Letters To The Editors: Primary Sjögren's Syndrome As Paraneoplastic Disorder: A Case Report

Letters to the Editors
Primary Sjögren’s syndrome Fig. 1. Abdomen

CT scan showing
as paraneoplastic disorder: enhancing lesion in
a case report lower pole of right
kidney (arrow).
Sirs,
A 57-year-old man was referred for a 4-
month history of symmetrical arthritis at
wrist and hand small joints with concomi-
tant oral and ocular sicca syndrome. Patient
reported heavy cigarette smoking. Except-
ing a systemic arterial hypertension on cal-
cium channel blocker therapy, his medical
history was otherwise unremarkable.
Administration of medications known to
cause sicca symptoms as well as the most
common causes of sicca syndrome, in-
cluding hepatitis C virus or HIV infection,
diabetes mellitus, thyroid dysfunction and
sarcoidosis, were ruled out. Antinuclear
antibodies (ANA) were positive at im-
munofluorescence assay on Hep-2 cells at
1:640 titer with a fine granular pattern of
nucleoplasm and weak staining of the nu-
cleoli. Anti-SSA(Ro) 52/60 and anti-SSB cluding rheumatoid arthritis (RA) and SS, human renal carcinoma cells and monkey
(La) antibodies were detected at high titer are associated with a high long-term risk kidney fibroblasts (5). Thus, the induction
by enzyme immunoassay. Serum levels of of developing lymphoproliferative disor- of a specific humoral and cell-mediated au-
other immunological parameters, includ- ders and solid tumors. On the other hand, toimmune response by some cancer-associ-
ing C-reactive protein, rheumatoid factor, some RDs may be associated with or pre- ated antigens may be a possible pathogenic
C3 and C4, IgG, IgA and IgM and anti-cy- cede the clinical manifestations of a vari- mechanism leading to paraneoplastic syn-
clic peptide citrullinated antibodies, were ety of solid and haematological cancers. In drome clinical manifestations.
normal. Impaired lacrimal and salivary se- this setting, inflammatory myopathies, se-
cretion was confirmed by Schirmer’s and ronegative RA, some atypical vasculitides E. BARTOLONI, MD
unstimulated whole salivary flow tests, and scleroderma-like syndromes are the A. ALUNNO, MD
respectively. Parotid ultrasonography re- most frequently reported paraneoplastic R. GERLI, MD
sulted normal. Finally, minor salivary gland RDs (2). Their prevalence ranges from 3% Rheumatology Unit, Department of Clinical &
biopsy showed a mild, non-specific, mono- to 15% and etiopathogenesis has not been Experimental Medicine, University of Perugia,
nuclear cell infiltrate. fully clarified (3). Symmetrical small joints Perugia, Italy.
A diagnosis of primary SS was made ac- polyarthritis is the most common articular Address correspondence to:
cording to the American-European Con- paraneoplastic manifestation, together with Prof. Roberto Gerli, MD, Rheumatology Unit,
Department of Clinical and Experimental
sensus Group classification criteria (1) and Raynaud phenomenon (3). Rapid onset of Medicine, Università di Perugia, via dal Pozzo,
low-dose corticosteroid (CS) treatment was an unusual inflammatory polyarthritis in 06122 Perugia, Italy.
introduced to control articular pain. How- subjects older than 50 years without fam- E-mail: gerlir@unipg.it
ever, arthritis remission was not achieved ily history for RDs, smoking habit and poor Competing interests: none declared.
despite prednisone doses up to 25 mg daily. response to CS and/or immunosuppressive
Because of the poor response to CS therapy therapy have been identified as helpful clues References
and the atypical late onset of a connective to suspect paraneoplastic conditions (3). 1. VITALI C, BOMBARDIERI S, JONSSON R et al.;
tissue disease in a male subject, screening Of interest, kidney and urogenital tumors FOR EUROPEAN STUDY GROUP ON CLASSIFICATION
tests were performed to exclude an occult have been frequently reported in paraneo- CRITERIA FOR SJÖGREN’S SYNDROME: Classification
criteria for Sjögren’s syndrome: a revised version
underlying pathologic condition. Chest ra- plastic RDs (3). In general, immunologic
of the European criteria proposed by the American-
diography, dosage of prostate specific an- parameters are not helpful to discriminate European Consensus Group. Ann Rheum Dis 2002;
tigen and fecal occult blood resulted nega- such conditions and increased incidence of 61: 554-8.
tive, whereas upper abdominal US revealed ANA positivity without recognition of ex- 2. RACANELLI V, PRETE M, MINOIA C et al.: Rheu-
a right kidney mass confirmed by computed tractable nuclear antigen specificity usually matic disorders as paraneoplastic syndromes. Au-
tomography scan. One month later, patient characterises these patients (3). toimmun Rev 2008; 7: 352-8.
3. RUGIENÉ R, DADONIENÉ J, ALEKNAVIČIUS E
underwent right total nephrectomy. Histo- The present clinical case is of particular et al.: Prevalence of paraneoplastic rheumatic sin-
logical examination was consistent with interest as it is a report of a paraneoplas- drome and their antibody profile among patients
multilocular cystic clear cell carcinoma, tic SS, a CTD almost exclusively affecting with solid tumours. Clin Rheumatol 2011; 30: 373-
Fuhrman grade II, not infiltrating renal young women, occurring in a male subject 80.
capsule. Complete remission of arthritis and completely resolved after neoplasm re- 4. GERSTEN DM, BIJWAARD KE, LAW LW et al.:
moval. From a pathogenic point of view, it Homology of the B50 murine melanoma antigen to
and sicca symptoms were rapidly obtained
the Ro/SS-A antigen of human systemic lupus ery-
following neoplasm removal. Anti-Ro/SSA is intriguing the finding that some specific thematosus and to calcium-binding proteins. Bio-
and anti-La/SSB antibodies slowly de- cancer-associated antigens display a sig- chim Biophys Acta 1990; 1096: 20-5.
creased and resulted negative three months nificant structural homology with Ro/SSA 5. DI DONATO F, CHAN EK, ASKANASE AD et al.:
after surgery. Patient is still asymptomatic antigen (4). In addition, Ro52 antigen has Interaction between 52 kDa SSA/Ro and deubiq-
at nine months of follow-up. been shown to interact with a specific deu- uitinating enzyme UnpEL: a clue to function. Int J
Biochem Cell Biol 2001; 33: 924-34.
It is well established that some chronic in- biquitinating enzyme, UnpEL, commonly
flammatory rheumatic diseases (RDs), in- detected in cultured human cardiocytes,
454

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Letters to the Editors

Primary Sjögren’s syndrome Fig. 1. Abdomen

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