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Letters to the Editor 233
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Rheumatology 2001;40:233±235
Microscopic polyangiitis in a patient with relapsing
polychondritis
SIR, We report a 40-yr-old female patient in whom
relapsing polychondritis was associated with systemic
vasculitis affecting the skin, kidneys and lungs.
In 1997 the patient was admitted to our hospital
because of swelling and erythema of both ears (Fig. 1).
Clinical examination revealed in¯ammation of the
cartilage-containing portion of the ears with sparing of
the earlobe. The patient complained of generalized
arthralgia which had begun 2 years previously. The
laryngotracheal, nasal, ocular and vestibulocochlear
cartilages were unaffected. Laboratory investigation
showed an elevated erythrocyte sedimentation rate by
the Westergren method (80 mmuh), haematuria (250 red
blood cellsuml) and positive perinuclear antineutrophil
cytoplasmic antibodies (p-ANCA) at a dilution of 1:160.
A complete blood count and renal and liver function
tests were within the normal ranges, and cytoplasmic-
ANCA was negative. A biopsy of the ear showed
234 Letters to the Editor
chondrocyte degeneration with in®ltration of lympho-

cytes, macrophages and plasma cells, con®rming the
clinical diagnosis of relapsing polychondritis. Systemic
treatment with methylprednisolone at an initial daily
dose of 60 mg resulted in complete remission of the ear
swelling after 4 weeks without any further relapse.
However, arthralgia recurred as the dose of methyl-
prednisolone was tapered to 30 mg daily. Concomitant
treatment with sulphasalazine 1000 mg b.i.d. was
initiated but failed to improve the arthralgia consistently
within 6 months.
Twenty months after the ®rst signs of auricular
chondritis, the patient developed cough and haemo-
ptysis accompanied by Coombs-negative anaemia
(haemoglobin 9.6 gudl) and a marked reduction of her
general condition. High-resolution computed tomo-
graphy revealed an interstitial in®ltrative pattern that

was accompanied by an increased diffusion capacity on
pulmonary function tests. Treatment with clarithromycin
was started for presumed atypical pneumonia.
Because of persistent haematuria, proteinuria (0.5 g
per 24 h) and reduced creatinine clearance (60 mlumin),
a kidney biopsy was performed and revealed a non- FIG. 2. Livedo racemosa on the right foot as a cutaneous
granulomatous vasculitis of small and medium-sized manifestation of microscopic polyangiitis.
vessels with tubular atrophy and glomerular shrinkage
FIG. 3. Skin biopsy at the right ankle, revealing a non-

granulomatous, dense perivascular in®ltrate of neutrophils
and lymphocytes in the lower dermis. The vessel walls appear
FIG. 1. In¯ammation of the cartilage-containing portion of the thickened and destroyed by the in®ltrate. Haematoxylin and
ear produced by relapsing polychondritis. eosin staining. Original magni®cation, 3 400.
Letters to the Editor 235
and scarring. Additionally, our patient developed as a separately de®ned clinical complex, a signi®cant
Raynaud's phenomenon, livedo racemosa predomin- number of patients may simultaneously suffer from
antly on the right foot (Fig. 2) and diffuse, painful, other autoimmune diseases [9].
erythematous swelling at the right lateral ankle due Due to multiple organ involvement during the course
to a deep and tender in®ltration. Biopsy revealed a non- of disease, an interdisciplinary approach was necessary
granulomatous vasculitis of small and medium-sized in order to make the proper diagnosis. However, in this
vessels (Fig. 3) without immune deposits on direct case the dermatological ®ndings, i.e. livedo racemosa,
immuno¯uorescence. p-ANCA was still positive at cartilaginous in¯ammation of the ears and deep
a dilution of 1 : 5120. These laboratory results, in cutaneous in¯ammation at the right ankle, were crucial
conjunction with the clinical and histopathological in recognizing the disease.
features, led to the diagnosis of microscopic polyangiitis.
The p-ANCA-positive (non-granulomatous) vasculitis F. WEBER, E. KOWALD, M. SCHMUTH, N. SEPP
of small and medium-sized vessels with pulmonary ®nd- Department of Dermatology and Venereology,
ings (haemoptysis, haemorrhagic capillaritis, increased University of Innsbruck, Anichstrasse 35, 6020
carbon monoxide transfer) in the absence of eosino- Innsbruck, Austria
philia, asthma and immune deposits made other sys- Accepted 29 August 2000
temic vasculitis syndromes, such as polyarteritis nodosa, Correspondence to: F. Weber.

Wegener's granulomatosis, Churg±Strauss syndrome
and leukocytoclastic vasculitis, unlikely [1].
Treatment with three courses of cyclophosphamide 1. Jennette JC, Ronald JF. Small-vessel vasculitis. N Engl J Med
1997;337:1512±23.
1000 mg (20 mgukg) intravenously every 4 weeks with 2. Small P, Black M, Davidma M, de Champlain ML, Kapusta MA,
concomitant oral methylprednisolone resulted in marked Kreisman H. Wegener's granulomatosis and relapsing poly-
improvement of arthralgia and general physical condi- chondritis: a case report. J Rheumatol 1980;7:915±8.
tion and normalization of pulmonary diffusion capacity. 3. Thomas RH, Payne CM, Black MM. Polychondritis as
a concomitant feature of polyarteritis nodosa. Clin Exp Dermatol
Haematuria and anaemia were not detectable after the 1982;7:519±22.
third administration of cyclophosphamide, and oral 4. Conn DL, Dickson ER, Carpenter HA. The association of
methylprednisolone was tapered slowly over a period of Churg-Strauss vasculitis with temporal artery involvement, primary
9 months. biliary cirrhosis and polychondritis in a single patient. J Rheumatol
Relapsing polychondritis is a rare systemic auto- 1982;9:744±8.
5. Walker UA, Weiner SM, Vaith P, Uhl M, Peter HH. Aortitis in
immune disorder characterized by in¯ammation of relapsing polychondritis. Br J Rheumatol 1998;37:1359±61.
cartilaginous tissues. Systemic vasculitis syndromes 6. Varonos S, Kostaki M, Tsapra H, Pantelidaki A, Karpathios T.
seem to be a frequent concomitant feature of relapsing Polychondritis associated with Schonlein±Henoch purpura: report
polychondritis. The association with Wegener's granulo- of a case. Clin Exp Rheumatol 1994;12:443±5.
7. Bernard P, Bedane C, Delrous JL, Catanzano G, Bonnetblanc JM.
matosis [2], polyarteritis nodosa [3], Churg±Strauss Erythema elevatum diutinum in a patient with relapsing
syndrome [4], aortitis [5] and leukocytoclastic vasculitis, polychondritis. J Am Acad Dermatol 1992;26:312±5.
including SchoÈnlein±Henoch syndrome [6] and eryth- 8. McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM. Relapsing
ema elevatum diutinum [7], have been described in the polychondritis: prospective study of 23 patients and review of the
literature. Medicine 1976;55:193±215.
iterature. However, to the best of our knowledge, this 9. Michet CJ. Vasculitis and relapsing polychondritis. Rheum Dis Clin
is the ®rst report of microscopic polyangiitis preceded North Am 1990;16:441±4.
by the diagnosis of relapsing polychondritis. Retro-
spectively, positive p-ANCA, arthralgia recurring after
systemic steroid reduction and persistent haematuria
were early signs of systemic vasculitis in our patient.
Relapsing polychondritis with systemic vasculitis
generally re¯ects a poor prognosis, and the course of
disease is dictated by the manifestations and complica-
tions of the vasculitis [8, 9]. In microscopic polyangiitis,
haemorrhagic capillaritis is a very serious and often
life-threatening complication and the course of glomer-
ulonephritis usually shows rapid progression. In the
present case the disease had a relatively benign and
slowly progressive course, with complete remission after
immunosuppressive therapy with cyclophosphamide
and methylprednisolone.
The vasculitides mentioned above have a common
autoimmune pathogenesis. Relapsing polychondritis
occurs in association with many of these vasculitides,
suggesting that cartilaginous in¯ammation may be
a non-speci®c presentation of these syndromes. On the
other hand, while relapsing polychondritis occurs

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Letters to the Editor 233

Downloaded from rheumatology.oxfordjournals.org by guest on March 9, 2011

chondrocyte degeneration with in®ltration of lympho-

Downloaded from rheumatology.oxfordjournals.org by guest on March 9, 2011

FIG. 3. Skin biopsy at the right ankle, revealing a non-

Downloaded from rheumatology.oxfordjournals.org by guest on March 9, 2011

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