|LETTER TO THE EDITOR
|Year : 2018 | Volume
| Issue : 2 | Page : 84-85
Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) with Interstitial Lung Disease—An Unusual Association
Arjun Khanna, Ankit K Sinha, Kamal K Pandey
Pulmonary and Critical Care Medicine, Yashoda Super Speciality Hospitals Kaushambi, Ghaziabad, Uttar Pradesh, India
|Date of Web Publication||10-Jul-2018|
Consultant, Pulmonary and Critical Care Medicine, Yashoda Superspeciality Hospital, Kaushambi, UP
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Khanna A, Sinha AK, Pandey KK. Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) with Interstitial Lung Disease—An Unusual Association. J Assoc Chest Physicians 2018;6:84-5
|How to cite this URL:|
Khanna A, Sinha AK, Pandey KK. Remitting Seronegative Symmetrical Synovitis with Pitting Edema (RS3PE) with Interstitial Lung Disease—An Unusual Association. J Assoc Chest Physicians [serial online] 2018 [cited 2019 Jan 18];6:84-5. Available from: http://www.jacpjournal.org/text.asp?2018/6/2/84/236275
RS3PE (remitting seronegative symmetrical synovitis with pitting edema) is an uncommon, symmetrical, seronegative rheumatic disease, with acute onset pitting edema on the dorsa of bilateral hands and feet. It characteristically involves elderly males and shows good response to low doses of oral steroids, although respiratory involvement and complications are seen in a number of respiratory involvements that have not been reported with this disease entity, to the best of our knowledge.
A 75-year-old male, with preexisting hypertension being treated with atenolol, for the 5 years, presented with bilateral symmetrical synovitis involving predominantly the wrists, the small joints of the hand, and the flexor digitorum sheaths, along with marked dorsal swelling of the hands with pitting edema. There was no history of fever or involvement of other joints of the body. There was no history of any respiratory or significant cardiovascular diseases. The patient was active and was an avid jogger, who walked for 2 km every day, without any limitation. On examination, there was swelling over the metacarpophalangeal and proximal interphalangeal joints of all the digits and wrists. All movements across these joints were restricted. Rest of the musculoskeletal system examination was normal. He was evaluated somewhere else and tested negative for rheumatoid factor and anticyclic citrullinated peptide (anti-CCP) antibodies. Rest of the laboratory tests revealed hemoglobin of 11.2 g/dL, total leukocyte count: 18,080/mm3 with 64% neutrophils, platelet count: 529,000/mm3, blood urea: 48 mg/dL, creatinine: 1.4 mg/dL, uric acid: 6.8 mg/dL, aspartate transaminase (AST): 59 IU/L, alanine transaminase (ALT): 56 IU/L, alkaline phosphatase (ALP): 230 IU/L, lactate dehydrogenase (LDH): 250 U/L; acute phase reactants like erythrocyte sedimentation rate (ESR): 92 mm/h (Westergren method) and C-reactive protein: 102 mg/L were markedly elevated. Anti nuclear antigen (ANA) by enzyme linked immunosorbent assay (ELISA) was negative. Hand radiographs revealed narrowing between-joint spaces of all proximal and distal interphalangeal joints without any erosion. X-rays of other joints of the axial and appendicular skeleton were normal. Owing to the classical presentation, a diagnosis of RS3PE was made. The patient was started on Tablet Etoricoxib 120 mg and Tablet Wysolone 60 mg daily. There was marked improvement in the joint pains by day 3 of therapy. However, swelling over the joints persisted.
Around 3 weeks into the illness, the patient first noticed associated shortness of breath as he went for his morning walk. On examination, there were fine crackles bilaterally. Room air saturation was 92%, respiratory rate was 28/min, pulse rate 110/min. There was no pedal edema, and jugular venous pressure (JVP) was not raised. To evaluate the same, a 2D Echo was done which revealed grade 1 diastolic dysfunction. Chest X-ray Postero - Anterior (PA) view was suggestive of bilateral reticular shadows, more at the bases of the lungs. High-resolution computer tomography (CT) scan of the chest was done, which revealed bilateral ground glass opacities with basal predominance and immediate subpleural sparing, with bilateral linear and reticular opacities, commensurate with a diagnosis of cellular NSIP (nonspecific interstitial pneumonia).
Laboratory investigations were similar to the ones done 3 weeks ago. There was no evidence of infection. ESR was found to be 81 mm/h (Westergren method), and C-reactive protein was measured 74 mg/L. Rheumatoid factor, anti-CCP antibodies, ANA, and extended autoimmune profile for extractable nuclear antigen were conducted and found negative. Test for human leucocyte antigen (HLA) B27 was carried out, which came out to be positive. HLA B27 positivity has been described in RS3PE. Pulmonary function testing was conducted, which was suggestive of a moderately severe restrictive ventilator defect with severe impairment of the diffusion capacity. He was started on supplemental oxygen and intravenous methylprednisolone 40 mg twice daily, in conjugation with the rheumatologist. Over the next 7 days, the patient improved. Respiratory distress and cough decreased, and he was discharged on oral Deflazacort 30 mg daily.
NSIP pattern interstitial lung disease (ILD) has been well reported with a number of autoimmune and connective tissue disorders. The rarity of RS3PE as a disease entity and the association of new onset NSIP in this entity makes this case unique. RS3PE syndrome, an uncommon rheumatic disease, is seen most commonly in elderly males. It usually presents as acute-onset nonerosive polyarthritis with pitting edema over the extremities. The entity is exquisitely steroid responsive and sustained remissions are uncommon. RS3PE has also been reported to be the part of paraneoplastic syndromes with an underlying occult malignancy. A thorough clinical, radiological, and laboratory examination did not reveal any discernable malignancy in our patient. As respiratory involvement has typically not been described as a part of RS3PE, the exact management of this pattern of NSIP is not known. Our patient responded well to a short course of intravenous steroids and is doing well in follow-up. Follow-up pulmonary function testing and 6-min walk test have been planned in the due course of time.
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