CC BY
6mediterranean journal
of RHEUMATOLOGY
34 1
2023
G2023 The Author(s).
This work is licensed under a Creative Commons Attribution 4.0 International L
LETTER TO THE EDITOR
Organising Pneumonia as an Initial Manifestation of Rheumatoid Arthritis: A Case Report and Review of Literature
Nayan Patel Sureja1 , Anuradha T2
department of Rheumatology and Clinical Immunology, Star Hospitals, Hyderabad, India, 2Department of Pulmonology, Star Hospitals, Hyderabad, India
Mediterr J Rheumatol 2023;34(1):110-2 https://doi.Org/10.31138/mjr.34.1.110
Article Submitted: 10 Aug 2021; Revised Form: 23 Nov 2022; Article Accepted: 03 Mar 2023; Available Online: 31 Mar 2023
Keywords: interstitial lung disease, organising pneumonia, rheumatoid arthritis
Dear Editor,
Interstitial lung disease (ILD) is a frequent extra-articular manifestation of rheumatoid arthritis (RA), which usually manifests several years after the onset of articular symptoms. However, less than 10% of RA patients have ILD as an initial manifestation.1,2 Organising pneumonia (OP) preceding articular manifestations of RA is extremely uncommon with only few cases reported.1-8 A 28-year-old female with three months of gestation, presented with dry cough for one year, and symmetrical inflammatory polyarthritis involving small and large joints of all the extremities for one month. Examination revealed multiple tender joints, and swollen left wrist joint. Fine inspiratory crepitations were heard on auscultation over both the lungs. Rest of the physical examination was normal.
On evaluation, complete blood counts, urine examination, creatine phosphokinase, renal and liver function tests were normal. Computed tomography imaging of the chest (performed five months prior to the present presentation) was suggestive of OP (Figure 1). C-reactive protein was elevated, rheumatoid factor (RF) was positive (20 times above normal limit), and anti-cyclic citrullinated
peptide antibodies (ACPA) were negative. Anti-nuclear antibodies by indirect immunoflu-orescence on Hep-2 cells at 1:100 titre showed fine speckled nuclear pattern. Line immunoassays for myositis specific/asso-
Figure 1. Organising pneumonia. Computed tomography of the chest showing multiple consolidations with central ground glassing (reverse halo sign), predominantly in the peripheral and subpleural locations in both the lungs.
ciated and anti-extractable nuclear antigen antibodies were negative. Anti-neutrophilic cytoplasmic antibodies were negative, and serum complement (C3, C4) levels were normal. Forced vital capacity on spirometry was 80%. Sputum did not yield any pathogenic organisms. Polymerase chain reaction for mycobacterium tuberculosis antigen in the sputum was negative. Lung biopsy was
Corresponding Author:
Nayan Patel Sureja
Consultant, Department of Rheumatology
and Clinical Immunology
Star Hospitals, Banjara Hills, Hyderabad,
500034, Telangana, India
E-mail: [email protected]
110 Cite this article as: Sureja NP, Anuradha T. Organising Pneumonia as an Initial Manifestation of Rheumatoid Arthritis: A Case Report and Review of Literature. Mediterr J Rheumatol 2023;34(1):110-2.
ORGANISING PNEUMONIAAS AN INITIAL MANIFESTATION OF RHEUMATOID ARTHRITIS:
A CASE REPORT AND REVIEW OF LITERATURE
Table 1. Previously reported cases of rheumatoid arthritis with organising pneumonia as an initial manifestation.
Author Age Sex Arthritis onset after OP (months) RF at onset of OP RF at onset of arthritis ACPA at onset of OP ACPA at onset of arthritis Treatment
Kalinova et al.3 56 F 4 + + + + MP 40 mg
Hoshino et al.4 71 M 0.75 + + + + Pred 30 mg
Kinoshita et al.1 58 M 24 + + + + Pred 40 mg
Komiya et al.2 86 F 8 + + + + Pulse steroid
Cavallasca et al.5 65 M 6 - + NA + Pred 80 mg
Ippolito et al.6 68 F 0.5 NA + NA NA Pred 60 mg
Henriet et al.7 69 F 8 NA + NA + Pred 1 mg/kg
33 F 3 NA + NA + Pred 40 mg
Mori et al.8 41 F 32 + + + + Pred 40 mg
53 M 27 + + + + Pulse followed by Pred 50 mg
65 F 4 + + + + Pulse followed by Pred 50 mg
This case 28 F 11 NA + NA - Pred 30 mg
F: Female; M: Male; MP: Methylprednisolone; Pred: Prednisolone; OP: Organising pneumonia; RA: Rheumatoid arthritis; RF: Rheumatoid factor; ACPA: Anti-cyclic citrullinated peptide antibodies.
deferred, as the clinical and radiological characteristics of the lung lesions were consistent with OP. With a diagnosis of seropositive RA with ILD, patient received oral prednisolone 30 mg/day (with tapering of 5 mg every fortnightly), and sulfasalazine 2 gms/day. Within next few days, her pulmonary and articular symptoms subsided. OP is a type of ILD, histologically characterized by buds of granulation tissue within the bronchioles and alveoli.6 It is called cryptogenic OP when the etiology remains uncertain, and secondary OP when associated with infections, drugs, and connective tissue diseases including RA.9 Compared with other types of ILD seen in RA, such as nonspecific interstitial pneumonia and usual interstitial pneumonia, OP is less common and has better prognosis.8 Occasionally OP may resolve spontaneously. However, most of the patients require glucocorticoids, which is the standard of care. Cyclophosphamide can be used in patients not responding to corticosteroids.10 PubMed search with terms "rheumatoid arthritis" AND ("organising pneumonia" OR "organizing pneumonia" OR "organising pneumonitis" OR "organizing pneumonitis" OR "BOOP" OR "interstitial lung disease") identified 11 patients of RA with OP as an initial manifestation, published in English literature.1-8 Clinical characteristics of these patients including the present case are summarised in Table 1. The median age of these patients was 61.5 years (range: 28-86), with present case being the youngest, and 8/12 patients were female.
At the onset of respiratory symptoms, RF and ACPA were positive in 7/8 and 7/7 patients respectively. Whereas at the onset of articular symptoms, RF was positive in all the patients, and 10/11 patients had ACPA. In the present case it is uncertain whether the antibodies were present at the onset of ILD or developed thereafter. The present case along with previously published cases emphasises on the importance of testing for RF and ACPA, and a cautious follow-up for development of arthritis in patients with cryptogenic OP.
CONSENT
Informed consent could not obtained, as we lost contact with the patient. However, patient's identity has not been revealed.
CONFLICT OF INTEREST
The authors declare no conflict of interest.
REFERENCES
1. Kinoshita Y, Sakamoto A, Hidaka K. Organizing pneumonia preceding rheumatoid arthritis. Case Rep Pulmonol 2014;2014:758619.
2. Komiya K, Teramoto S, Kurosaki Y, Kashizaki F, Kawashima M, Masuda K, et al. Organizing pneumonia with a positive result for anti-CCP antibodies as the first clinical presentation of rheumatoid arthritis. Intern Med 2010;49:1605-7.
3. Kalinova D, Kolarov Z, Rashkov R. Organising pneumonia - the first manifestation of rheumatoid arthritis. Reumatologia 2017;55:314-7.
4. Hoshino C, Satoh N, Narita M, Kikuchi A, Inoue M. Organising
mediterranean journal 34
of RHEUMATOLOGY 2023
pneumonia as the first manifestation of rheumatoid arthritis. BMJ Case Rep 2011;2011:bcr1120103558.
5. Cavallasca JA, Caubet M, Helling CA, Tate GA. Cryptogenic organizing pneumonia (COP), as presentation of rheumatoid arthritis. Rheumatol Int 2008;29:99-101.
6. Ippolito JA, Palmer L, Spector S, Kane PB, Gorevic PD. Bronchiolitis obliterans organizing pneumonia and rheumatoid arthritis. Semin Arthritis Rheum 1993;23:70-8.
7. Henriet AC, Diot E, Marchand-Adam S, de Muret A, Favelle O, Crestani B, et al. Organising pneumonia can be the inaugural manifestation in connective tissue diseases, including Sjogren's syndrome. Eur Respir Rev 2010;19:161-3.
8. Mori S, Koga Y, Sugimoto M. Organizing Pneumonia in Rheumatoid Arthritis Patients: A Case-Based Review. Clin Med Insights Circ Respir Pulm Med 2015;9:69-80.
9. Drakopanagiotakis F, Polychronopoulos V, Judson MA. Organizing pneumonia. Am J Med Sci 2008;335:34-9.
10. Raghu G, Meyer KC. Cryptogenic organising pneumonia: current understanding of an enigmatic lung disease. Eur Respir Rev 2021;30:210094.
