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Hodgkin's disease presenting as relapsing polychondritis a previously undescribed association.

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Hodgkin’s Disease Presentingas Relapsing Polychondritis
A Previously Undescribed Association
Stephen B. Miller, Charles J. Donlan and Steven B. Roth
Relapsing polychondritis is a rare syndrome thought to be autoimmune
in nature and characterized by inflammation and degeneration of cartilage. The association with a reticuloendothelial malignancy has not previously been reported. A 46-year-old man presented typical clinical manifestations of relapsing polychondritis requiring 30 mg of prednisone
daily for suppression. However within 6 months he developed chronic
fatigue, fevers, night sweats, adenopathy, and splenomegaly leading to
the diagnosis of Hodgkin’s disease.
Relapsing polychondritis is a rare syndrome characterized by inflammation and
degeneration of cartilage, specifically ear
and nasal cartilage, and often associated
with ocular and cardiac involvement (1-3).
Anemia, elevated sedimentation rate, and
hypergammaglobulinemia are prominent
laboratory features. T h e etiology of this
disease is unknown hut the prevalent speculation is that it is autoimmune in nature.
A number of concomitant disorders have
been found in patients with relapsing polychondritis. These include rheumatoid arFrom the Department of Medicine, Naval Hospital, Portsmouth, Virginia 23708.
Presented at the Virginia Regional American College of Physicians Meeting, Williamsburg, Virginia,
March 17, 1973.
The opinions or assertions contained herein are
those of the authors and are not to be construed
as official or reflecting the views of the Navy Department or of the Naval Service at large.
thritis, Sjogren’s syndrome, pregnancy, tuberculosis, myxedema, systemic lupus erythematous, rheumatic myocarditis, scleroderma, psoriasis, syphilis, diabetes insipidus,
diabetes mellitus, renal tubular acidosis,
herpes zoster, herpes simplex, and exophthalamus (1). An association with malignancy has been suggested with 2 cases in
in patients who were later diagnosed as
having carcinoma of the pancreas and prostate (4). T h e association with a reticuloendothelial malignancy has not been reported previously. We present a case of a
man who developed typical clinical manifestations of relapsing polychondritis s u p
pressed, as expected, by corticosteroid therapy. However his clinical course changed
with the development within 6 months of
peripheral adenopathy and splenomegaly,
leading to the diagnosis of Hodgkin’s disease.
LIEUTEANT COMMANDER STEPHEN B MILLER, MC USNR:
Chief, Rheumatology Section;
LIEUTENANT COMMANDER CHARLES J DONUN,
JR, Mc, UsN: Resident;
LIEUTENANT COMMANDER STEPHEN B MILLER, MC, USNR:
Staff Physician.
Address reprint requests to: Dr Stephen B Miller,
38 Grove Street, Ridgefield, Connecticut 06877.
Submitted for publication May 8, 1973; accepted
December 13, 1973.
598
CASE REPORT
Patient JB, a 46-year-old Caucasian male was in
excellent health until the fall of 1971 when he
began to experience episodic tenderness, swelling,
and redness of both ears. These manifestations
became persistent but were never associated with
Arthritis and Rheumatism, Vol. 17, No. 5 (September-October 1974)
HODGKIN’S DISEASE
Fig 1. Section of external ear cartilage showing destruction of cartilage and proliferation of adjacent connective tissue (H & E stain, 400X).
nasal or tracheobronchial symptoms. All laboratory
tests were unremarkable with the exception of a
persistently elevated sedimentation rate, in the 4050 m m / h r range by the Wintrobe method. Trials
with salicylates and indomethacin were unsuccessful
in suppressing the external ear inflammation, and
prednisone, in doses u p to 30 mg per day, was
required. T h e working diagnosis was relapsing
polychondritis.
T h e patient’s condition stabilized but frequent
attempts to lower the pretlnisone dose led to exacerbation of symptoms. In the spring of 1972 he
noted increasing fatigue associated with evening
fevers to 102°F and drenching night sweats. He was
then admitted to the hospital for a complete reevaluation. Physical examination was unremarkable;
specifically, there was no acute inflammation of the
ears, nor were any ocular, joint, or cardiac abnormalities noted. Laboratory studies revealed a hematocrit of 34%, a white blood cell count of 6200,
and a sedimentation rate of 45 mm/hr. Peripheral
blood smear showed normocytic, normochronic red
cells with marked rouleaux formation. Total protein was 9.3 g/lOO ml with a n albumin of 1.9 gyo
and a gamma globulin of 3.9 g/100 ml. There was
no gamma spike. T h e alkaline phosphatase was
elevated to 90 IU units (normal 11-36). An exten-
sive laboratory investigation was unrevealing, and
included normal serum electrolytes, BUN, creatinine, calcium, phosphorous, CPK, and transaminases. Latex fixation and LE preps were negative.
Chest X-ray, UGI series and barium enema were
normal, and fungal serologies and an intermediate
PPD skin test were negative. A liver scan and
biopsy were unremarkable as was a bone narrow
examination. Cultures of the blood, liver, and bone
marrow were negative. Biopsy of external ear cartilage showed changes coilsisterit with relapsing
polychondritis. There was loss of basophilic staining of the cartilaginous matrix with invasion of
proliferating connective tissue (Figure 1).
T h e hospital course was characterized by a slowly
declining hematocrit to 250/,, chronic fatigue, and
daily fevers to 104°F. T h e alkaline phosphdtase
continued elevated with mild transaminase elevation. Prednisone was increased to a maximal dose
of 100 mg per day with moderate improvement in
his symptoms. T h e fever subsided, his strength increased, and the sedimentation rate and alkaline
phosphatase levels declined, although not to normal. This improvement was short-lived however,
and he was readmitted for another evaluation.
His physical examination was essentially u n changed except for a cushinoid appearance: Labo-
Arthritis and Rheumatism, Vol. 17, No. 5 (September-October 1974)
599
MILLER ET AL
Fig 2. Section of spleen revealing Reed-Sternberg cell (H & E, 400X).
ratory studies revealed the alkaline phosphatase
again elevated to its previous levels, a leukocytosis
in the 15,000-30,000 range and elevated blood sugars which were felt to be steroid-induced. Bone
marrow biopsy and radiologic metastatic bone survey were unrevealing and a repeat liver biopsy
showed only mild fatty change. High fevers, night
sweats, and marked anemia continued. There was
no evidence of hemolysis.
Due to continued refractoriness to high dose
prednisone a trial of gradually increasing methotrexate (amethopterin) was begun. T h e prednisone
was tapered to 20 mg per day concomitant with
the methotrexate administration. No clinical improvement occurred and the methotrexate was discontinued. Subsequently new physical findings of a
palpable axillary node and splenomegaly were
noted. Biopsy of the node revealed Hodgkin’s disease of the mixed cell type. Staging laparotomy
revealed Hodgkin’s involvement in the spleen (Figure 2) with negative liver and para-aortic nodes.
The patient was then begun on cyclic chemotherapy, using nitrogen mustard, vincristine, procarbazinc, and prednisone. His clinical course deteriorated steadily however, and he died of staphylococcal pneumonia after completing two courses of
MOPP therapy.
600
DISCUSSION
T h e patient, as he originally presented,
demonstrated clinical and laboratory manifestations which supported the diagnosis of
relapsing polychondritis, ie, ear cartilage
inflammation, fever, hypergammaglobulinemia, anemia, elevated sedimentation rates,
and cartilaginous histologic confirmation.
He did not have cardiac, ocular, or joint
involvement. Initially his response to relatively low dose steroids was adequate and
consistent with the usual response in previously reported cases of relapsing polychondritis. Subsequently however, the illness changed character with the development of marked systemic symptoms. High
fevers, night sweats, weakness, and weight
loss were prominent features. I n addition
the liver function abnormalities and persistent anemia were more severe than previously recorded in the literature. It was
these atypical features and the inability to
Arthritis and Rheumatism, Vol. 17,
No. 5 (September-October 1974)
HODGKIN’S DISEASE
suppress disease activity with even high
dose steroids that led to an extensive search
for another illness.
Initial ear cartilage biopsy during a period of active inflammation failed to reveal
any cartilage despite sampling from usually
cartilaginous areas. T h e same situation occurred in case 2 of Dolan’s review (1). I n
both their case and ours however, characteristic cartilaginous findings were obtained
during the quiescent period.
Prior to the emergence of the diagnosis
of Hodgkin’s disease our attempts to suppress the systemic manifestations of the
polychondritis with a n immunosuppressive
agent ie, methotrexate, were unsuccessful.
A number of authors (1,2) have speculated that relapsing polychondritis has an
autoimmune etiology. Herman and Dennis
specifically studied this problem. I n a study
entitled Zmmunopathologic Studies in Relapsing Polychondritis (5), their findings
suggested a role for cellular immune mechanisms in the disease.
As noted earlier there has been only one
reference in the literature to relapsing polychondritis preceding the emergence of a
neoplasm (4). I n this report of two cases,
only one is accompanied by histologic documentation of cartilage involvement. I n
this case an SO-year-old man was found to
have carcinoma of the pancreas discovered
4 months after presenting with ear cartilage inflammation, elevated sedimentation
rates, and hypergammaglobulinemia.
hIiller (6) reported 17 patients who presented evidence of both malignant and immune disease. T h e disease associated with
the malignant lymphomas ranged from diffuse connective tissue disease such as rheumatoid arthritis and systemic lupus erythematosus to the nephrotic syndrome and
ataxia telangiectasis. They were referred to
as “immune diseases” in the sense that they
have a possible immunological etiology or
an associated immune disturbance.
Five of the 17 patients had Hodgkin’s
disease. Two patients experienced the simultaneous appearance of Hodgkin’s disease
with another disease, namely dermatomyositis in one and the nephrotic syndrome in
the other. Two additional patients showed
dissimultaneous appearance of Hodgkin’s
disease with another disease, namely lupus
erythematosus and discoid lupus erythematosus. T h e fifth case was originally diagnosed as Hodgkin’s disease, but after 4
years of clinical observation, the most compatible diagnosis was systemic lupus erythematosus.
Hench et a1 (7) found 22 examples of
diffuse connective tissue disease in 1000 patients with lymphoma and malignancies
(22y0).Lea (8) found a 6.5y0 presence of
both malignant lymphoproliferative disease
and rheumatoid arthritis compared to a
control population of 1.5y0. Two studies of
the prevalence of rheumatoid arthritis and
systemic lupus erythematosus in the general population (9,lO) give a combined prevalence of these disorders of only O.41y0.
We can only speculate whether the occurrence of relapsing polychondritis with
Hodgkin’s disease represents another example of the association of immune disease
with lymphoproliferative disorders.
ACKNOWLEDGMENT
The authors wish to thank the staff pathologists
of the Naval Hospital, Portsmouth, Virginia for
their assistance.
REFERENCES
1. Dolan DG, Lemmon GB, Teitelbaum: Relapsing polychondritis. Am J Med 41 :285-
299, 1966
2. Hollander JE, McCarty DJ: Arthritis and
Allied Conditions. Philadelphia, Lea and
Febiger, 1972
Arthritis and Rheumatism, Vol. 17, No. 5 (September-October 1974)
601
MILLER E l AL
3. Pearsori CM, Kline HM, Newcomer VD: Relapsing polychondritis. New Engl J Med 263:
51-58, 1960
4. Odkuist L: Relapsing polychondritis. Acta
Otolaryngo 70:448-454, 1970
5. Herman JH, Dennis MV: Immunopathologic studies in relapsing polychondritis. J
Clin Invest 52:549, 1973
6. Miller DG: T h e association of immune disease and malignant lymphoma. Ann Intern
Med 66:507-521, 1967
7. Hench PK, Mayne JG, Kiely JM, et al:
Clinical study of the rheumatic manifesta-
602
tions of lymphoma. Arthritis Rheum 5:301,
1962 (abstr)
8. Lea AJ: An association between the rheumatic diseases and the reticuloses. Ann
Rheum Dis 23:480,1964
9. Mikkelson WM, Dodge DJ, Duff I, et al:
Clinical and serological estimates of the
prevalence of rheumatoid arthritis in the
population of Tecumseh, Michigan. Arthritis Rheum 5:117, 1962 (abstr)
10. Siege1 M, Seelenfreund M: Racial and social factors in systemic lupus erythematosus.
JAMA 191:77, 1965
Arthritis and Rheumatism, Vol. 17, No. 5 (September-October 1974)
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associations, relapsing, polychondritis, disease, presenting, hodgkin, undescribed, previously
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