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 Table of Contents  
IMAGES IN RHEUMATOLOGY
Year : 2018  |  Volume : 13  |  Issue : 3  |  Page : 207-208

Inflammatory polyarthritis: It's Not always about the Joints!


1 Departments of Rheumatology, Saveetha Medical College Hospital, Chennai, Tamil Nadu, India
2 General Medicine , Pathology Saveetha Medical College Hospital, Chennai, Tamil Nadu, India
3 Pathology, Saveetha Medical College Hospital, Chennai, Tamil Nadu, India

Date of Web Publication21-Aug-2018

Correspondence Address:
Dr. Kavitha Mohanasundaram
6, First Street Shanthi Nagar, Adambakkam, Chennai - 600 088, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/injr.injr_40_18

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  Abstract 


Keywords: Non-Hodgkin's lymphoma, paraneoplastic arthritis, RS3PE


How to cite this article:
Mohanasundaram K, Nithyananthan P, Vimalchander R, Yogalakshmi E, Sulochana S, Gowrishankar A. Inflammatory polyarthritis: It's Not always about the Joints!. Indian J Rheumatol 2018;13:207-8

How to cite this URL:
Mohanasundaram K, Nithyananthan P, Vimalchander R, Yogalakshmi E, Sulochana S, Gowrishankar A. Inflammatory polyarthritis: It's Not always about the Joints!. Indian J Rheumatol [serial online] 2018 [cited 2019 Oct 17];13:207-8. Available from: http://www.indianjrheumatol.com/text.asp?2018/13/3/207/236716



Inflammatory polyarthritis is a great mimicker of many systemic illnesses, ranging from hypothyroidism to malignancy. The image reported below describes a paraneoplastic presentation in rheumatology and emphasizes the need for a detailed clinical examination in all cases of inflammatory polyarthritis, particularly in those with atypical manifestations.

A 34-year-old male was referred to us with symptoms of joint pain, swelling, and inability to make a fist for a period of 20 days. History revealed that the patient had diarrhea and had been treated at the nearby hospital with antibiotics 2 weeks before the onset of joint pains. The patient did not have any history of psoriasis, early morning stiffness, or sicca symptoms. On examination, the patient had symmetrical polyarthritis with dactylitis [Figure 1]a. No skin lesions or associated conjunctivitis was noted. A working diagnosis of reactive arthritis was made, and the patient was started on nonsteroidal anti-inflammatory drugs (NSAIDs). Investigations revealed hemoglobin of 10.4 g/dL, total count of 18,000 cells/cu.mm and platelet count was 9.84 lakhs/cu.mm, erythrocyte sedimentation rate was 10 mm/h, and C-reactive protein and rheumatoid factor (RF) were negative. Peripheral smear showed eosinophilia and thrombocytosis. During the second visit, the pain was persistent, and he had bilateral hand edema which was of pitting type [Figure 1]b. Ultrasound revealed diffuse edema of the hands with tenosynovitis of extensor tendon sheath of the hands. The differential diagnosis entertained was reactive arthritis (in favor: short duration of illness, preceding diarrhea, and dactylitis; points against: predominant upper-limb involvement, small joint arthritis along with significant edema, and the absence of enthesitis), reflex sympathetic dystrophy (in favor: edema disproportionate to arthritis and associated intense pain; points against: usually unilateral and no prior trauma), and remitting seronegative symmetrical synovitis with pitting edema (RS3PE) (in favor: affecting both upper and lower limbs, pitting nature of edema, and RF negativity; points against: age – generally more common in the elderly). In our patient, though he was middle-aged, the symmetrical nature of involvement made us to consider RS3PE as a differential diagnosis.
Figure 1: (a) Initial presentation showing suspected dactylitis of the hand and polyarthritis with minimal edema. (b) Pitting edema of both hands as noted in the next visit

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The patient was subjected to a further detailed clinical examination. He had a 2.5-cm firm lymph node in the inguinal region on the left side, no sternal tenderness, or hepatosplenomegaly. Fine-needle aspiration cytology (FNAC) of the inguinal lymph node showed sheets of lymphocytes in various stages of maturation, histiocytes, plasma cells with occasional neutrophils, and eosinophils. Computerized tomography of the chest and abdomen revealed bilateral axillary (maximum size 8 mm), aortocaval, external iliac, internal iliac, and inguinal adenopathy [Figure 2]a. FNAC and tissue biopsy of the inguinal lymph node were suggestive of non-Hodgkin's lymphoma (NHL) [Figure 2]b. A diagnosis of RS3PE as the first clinical presentation of NHL was made, and the patient was initiated on chemotherapy, which is being continued.
Figure 2: (a) Computed tomography of the chest showing the presence of axillary lymphadenopathy. (b) Histopathological examination of inguinal node showing complete effacement of architecture with sheets of lymphoid cells with pleomorphism and mitotic figures

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RS3PE is a rare rheumatological entity, considered as a variant of late-onset rheumatoid arthritis by many. It is characterized by significant edema of bilateral upper limbs or lower limbs, and the former is the most common. It has a predilection to affect the male and the elderly population and hence considered as a differential diagnosis of late-onset rheumatoid arthritis and polymyalgia rheumatica. RF negativity and lack of bone erosions are major differentiating features.[1] This was described as a distinct clinical entity by McCarty et al. in 1985, and its association with malignancy has been described previously.[2] Although a validated classification criterion is not yet available for RS3PE, a proposed criterion by Olivieri et al. describes this entity by the following features: (a) age >50 years, (b) RF negativity, (c) robust onset of polyarthritis, and (d) symmetrical pitting edema.[3] The age of presentation was much younger in our patient, which can occur when this entity is associated with malignancy. RS3PE has been known to be associated with both solid organ and hematological malignancies, at times being the first presentation.[4] NHL has been previously reported to be associated with RS3PE, though frank arthritis in NHL is considered rare when compared to leukemias.[5],[6]

RS3PE shows a good response to steroids with no major residual damage.[7] The response to steroids is poor when it occurs as a part of paraneoplastic syndrome. The presence of disproportionate edema, lymphadenopathy, poor response to NSAIDs, absence of RF, and anticyclic citrullinated peptide in a case of arthritis should prompt us toward a possibility of systemic illness. This entity can occur in the middle-aged people too and often warrants a close search for a possible malignancy as evidenced in our case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Yao Q, Su X, Altman RD. Is remitting seronegative symmetrical synovitis with pitting edema (RS3PE) a subset of rheumatoid arthritis? Semin Arthritis Rheum 2010;40:89-94.  Back to cited text no. 1
    
2.
McCarty DJ, O'Duffy JD, Pearson L, Hunter JB. Remitting seronegative symmetrical synovitis with pitting edema. RS3PE syndrome. JAMA 1985;254:2763-7.  Back to cited text no. 2
    
3.
Olivieri I, Salvarani C, Cantini F. RS3PE syndrome: An overview. Clin Exp Rheumatol 2000;18:S53-5.  Back to cited text no. 3
    
4.
Matsunaga T, Izumi Y, Iwanaga N, Kawahara C, Shigemitsu Y, Yoshida S, et al. Myelodysplastic syndrome precedes the onset of remitting seronegative symmetrical synovitis with pitting edema (RS3PE) syndrome. Tohoku J Exp Med 2015;235:47-52.  Back to cited text no. 4
    
5.
Ravindran V, Anoop P. Rheumatologic manifestations of benign and malignant haematological disorders. Clin Rheumatol 2011;30:1143-9.  Back to cited text no. 5
    
6.
Goldenberg K, Rozenbaum M, Rosner I, Naschitz J, Kotler C. Remitting symmetric seronegative synovitis with pitting edema (RS3PE) secondary to non-Hodgkin's lymphoma. Clin Exp Rheumatol 1998;16:767-8.  Back to cited text no. 6
    
7.
Li H, Altman RD, Yao Q. RS3PE: Clinical and research development. Curr Rheumatol Rep 2015;17:49.  Back to cited text no. 7
    


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  [Figure 1], [Figure 2]



 

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