Sjogren's syndrome presenting with hypereosinophilia, lymphopenia and circulating immune complexes

J. Farnam, J. L. Jorizzo, J. A. Grant

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Abstract

Although mild peripheral eosinophilia is a common finding in Sjogren's syndrome (SS), severe eosinophilia with a clinical picture simulating hypereosinophilic syndrome is extremely rare. We report a 24 year old male with SS presenting with swelling of the parotid glands, redness and irritation of the eyes, polyarthralgias and polyarthritis, weight loss, exertional dyspnea, malaise, erythematous and urticarial skin lesions and enlarged lymph nodes. Laboratory tests showed hypereosinophilia (34%, total 3800/mm3), lymphopenia (2%, total 220/mm3), a positive RA factor (1:2560) and decreased C3 and C4. Biopsy of an enlarged submaxillary gland was consistent with SS. A Schirmer test showed decreased tear production. Salivary glands showed a marked decrease in uptake of radioactive (Tc99) dye. Circulating immune complexes (CIC) were markedly elevated by both Clq binding and Raji cell assays. T-cell subsets showed OKT3=63%, OKT4=32% and OKT8=16%. 'Histamine trap' in vivo test for CIC revealed fluorescence in upper dermal blood vessels with IgM, Clq, C3 and fibrin. Biopsies of the liver, bone marrow and skin revealed eosinophilic infiltration. A notable response to therapy with high doses of corticosteroids was seen with recurrence of symptoms and laboratory abnormalities after the therapy was stopped. In conclusion, we present a case of SS which is remarkable for the age and sex of the patient, extreme hypereosinophilia, marked lymphopenia, and CIC.

Original languageEnglish (US)
Pages (from-to)41-46
Number of pages6
JournalClinical and Experimental Rheumatology
Volume2
Issue number1
StatePublished - 1984

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Lymphopenia
Sjogren's Syndrome
Antigen-Antibody Complex
Eosinophilia
Skin
Hypereosinophilic Syndrome
Biopsy
Muromonab-CD3
Submandibular Gland
Parotid Gland
Arthralgia
T-Lymphocyte Subsets
Salivary Glands
Fibrin
Tears
Dyspnea
Histamine
Arthritis
Immunoglobulin M
Blood Vessels

ASJC Scopus subject areas

  • Immunology
  • Rheumatology

Cite this

Sjogren's syndrome presenting with hypereosinophilia, lymphopenia and circulating immune complexes. / Farnam, J.; Jorizzo, J. L.; Grant, J. A.

In: Clinical and Experimental Rheumatology, Vol. 2, No. 1, 1984, p. 41-46.

Research output: Contribution to journalArticle

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abstract = "Although mild peripheral eosinophilia is a common finding in Sjogren's syndrome (SS), severe eosinophilia with a clinical picture simulating hypereosinophilic syndrome is extremely rare. We report a 24 year old male with SS presenting with swelling of the parotid glands, redness and irritation of the eyes, polyarthralgias and polyarthritis, weight loss, exertional dyspnea, malaise, erythematous and urticarial skin lesions and enlarged lymph nodes. Laboratory tests showed hypereosinophilia (34{\%}, total 3800/mm3), lymphopenia (2{\%}, total 220/mm3), a positive RA factor (1:2560) and decreased C3 and C4. Biopsy of an enlarged submaxillary gland was consistent with SS. A Schirmer test showed decreased tear production. Salivary glands showed a marked decrease in uptake of radioactive (Tc99) dye. Circulating immune complexes (CIC) were markedly elevated by both Clq binding and Raji cell assays. T-cell subsets showed OKT3=63{\%}, OKT4=32{\%} and OKT8=16{\%}. 'Histamine trap' in vivo test for CIC revealed fluorescence in upper dermal blood vessels with IgM, Clq, C3 and fibrin. Biopsies of the liver, bone marrow and skin revealed eosinophilic infiltration. A notable response to therapy with high doses of corticosteroids was seen with recurrence of symptoms and laboratory abnormalities after the therapy was stopped. In conclusion, we present a case of SS which is remarkable for the age and sex of the patient, extreme hypereosinophilia, marked lymphopenia, and CIC.",
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