Unusual association between pure red cell aplasia and primary Sjögren's syndrome

A case report

Ilaria Cavazzana, Angela Ceribelli, Franco Franceschini, Roberto Cattaneo

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Pure red cell aplasia (PRCA) is an acute anemia due to selective suppression of erythropoiesis. We report a case of PRCA diagnosed before the onset of primary Sjögren's syndrome (SS). A young woman, with autoimmune thyroiditis, developed polyarthritis with ANA and Rheumatoid factor positivity, diagnosed as Rheumatoid arthritis. During the time she developed anti-Ro and anti-La antibodies and deformities at proximal interphalangeal joints. After 4 years, she developed severe acute anemia, without reticolocytes: a bone marrow biopsy indicated a PRCA and she started transfusions, steroids, cyclosporin. A steroid-dependent anemia persisted. After 2 years she developed sicca, parotid swelling, fatigue, mild leukopenia, elevated serum creatinine, hypokalemia, hyposthenuria and tubular proteinuria. Lip biopsy and dacriologic tests confirmed a diagnosis of SS, while X-ray revealed a deforming non-erosive arthropathy (Jaccoud type). In present case, PRCA could be considered an autoimmune bone marrow disease within SS, whose extra-glandular manifestations onset before the sicca symptoms.

Original languageEnglish
Pages (from-to)309-311
Number of pages3
JournalClinical and Experimental Rheumatology
Volume25
Issue number2
Publication statusPublished - Mar 2007

Fingerprint

Pure Red-Cell Aplasia
Anemia
Bone Marrow Diseases
Steroids
Biopsy
Autoimmune Thyroiditis
Hypokalemia
Joint Diseases
Rheumatoid Factor
Erythropoiesis
Leukopenia
Lip
Proteinuria
Cyclosporine
Arthritis
Fatigue
Anti-Idiotypic Antibodies
Creatinine
Rheumatoid Arthritis
Joints

Keywords

  • Anti-Ro/SSA
  • Jaccoud arthropathy
  • PRCA
  • Sjögren's syndrome

ASJC Scopus subject areas

  • Rheumatology
  • Immunology

Cite this

Unusual association between pure red cell aplasia and primary Sjögren's syndrome : A case report. / Cavazzana, Ilaria; Ceribelli, Angela; Franceschini, Franco; Cattaneo, Roberto.

In: Clinical and Experimental Rheumatology, Vol. 25, No. 2, 03.2007, p. 309-311.

Research output: Contribution to journalArticle

Cavazzana, Ilaria ; Ceribelli, Angela ; Franceschini, Franco ; Cattaneo, Roberto. / Unusual association between pure red cell aplasia and primary Sjögren's syndrome : A case report. In: Clinical and Experimental Rheumatology. 2007 ; Vol. 25, No. 2. pp. 309-311.
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