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Not All Rheumatoid Factor-Positive Tests Mean RA

Francis Essien, DO, & Matthew B. Carroll, MD, FACP, FACR  |  Issue: April 2020  |  April 15, 2020

This CD3 stain at 40x magnification of the retroperitoneal lymph node demonstrates diffuse uptake.

This CD3 stain at 40x magnification of the retroperitoneal lymph node demonstrates
diffuse uptake.

A case report in 1982 by Meyers et al. discussed a patient who presented with generalized arthralgias and failure to respond to conventional therapy for RA who was subsequently diagnosed with AITL.5

Another case report in 1986 by Bignon et al. reported the development of AITL in a patient with sicca symptoms.8 AITL has been reported to arise from extra-nodal lymphoid tissue, and in the case reported by Bignon et al., lymphoproliferation was observed involving the lip of the patient with the immunohistological comparison similar to the primary site of the kidney.9-11

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Pathak et al. reported a case of AITL in association with Guillain-Barré syndrome diagnosed after rapid onset of ascending lower extremity paralysis.12 In this case the patient developed lymphadenopathy after being discharged, and immunohistological biopsy later revealed the diagnosis.12

The correlation of malignancy with rheumatic illness is a complex, fascinating aspect of immunology. The arthritis associated with AITL can present very similarly to that of RA, with symmetric involvement of proximal joints. It is usually non-erosive, sero­negative and non-deforming, but is typically associated with constitutional symptoms and elevated markers of inflammation.

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Although [the G17V mutation of Ras homolog family member A] mutation has also been identified in other malignancies, … it may serve as a non-invasive diagnostic test for AITL in the future.

The arthritis of AITL responds well to low to moderate prednisone doses, but remains unresponsive to disease-modifying anti-rheumatic drugs and biologic response modifiers. Our patient demonstrated this with multiple relapses after his prednisone dose was decreased.

This case highlights several diagnostic and therapeutic challenges. First, not all positive rheumatoid factor tests are associated with RA.

Second, a patient’s difficulty tolerating a taper in prednisone, especially when used in conjunction with other disease-modifying drugs or biologic response modifiers, should prompt the rheumatologist to consider an alternative etiology for arthritis.

Finally, failure of RA to respond to triple or even quadruple therapy should also prompt concern for an alternative etiology.


Francis Essien, DO, is an internal medicine resident at Keesler Medical Center, Keesler Air Force Base, Miss.

Matthew B. Carroll, MD, FACP, FACR, is a rheumatologist with Singing River Health System, Ocean Springs, Miss.

References

  1. Lunning MA, Vose JM. Angioimmunoblastic T-cell lymphoma: The many-faced lymphoma. Blood. 2017 Mar 2;129(9):1095–1102.
  2. Mangana J, Guenova E, Kerl K, et al. Angioimmunoblastic T-Cell lymphoma mimicking drug reaction with eosinophilia and systemic symptoms (DRESS syndrome). Case Rep Dermatology. 2017 Mar 21;9(1):74–79.
  3. Fukumoto K, Nguyen TB, Chiba S, Sakata-Yanagimoto M. Review of the biologic and clinical significance of genetic mutations in angioimmunoblastic T-cell lymphoma. Cancer Sci. 2018 Mar;109(3):490–496.
  4. Yachoui R, Farooq N, Amos JV, Shaw GR. Angioimmunoblastic T-cell lymphoma with polyarthritis resembling rheumatoid arthritis. Clin Med Res. 2016 Dec;14(3–4):159–162.
  5. Meyers OL, Klemp P. Lymph node enlargement in rheumatoid arthritis. A case of angio-immunoblastic lymphadenopathy. S Afr Med J. 1982 Dec 25;62(27):1038–1039.
  6. Mukherjee T, Dutta R, Pramanik S. Aggressive angioimmunoblastic T cell lymphomas (AITL) with soft tissue extranodal mass varied histopathological patterns with peripheral blood, bone marrow, and splenic involvement and review of literature. Indian J Surg Oncol. 2018 Mar;9(1):11–14.
  7. Ozoran K, Turgay M, Kinikli G, et al. Angioimmunoblastic lymphadenopathy with dysproteinemia arising in a patient with rheumatoid arthritis. Scand J Rheumatol. 1995;24(1):58–60.
  8. Bignon Y, Mercier A, Dubost J, Ristori J, et al. Angioimmunoblastic lymphadenopathy with dysproteinaemia (AILD) and sicca syndrome. Ann Rheum Dis. 1986 Jun;45(6):519–522.
  9. Hashefi M, McHugh TR, Smith GP, et al. Seropositive rheumatoid arthritis with dermatomyositis sine myositis, angioimmunoblastic lymphadenopathy with dysproteinemia-type T cell lymphoma, and B cell lymphoma of the oropharynx. J Rheumatol. 2000 Apr;27(4):1087–1090.
  10. Kojima M, Motoori T, Hosomura Y, et al. Atypical lymphoplasmacytic and immunoblastic proliferation from rheumatoid arthritis: A case report. Pathol Res Pract. 2006;202(1):51–54.
  11. Pierce DA, Stern R, Jaffe R, et al. Immunoblastic sarcoma with features of Sjögren’s syndrome and systemic lupus erythematosus in a patient with immunoblastic lymphadenopathy. Arthritis Rheum. 1979 Aug;22(8):911–916.
  12. Pathak P, Perimbeti S, Ames A, Moskowitz A. Guillain Barré syndrome heralding the diagnosis of angioimmunoblastic T-cell lymphoma. Leuk Lymphoma. 2019 Jul;60(7):1835–1838.

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