.L.), Harvard Medical
School — all in Boston. lowing weeks, the pain persisted, gradually spreading to both arms and both legs,
and was exacerbated by movement; she began having difficulty rising from chairs
N Engl J Med 2013;369:764-73.
DOI: 10.1056/NEJMcpc1208152 and climbing stairs because of pain. Two weeks before admission, examination by
Copyright © 2013 Massachusetts Medical Society. her primary care physician at another hospital reportedly revealed no focal muscle
weakness. Red-cell indexes and blood levels of electrolytes, calcium, and glucose
Paciente viene con dolor muscular e
hinchazón en sus brazos y piernas inferiores.were normal, as were renal-function tests; testing for parvovirus B19 IgG and IgM
antibodies was negative; other test results are shown in Table 1. Three days before
Tríceps izquierdo le empezó hace tres admission, pain and swelling in the left arm worsened. She returned to the other
semanas, sin trauma aparente, las hospital. Blood levels of alkaline phosphatase, direct and total bilirubin, total
siguientes semanas se expandió a ambos protein, albumin, and thyrotropin were normal, and testing for antibodies to the
brazos y ambas piernas.
human immunodeficiency virus and Borrelia burgdorferi were negative; other test
Dificultad para levantarse de las sillas y subir results are shown in Table 1. The patient returned home, with persistent symptoms.
escaleras Three days later, she came to the emergency department at this hospital.
The patient reported restricted range of motion of her arms because of pain, as
well as stiffness in her proximal arm muscles that was worse in the morning. She
also reported intermittent nondrenching night sweats that she attributed to meno-
pause. She reported no muscle weakness, fever, chills, malaise, dysphagia, nasal
regurgitation, synovitis, weight loss, numbness or tingling in her hands, shortness
of breath, chest pain, changes in bowel or bladder function, nausea, vomiting,
photosensitivity, or rash. A diagnosis of uterine fibroids with menorrhagia had
been made 2 years earlier. Routine mammograms had been normal. Her only
medication was megestrol acetate, taken midcycle (10 days per month). She had
no known allergies. She was single, physically active, and had traveled widely. She
drank alcohol in moderation, and she did not smoke or use illicit drugs. Her
mother had had breast cancer at age 65 years, and her father had had a myocar-
dial infarction at age 57 years. There was no family history of rheumatologic or
neuromuscular disorders.
764 n engl j med 369;8 nejm.org august 22, 2013
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Enzimas muscular séricas:
Creatinin kinasa, Lactato case records of the massachusetts gener al hospital
desidrogenasa, Altolasa
Table 1. Laboratory Data.
Reference 11 Days before 3 Days before

Range, Admission, Admission,
Variable Adults* Other Hospital Other Hospital On Admission
Hematocrit (%) 36.0–46.0 39.4 33.3
Hemoglobin (g/dl) 12.0–16.0 12.8 11.3
White-cell count (per mm3) 4500–11,000 6970 7700
Differential count (%)
Neutrophils 40–70 68 65
Lymphocytes 22–44 18 22
Monocytes 4–11 5 4
Eosinophils 0–8 4 9
Platelet count (per mm3) 150,000–400,000 373,000 511,000
Erythrocyte sedimentation rate (mm/hr) 0–17 34 38 58
Prothrombin time (sec) 11.0–13.7 13.8
International normalized ratio for prothrombin time 1.1
Aspartate aminotransferase (U/liter) 9–32 120 132
Alanine aminotransferase (U/liter) 7–30 97 101
Lactate dehydrogenase (U/liter) 110–210 421
Creatine kinase (U/liter) 40–150 1063 2150 2784
C-reactive protein (mg/liter) <8.0 11.5 70.4
Antinuclear antibody Negative at 1:40 and Positive at 1:40 dilution
1:160 dilutions (speckled pattern);
negative at 1:80 and
1:160 dilutions
* Reference values are affected by many variables, including the patient population and the laboratory methods used. The ranges used at Massa
chusetts General Hospital are for adults who are not pregnant and do not have medical conditions that could affect the results. They may
therefore not be appropriate for all patients.
On examination, the blood pressure was cose, calcium, phosphorus, magnesium, total pro-
113/82 mm Hg, the pulse 101 beats per minute, tein, albumin, globulin, alkaline phosphatase, di- PAN : 120/80
the temperature 36.7°C, the respiratory rate 18 rect and total bilirubin, and thyrotropin. Results of PULSO N: 60-100 lpm
breaths per minute, and the oxygen saturation renal-function tests were normal; other test results Resp N: 12-18 rpm
100% while the patient was breathing ambient are shown in Table 1. Urinalysis revealed positive
air. When she was standing, her pulse increased nitrites, 3+ ketones, and trace occult blood and
to 120 beats per minute, without symptoms of albumin; few bacteria, few squamous cells, and
dyspnea or light-headedness. Her voice was slightly very few transitional cells were seen per high-
gravelly, which she reported was unchanged. There power field. Screening of the urine for human Se descarta hCG y
was firm, nonpitting swelling and tenderness to chorionic gonadotropin (hCG) and toxins was toxinas
palpation on the right shoulder and from the left negative. Ultrasonography of the left arm and
shoulder to the hand. Both calves were tender to right leg showed no deep venous thrombosis.
palpation, without visible swelling. Gait and mus- Dr. Carol C. Wu: A chest radiograph showed a
cle strength were normal, and she could easily soft-tissue opacity, 9.0 cm by 9.0 cm by 12.4 cm,
squat, stand, and rise up on either foot on tiptoe. in the right lower hemithorax that obscured the
There was no rash, evidence of nail-bed infarcts, right heart border‎, with no evidence of pneumo-
or impairment of respiratory excursions; the re- nia or pulmonary edema (Fig. 1A). The opacity was
mainder of the examination was normal. seen anteriorly on the lateral view. The appearance
The activated partial-thromboplastin time was was suggestive of an anterior mediastinal mass.
normal, as were blood levels of electrolytes, glu- Computed tomography (CT) after the administra-
n engl j med 369;8 nejm.org august 22, 2013 765

A Figure 1. Chest Images.

A posteroanterior chest radiograph (Panel A) shows a
soft-tissue opacity with a smooth margin (arrows) in
the right lower thorax, silhouetting the right heart bor-
der. An axial CT image (Panel B) shows a large right
anterior mediastinal mass abutting the right pericardi-
um (arrows) with a small pericardial effusion (arrow-
head). The mass causes external compression of the
right atrium (RA). A coronal reformatted image (Panel
C) from a contrast-enhanced CT scan shows the cra-
niocaudal extent of the mass. The superior vena cava
(arrow) is patent.
dence of internal calcification or fat. The mass

abutted the right pericardium, with no distinct
fat plane between the mass and the pericardium.
There was no evidence of invasion into the peri-
cardium. There was a small pericardial effusion.
B The superior vena cava was patent, but there was
compression of the right atrium. There was no
evidence of intrathoracic lymphadenopathy. There
Mass was no connection between the mass and the
RA
thyroid gland. The differential diagnosis included
a thymic neoplasm, lymphoma, and a germ-cell
tumor. In view of the absence of connection to
the thyroid gland, a thyroid origin was unlikely.
Lymphoma was considered to be unlikely in the
absence of any other evidence of lymphadenopathy.
Dr. Neilans: An electrocardiogram showed si-
nus rhythm at a rate of 111 beats per minute, left
atrial enlargement, and minor, nonspecific ST-
C
segment and T-wave abnormalities. The patient
was admitted to the hospital. Blood levels of para-
thyroid hormone, hCG, and alpha-fetoprotein were
normal, and testing for antibodies to Ro, La,
Sm, RNP, and Jo-1 was negative.
On the third day, a diagnostic procedure was
performed.
Differ en t i a l Di agnosis
Mass
Dr. Margaret Seton: I am aware of the diagnosis in
this case. This 46-year-old woman presented with
asymmetric swelling and pain in the arms, calf
pain, an elevated creatine kinase level, and a large
mediastinal mass. The patient was usually healthy,
worked as a journalist, and had traveled widely. She
described no cough, dyspnea, fever, or chest pain.
The rheumatology service was asked to see the
tion of intravenous contrast material (Fig. 1B and patient to address the question of myositis in
1C) confirmed the presence of a large, anterior this context. We were initially concerned about
mediastinal soft-tissue mass with a smooth mar- vascular compression, infection, or trauma, since
gin and heterogeneous density but with no evi- the predominant symptom was pain and the pre-

case records of the massachusetts gener al hospital
dominant finding was taut and swollen arms. the autoimmunity expressed in many patients with
Although the cardiac ultrasound examination thymomas.3
confirmed that the mass was compressing the
right atrium and causing turbulence in the right Myasthenia Gravis
pulmonary venous return, there was no frank The striking feature in most thymomas is the pref-
obstruction, no thrombosis, and no muscle in- erential targeting of autoantibodies to neuromus-
farction, and the neurovascular findings of com- cular end plates and other muscle antigens. The
partment syndrome were absent. most common paraneoplastic process in thymo-
ma is myasthenia gravis, an autoimmune disease
Polymyositis of the neuromuscular junction4 that occurs in
Tropical pyomyositis can be manifested as a fo- about 25% of patients with thymoma.3 In pa-
cal lesion in muscle, usually in association with tients with thymoma-associated myasthenia gra-
fever and antecedent trauma, or alternatively, in vis, acetylcholine receptor binding antibodies are
an immunocompromised or malnourished host. characteristically present; the antibody titers mark
These findings were not present in this case. the disease rather than correlate with clinical se-
Asymmetric muscle pain and swelling are not verity. Striated-muscle antibodies targeting pro-
typical manifestations of the inflammatory myop- teins found in both skeletal and cardiac muscle
athies. Rather, these myopathies are characterized are also present in some patients with thymoma
by symmetric and proximal-muscle weakness, and myositis.5,6 Polymyositis, giant-cell myocar-
difficulty swallowing, and sometimes dyspnea ditis, pure red-cell aplasia, hypogammaglobu-
(when the respiratory muscles become involved). linemia, and a spectrum of movement disorders
Idiopathic inflammatory myopathies may be para- spawned by antibodies that target muscle com-
neoplastic syndromes, may be associated with ponents are also described in cases of thymo-
distal neuromuscular weakness (e.g., inclusion- ma.5-12 Despite repeated questioning, this patient
body myositis), or may be accompanied by a pho- did not report symptoms attributable to myasthe-
tosensitive rash (e.g., dermatomyositis). Myalgias nia gravis. Instead, she reported symptoms of a
can occur but are usually mild, except in viral myositis.
myopathies.
Myositis
Thymoma The idiopathic inflammatory myopathies (derma-
The mediastinal mass in this case is highly sug- tomyositis, polymyositis, and inclusion-body my-
gestive of thymoma. Patients with thymoma may ositis) are rare.13 Polymyositis and giant-cell
be asymptomatic or may present with paraneo- myocarditis are the most commonly reported in-
plastic autoimmune disease or with chest pain flammatory myopathies associated with myas-
and vascular compression symptoms.1 In this case, thenia gravis or thymoma.7 Dermatomyositis is
vascular compromise did not account for the re- recognized in case reports of patients with thy-
gional muscle swelling and pain. Therefore, we moma or myasthenia gravis,14-18 but inclusion-
favored a diagnosis of a paraneoplastic manifes- body myositis has not been described in such re-
tation of thymoma, most likely a myositis that was ports. Patients with such myopathies classically
atypical in distribution. present with proximal-muscle weakness, dyspha-
Central immune tolerance is orchestrated in gia, and anterior neck muscle weakness; there is
the thymus through the selective deletion of ef- a well-known association with a malignant tumor,
fector T cells targeted to self-antigens. Precursors which is manifested either concurrently with the
of lymphocytes and dendritic cells home to the inflammatory myopathy or in the early years af-
thymus, where cortical epithelial cells promote ter its diagnosis and treatment.19-22 New antibod-
maturation and lineage commitment. Through ies defining idiopathic inflammatory myopathies
the expression of tissue self-antigens, enhanced are promising to change the diagnostic criteria
by the autoimmune regulator gene (AIRE), med- and redefine the clinical spectrum of these dis-
ullary thymic epithelial cells educate immune cells eases.23,24
to recognize self and to delete autoreactive T cells This patient presented with features consis-
before they leave the thymus.2 Failure to induce tent with an inflammatory myopathy. Our initial
this tolerance to self-antigens may account for concern was that she had a myopathy heralding

Miopatía
The n e w e ng l a n d j o u r na l of m e dic i n e inflamatoria
necrosante
y
a malignant tumor; however, her presentation was DR . M A RG A R E T SE T ON’S DI AGNOSIS miastenia
atypical for this because of the focal and asym- gravis
metric muscle swelling and pain that signaled Inflammatory, necrotizing myopathy and myas- asociada
muscle injury in her arms and calves. thenia gravis associated with thymoma. con
Because of a strong suspicion of a neuromus- timoma.
cular disorder occurring in the context of thymo- Pathol o gic a l Discussion
ma, we recommended biopsy of the anterior
mediastinal mass and deltoid muscle. The pa- Dr. Abner Louissaint, Jr.: The fine-needle aspiration
tient was discharged on the fifth hospital day, biopsy specimen of the mediastinal mass revealed
with the results of the biopsies pending. During bland, spindle-shaped cells with admixed, small
the week after discharge, worsening symmetric lymphocytes (Fig. 2A). Flow-cytometric analysis
muscle pain, swelling, and loss of function de- revealed a population of CD45+CD4+CD8+
veloped, with contractures in her elbow, wrist, and CD1+TdT+ (terminal deoxynucleotidyl transfer-
ankles. The patient is here with us today. Would ase) cells lacking surface CD3, a finding consis-
you describe the new symptoms that developed tent with immature thymic T cells, and a comple-
in the days between hospital admissions? ment of CD3+CD1−TdT− mature T cells with
The Patient: Nine days after discharge, I sud- either CD4 or CD8 expression. A concurrent core-
denly noticed that my neck was weak. By the biopsy specimen revealed small fragments of tis-
time I woke up the next day, I had what I would sue containing small, mature-appearing lympho-
describe as a bobble head. Everything that I had cytes, admixed with oval or polygonal epithelial
been asked about was manifesting itself: diffi- cells (Fig. 2B). Immunohistochemical staining of
culty swallowing, double vision, garbled speech, the specimen revealed a predominance of
and difficulty holding up my head. Pyridostigmine CD1a+TdT+ immature T cells, and staining for
was prescribed by Dr. William S. David, my neu- Ki-67 showed a high proliferation fraction, fea-
rologist, that day. The next day, I was in his of- tures consistent with cortical thymocytes (Fig.
fice, and I would describe my posture as “Tyran- 2D). The epithelial cells were highlighted by a
nosaurus rex.” My hands and arms were less cytokeratin stain (Fig. 2C). There were small nod-
flexible and looked almost foreshortened, my ular areas with fewer CD1a+TdT+ cells, a finding
shoulders and neck were less flexible, and I walked suggestive of focal medullary differentiation.
on my tiptoes and had to work to keep my heels The morphologic and immunophenotypic find-
Timonas clasificaon:on the ground. ings are diagnostic of thymoma with a cortical
Dr. Seton: What symptoms responded to pyr- component. In the World Health Organization
Tipo A: Ovaladas o idostigmine? (WHO) classification,25 thymomas are primarily
fusiformes / Típicas
The Patient: The last symptoms to develop were classified according to the morphologic features
normales
those that responded to pyridostigmine. Within of the epithelial cells — that is, predominantly
Tipo B: Redondos o
24 hours after starting on the drug, I stopped oval or spindle-shaped, resembling normal thy-
poligonales/
getting worse. My head was staying up by itself, mic medullary epithelial cells (type A), or round
Timicas corticales
or polygonal, resembling normal cortical thymic
nomales. Según la but I still had some difficulty swallowing and
extensión del some double vision. Those symptoms were all but epithelial cells (type B). Type B thymomas are
componente gone within 4 days. further classified according to the extent of the
linfocítico y grado Dr. Seton: The new symptoms in this patient lymphocytic component and the degree of cyto-
de atipia citológica are consistent with myasthenia gravis, follow- logic atypia of the epithelial cells (types B1, B2,
de cels epiteliales. ing the onset of asymmetric myositis over the and B3). All types of thymomas may be associ-
course of weeks. A limited electromyographic ated with paraneoplastic phenomena, as seen in
study performed by Dr. David the day after the this case, but these phenomena are more com-
administration of pyridostigmine was begun mon in the type B thymomas. The epithelial
showed features consistent with a severe myo- cells of most thymomas lack expression of AIRE,
pathic disorder. Repetitive nerve stimulation a feature that is hypothesized to lead to the de-
revealed no significant decremental response velopment of autoreactive T cells and the genera-
to suggest a postsynaptic neuromuscular-junction of autoantibodies in some patients with
tion disorder, but the patient was taking pyr- thymoma.26,27 The combination of morphologic
idostigmine at the time. immunophenotypic features in this case is most

A B
C D
Figure 2. Fine-Needle Aspiration and Core-Biopsy Specimens of the Thymic Mass.

The fine-needle aspirate of the mass (Panel A, Papanicolaou stain) shows spindle-shaped cells admixed with lym-
phocytes. The core-biopsy specimen (Panel B, hematoxylin and eosin) contains numerous small or medium-size
lymphocytes and scattered larger oval or polygonal cells, features consistent with thymic epithelial cells. The epi-
thelial cells are highlighted by a cytokeratin stain (Panel C) and are present in a background of CD1a+ thymocytes
(Panel D).
suggestive of type B1 thymoma; however, defini- dividual fibers, which suggested involvement by
tive classification is best based on the resected an immune-mediated activation of the complement
tumor. system (Fig. 3D). The morphologic and immuno-
A biopsy specimen of the left triceps muscle phenotypic features are consistent with a severe
was obtained. The muscle was described as “un- destructive inflammatory myopathy and exten-
usually firm” at the time of biopsy. On micro- sive monophasic muscle injury resulting in wide-
scopical examination, the muscle was markedly spread degeneration and regeneration of muscle
cellular; extensive fibrosis between muscle fas- fibers.
cicles and the surrounding individual myofibers Dr. Seton: After the needle-biopsy results indi-
was highlighted by a trichrome stain (Fig. 3B). cated a diagnosis of thymoma, pulse therapy with
In contrast to normal muscle fibers, these fibers methylprednisolone sodium succinate was admin-
were smaller, more basophilic, and rounded, and istered for 3 consecutive days, followed by oral
many had multiple nuclei that were centrally lo- prednisone. I would like to ask the patient how
cated within the fiber, features consistent with she felt after the first infusion of intravenous
severe muscle injury and regeneration (Fig. 3A). glucocorticoids.
A lymphohistiocytic infiltrate was present between The Patient: I felt instantly better. Not just “not
muscle fibers. Immunohistochemical stains re- worse,” but better. I felt as though I could have
vealed focal large aggregates of CD68+ macro- run up 52 flights of stairs at the Prudential Center.
phages and CD3+ T cells surrounding individual Dr. Seton: Two weeks after the biopsy, the patient
muscle fibers (Fig. 3C). Deposition of a stain for was readmitted for resection of the thymoma.
membrane-attack complex appeared around in- Dr. Louissaint: The resected anterior mediasti-

A B
C D
Figure 3. Muscle-Biopsy Specimen.

The muscle-biopsy specimen is markedly cellular (Panel A, hematoxylin and eosin). In contrast to normal muscle,
the muscle fibers are small, basophilic, and rounded, and many have multiple nuclei that are centrally located within
the fiber, features consistent with severe muscle injury and regeneration. Trichrome stain highlights extensive fibro-
sis between muscle fascicles (Panel B). An immunohistochemical stain for CD68 shows increased CD68+ macro-
phages around muscle fibers (Panel C). Deposition of a stain for membrane-attack complex appears around individ-
ual fibers (Panel D).
nal mass consisted of an enlarged thymus with resection specimen could represent type A (med-
a well-encapsulated, firm, ovoid, white-pink mass, ullary) thymoma, or possibly type AB (mixed) or
10.2 cm in diameter, on its inferior aspect (Fig. type B1 (lymphocytic) thymoma with lympho-
4A). The mass had a tan, nodular cut surface with cyte depletion due to therapy; a definite subclas-
focal yellow areas, corresponding to necrosis sification of the thymoma could not be deter-
(Fig. 4B). Microscopical examination revealed a mined. There was focal microscopical capsular
predominance of oval or spindle-shaped, keratin- invasion, and all margins and lymph nodes were
positive epithelial cells with a marked decrease negative for involvement.
in the lymphocytic component (Fig. 4C and 4D) Dr. Seton: This patient presented initially with Mitosis:
as compared with the initial biopsy specimen. an asymmetric, necrotizing myositis that was muerte
Flow cytometry did not reveal immature T cells. rapidly followed by the evolution of symptoms of muscular
Immunohistochemical stains for CD1a and TdT myasthenia gravis, which became life-threaten- dolor y
showed only occasional cortical thymocytes ing. The myositis led to muscle death, pain, and contractura
(Fig. 4E and 4F). contractures; the myasthenia gravis led to dete-
It is possible that the marked reduction in the rioration in speech, swallowing, and vision. Miastenia:
lymphoid component is related to the glucocor- Additional testing showed high titers of ace- Deterioro
ticoid therapy, which causes rapid loss of corti- tylcholine receptor antibodies, acetylcholine re-
del habla,
visión,
cal thymocytes. The pathological findings in the ceptor modulating antibodies, and antibodies to
deglución

A B
C D
E F
Figure 4. Resected Thymus.

The thymus contains a well-encapsulated, firm, ovoid mass, 10.2 cm in diameter (Panel A). A lobulated cut surface
(Panel B) is tan and pink, with focal yellow areas indicating necrosis (Panel B). The tumor consists of a dense pop-
ulation of oval or spindle-shaped epithelial cells, with bland cytologic features (Panel C, hematoxylin and eosin),
that are positive for cytokeratin (Panel D). On immunohistochemical staining, very few CD1a+ (Panel E) and TdT+
(Panel F) thymocytes are present.
striated muscle (1:983,040; reference range, <1:60). the stiff person syndrome), or antibodies ac-
The patient did not have antibodies to MuSK, counting for other disorders of neurologic trans-
voltage-gated potassium channel (seen in neuro- mission.
myotonia), glutamic acid decarboxylase (seen in This patient’s prognosis, on the basis of the

WHO classification of either type A or type B1 Dr. Seton: I do think that is an astonishing
and complete surgical resection, is excellent; part of this case. However, in many autoimmune
patients with thymoma and myasthenia gravis diseases, autoantibodies may be present in a
may have better outcomes than patients who have patient’s blood for years before the onset of symp-
thymoma without myasthenia gravis.28 Clinically, toms, and the event that results in end-organ
the patient has improved dramatically. She has damage is unclear.
regained function in terms of caliber of voice,
restoration of vision, swallowing, and muscle A NAT OMIC A L DI AGNOSIS
strength. The administration of pyridostigmine
and glucocorticoids was tapered and discontin- Inflammatory myopathy and myasthenia gravis
ued. Mild residual flexion contractures in her associated with thymoma.
hands and left foot remain, as does left phrenic-
This case was presented at the medical case conference.
nerve palsy as a consequence of the surgery. Al- No potential conflict of interest relevant to this article was re-
though second malignant tumors are described ported.
in the literature in patients with thymoma, this Disclosure forms provided by the authors are available with
the full text of this article at NEJM.org.
patient is currently well and will be followed We thank Drs. Donald Bloch, E. Tessa Hedley-Whyte, and
conservatively during the next several years.29 Robert P. Hasserjian for their comments and review of an earlier
Dr. Hasan Bazari (Medicine): It seems likely that version of the manuscript; and Drs. William David (Neurology),
Michael Lanuti (Surgery), Emily Hyle (Medicine), and Andrew
this tumor had been present for some time. How Liteplo (Emergency Medicine) for their assistance with prepa-
can we explain the abrupt onset and fulminant ration of the case history.
progression of the patient’s symptoms?
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and an inhibitor of antigen-induced lympho- GJ, Vos PE, Boerman RH, ter Laak HJ, van 24. Mimori T, Imura Y, Nakashima R, Yo-
cyte transformation. Br J Haematol 1975;29: Engelen BG. Concomitant dermatomyosi- shifuji H. Autoantibodies in idiopathic
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9. Masuda M, Arai Y, Okamura T, Wada respiratory insufficiency. Muscle Nerve clinical and pathophysiological signifi-
M, Mizoguchi H. Pure red cell aplasia 2002;25:293-6. cance. Curr Opin Rheumatol 2007;19:
(PRCA) with thymoma: a possible distinct 16. Ago T, Nakamura M, Iwata I, et al. 523-9.
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type 1 (APS-1). J Pathol 2007;211:563-71. G, et al. Thirty-five-year follow-up analy- Copyright © 2013 Massachusetts Medical Society.
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The n e w e ng l a n d j o u r na l of m e dic i n e

case records of the massachusetts general hospital

Founded by Richard C. Cabot

Case 26-2013: A 46-Year-Old Woman

Pr e sen tat ion of C a se

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Table 1. Laboratory Data.

Reference 11 Days before 3 Days before

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A Figure 1. Chest Images.

dence of internal calcification or fat. The mass

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Figure 2. Fine-Needle Aspiration and Core-Biopsy Specimens of the Thymic Mass.

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Figure 3. Muscle-Biopsy Specimen.

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Figure 4. Resected Thymus.

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