The Journal of Association of Chest Physicians

: 2021  |  Volume : 9  |  Issue : 1  |  Page : 45--48

Organizing pneumonia as preliminary presentation of rheumatoid arthritis: A case report and review of literature

Vikrant Ranga1, Amitesh Gupta2, Parul Mrigpuri3, Sonam Spalgais4,  
1 MD Pulmonary Medicine, Consultant and Head of Department, Department Pulmonary Medicine, Dr. Prem Superspeciality Hospital, Panipat, Haryana, India
2 MD Pulmonary Medicine, Medical Officer, Department of TB and Respiratory Diseases, National Institute of Tuberculosis and Respiratory Diseases, New Delhi, India
3 MD Pulmonary Medicine, Assistant Professor Department Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, New Delhi, India
4 DNB Pulmonary Medicine, Assistant Professor Department Pulmonary Medicine, Vallabhbhai Patel Chest Institute, University of Delhi, New Delhi, India

Correspondence Address:
Dr. Parul Mrigpuri
Assistant Professor, Department of Pulmonary Medicine, Vallabhbhai Patel Chest Institute, Delhi-110007


Rheumatoid arthritis (RA) is a systemic disease frequently involving the respiratory system. Organizing pneumonia (OP) is a defined pulmonary manifestation seen in patients with RA-associated interstitial lung disease. Even though OP following articular involvement is common, rarely it could be the first manifestation of RA. We herein report a case of 45-year-old female, who presented with OP as the first manifestation of RA with negative serology at presentation.

How to cite this article:
Ranga V, Gupta A, Mrigpuri P, Spalgais S. Organizing pneumonia as preliminary presentation of rheumatoid arthritis: A case report and review of literature.J Assoc Chest Physicians 2021;9:45-48

How to cite this URL:
Ranga V, Gupta A, Mrigpuri P, Spalgais S. Organizing pneumonia as preliminary presentation of rheumatoid arthritis: A case report and review of literature. J Assoc Chest Physicians [serial online] 2021 [cited 2021 Apr 10 ];9:45-48
Available from:

Full Text


Rheumatoid arthritis (RA) is a systemic inflammatory disease. Pulmonary complications are frequently seen in RA and are responsible for 10–20% of mortality in patients of RA. Pulmonary manifestation of RA mainly includes interstitial lung disease (ILD), airway complications, pleural involvement, chest wall abnormalities, and vascular complications.[1] ILD is a common pulmonary manifestation of RA.[2] The prevalence of ILD in RA (high resolution computed tomography based diagnosis) varies from 20% to 44%. Generally, the onset of symptomatic ILD is preceded by joint symptoms by up to 5 years. Organizing pneumonia (OP) with patchy areas of consolidation is one of the major radiological patterns seen in patients with RA-associated ILD.[2] We present a case of OP as the initial manifestation of RA with negative serology at presentation.


A 45-year-old female presented to us with chief complaints of fever, dry cough, and shortness of breath for two weeks. She was a nonsmoker, and there was no history of long-term drug intake or radiation exposure. Initially, she was treated by a general practitioner with antibiotics, but there was no improvement. At presentation to our hospital, her symptoms were persistent, and breathlessness increased to Modified Medical Research Council grade III. On physical examination, she was febrile with temperature of 101°F, pulse rate of 118/minute, BP of 120/70 mmHg, and oxygen saturation was 95% with FiO2 of 32%. Chest radiograph revealed patchy opacities in the bilateral lower lung zones [Figure 1]. Routine blood investigations exhibited leukocytosis with the total leucocyte count of 16,000 cumm−3 and raised erythrocyte sedimentation rate of 105 mm in first hour. Renal and liver functions were normal. Sputum, blood, and urine cultures came out to be sterile. Sputum for acid fast bacilli (AFB) was negative and sputum for GeneXpert did not show presence of Mycobacterium tuberculosis. Contrast enhanced computed tomography chest was done, which showed patchy airspace opacities diffusely scattered but more prominent in periphery and lower lobes in both the lungs, suggestive of OP [Figure 2]. Ziehl-Neelsen (ZN) stain of bronchoalveolar lavage fluid was negative for AFB. Fungal and pyogenic culture was sterile, and cytology was neutrophilic predominant. Transbronchial lung biopsy for histopathology revealed organizing fibrinous material, consistent with fibrin within the alveolar spaces, mild interstitial fibrosis, which was suggestive of subacute fibrinous OP. Patient was further investigated and immunoglobulin M-rheumatoid factor (RF), antinuclear antibody, and CA-125 were within normal limits. Considering the diagnosis of OP, the patient was started on steroids, following which the patient improved clinicoradiologically and was on tapering doses of steroids. After 3 months of follow-up, when the patient was on 10 mg of prednisolone daily, she restarted having symptoms in the form of fever, cough, and shortness of breath along with small joint pain. Repeat investigations showed elevated RF of 120 IU/ml (normal <14 IU/ml) and anticyclic citrullinated peptide antibody (anti-CCP Ab) of greater than 35.10 U/ml (normal <5 U/ml). A diagnosis of RA-associated OP was made, and the patient was started on disease modifying antirheumatic drug (DMARD) (hydroxychloroquine plus leflunomide) along with steroids. Patient started improving symptomatically and radiologically and is on regular follow-up [Figure 3].{Figure 1}{Figure 2}{Figure 3}


OP is a type of interstitial pneumonia with peculiar histopathological features consisting of organization of inflammatory exudate resulting in intra-alveolar fibrosis.[3],[4] OP is generally classified into three types: consisting of OP of determined cause, OP of undetermined cause but occurring in a defined setting, and cryptogenic OP.[5] It can occur in connection with various conditions, such as infections, adverse drug reaction, autoimmune inflammatory diseases, radiotherapy, aspiration, and organ or bone marrow transplantation.[6] In our patient, no significant past medical and treatment history was there. Clinical presentation is variable and nonspecific consisting of symptoms such as fever, weight loss, cough, shortness of breath, and chest pain.[3],[7] Our patient was having similar symptoms on presentation. Bronchoalveolar lavage, blood, and urine cultures were negative. Pulmonary manifestation of RA mainly includes ILD, airway complications, pleural involvement, chest wall abnormalities, and vascular complications.[1] Symptoms of connective tissue disorders including RA in most of the cases precede lung involvement.[1],[7] Cases of RA preceded by OP are not very frequent. [Table 1] shows the review of cases where OP was the preliminary presentation of RA. Time interval from presentation of OP to development of articular symptoms of RA varied from few days to years. In our patient no articular symptoms were present at the time of presentation. In most of the cases, serology was positive at presentation. However, in our case, serology was negative at presentation, and steroids were started and clinicoradiological improvement was seen, but the patient deteriorated on tapering doses of steroid and started having joint pain. Repeat RF and anti-CCP were positive. DMARDs were started, after which steroids were gradually tapered. Patient was not started on methotrexate as it is known to cause lung fibrosis resulting in exacerbation of ILD and known to confer an increased risk of pneumonitis.[8] RF and anti-CCP have high sensitivity and specificity for diagnosis of RA. Also, these antibodies can be detected very early in the disease.[9] All the patients of OP should be investigated for RA as the serology is positive even in the absence of articular symptoms. A repeat RF and anti-CCP may be considered in patients not showing improvement. This will help in avoiding the delay in initiation of DMARDs in these patients. [10-16] To conclude, in a patient with OP, RA should be considered as a possible diagnosis even if there are no articular symptoms present initially.{Table 1}

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


1Amital A, Shitrit D, Adir Y. The lung in rheumatoid arthritis. Presse Med 2011;40:e31-48.
2Kim EJ, Collard HR, King TE. Rheumatoid arthritis-associated interstitial lung disease: the relevance of histopathologic and radiographic pattern. Chest 2009;136:1397-405.
3Cordier J-F. Cryptogenic organizing pneumonia. Clin Chest Med 2004;25:727-38.
4Hoshino C, Satoh N, Narita M, Kikuchi A, Inoue M. Organising pneumonia as the first manifestation of rheumatoid arthritis. BMJ Case Rep 2011;2011:bcr1120103558.
5Cordier J-F. Organising pneumonia. Thorax 2000;55:318-28.
6Schlesinger C, Koss MN. The organizing pneumonias: an update and review. Curr Opin Pulm Med 2005;11:422-30.
7Antoniou KM, Margaritopoulos G, Economidou F, Siafakas NM. Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement. Eur Respir J 2009;33:882-96.
8Iqbal K, Kelly C. Treatment of rheumatoid arthritis-associated interstitial lung disease: a perspective review. Ther Adv Musculoskelet Dis 2015;7:247-67.
9Matsui T, Shimada K, Ozawa N, Hayakawa H, Hagiwara F, Nakayama H et al. Diagnostic utility of anti-cyclic citrullinated peptide antibodies for very early rheumatoid arthritis. J Rheumatol 2006;33:2390-7.
10Ippolito JA, Palmer L, Spector S, Kane PB, Gorevic PD. Bronchiolitis obliterans organizing pneumonia and rheumatoid arthritis. Semin Arthritis Rheum 1993;23:70-8.
11Cavallasca JA, Caubet M, Helling CA, Tate GA. Cryptogenic organizing pneumonia (COP), as presentation of rheumatoid arthritis. Rheumatol Int 2008;29:99-101.
12Henriet 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.
13Komiya 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.
14Kinoshita Y, Sakamoto A, Hidaka K. Organizing. pneumonia preceding rheumatoid arthritis. Case Rep Pulmonol 2014;2014:758619
15Mori 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.
16Kalinova D, Kolarov Z, Rshkov R. Organising pneumonia − the first manifestation of rheumatoid arthritis. Reumatologia 2017;55:314-7.