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Rheumatoid Arthritis Overlap Syndrome: a Case Report Ashraf E 56 Case report Granulomatosis with polyangiitis: Rheumatoid arthritis overlap syndrome: A case report Ashraf E. Sileem, Ahmed M. Said Antineutrophil cytoplasm antibody (ANCA)-associated adalimumab therapy for rheumatoid arthritis. This clinical vasculitis (AAV) may rarely be associated with other observation must be considered in all patients treated with immune-mediated diseases. Knowledge of these overlap antitumor necrosis factor. On the basis of the previously syndromes is important in early recognition of potential published cases of AAV associated with rheumatoid complications and differences in clinical courses and arthritis as well as our case, the suggestion of a rare form management pathways. In this paper we describe the case of an AAV autoimmune overlap should be recognized and of a male Egyptian patient, 52 years old, with hypertension investigated for rapid initiation of appropriate management. and rheumatoid arthritis on disease-modifying antirheumatic Egypt J Bronchol 2017 11:56–61 drugs, with a recent history of ocular and renal manifestations © 2017 Egyptian Journal of Bronchology suggesting vasculitis, who presented to our Emergency Egyptian Journal of Bronchology 2017 11:56–61 Department with acute interstitial pneumonitis, which was successfully treated with high-dose steroids. His clinical Keywords: adalimumab, overlap syndrome, rheumatoid arthritis, Wegener’s course had deteriorated because of self-stoppage of the granulomatosis maintenance dose of steroid and appearance of bilateral Chest Department, Zagazig University, Zagazig, Egypt nodular infiltrates in the lungs on the chest radiographs. Correspondence to Ashraf E. Sileem, MD, Department of Chest, Zagazig His cytoplasmic c-ANCA autoantibodies were positive, in University Hospitals, Al-Salam Sector, Cardiothoracic Building, Zagazig, addition to the histopathological examination of open lung Sharkia, Egypt; Tel: +201273258514; biopsy, which was consistent with granulomatosis with e-mail: [email protected] polyangiitis. He was successfully treated with high-dose Received 3 September 2015 Accepted 29 September 2015 steroids and cyclophosphamide. We reported a new case of granulomatosis with polyangiitis developing during Introduction Case presentation Wegener’s granulomatosis (WG) is a multisystem We present the case of a male Egyptian patient, aged disease characterized by a necrotizing granulomatous 52 years, married and with two children, who was a vasculitis affecting predominantly the lower and upper known hypertensive diagnosed 6 years ago and who respiratory tract, lungs, and kidneys. The prevalence of was on oral candesartan 8 mg once daily. He was the disease is about three persons per 100 000 people, diagnosed as a case of RA 5 years ago on the basis equally in both sexes [1]. of seropositive RA [rheumatoid facto (90.7 IU/ml) and anticitrullinated protein antibody (58 IU/ml)], swelling The German pathologist Friedrich Wegener first and tenderness of his small joints in both hands for described the disease in 1936. In 1954, Godman and more than 2 months (the metacarpophalangeal and Churg more fully delineated the disease and established proximal interphalangeal joints) as shown in Fig. 1, and the three main clinical criteria of WG (vasculitis, abnormal acute-phase reactants [C-reactive protein glomerulonephritis, respiratory tract involvement). (CRP) and erythrocyte sedimentation rate (ESR)] Clinical manifestations and organ involvement of the during the diagnosis. His medication list included disease vary widely [2]. methylprednisolone 5 mg, methotrexate, and disease- modifying antirheumatic drugs (leflunomide 20 mg, Clinically, antineutrophil cytoplasm antibody (ANCA)- adalimumab injection 40 mg every other week), associated vasculitis (AAV) may rarely be associated omega 3, folic acid, and esomeprazole 20 mg. with other immune-mediated diseases. Those that have been well recognized include antiglomerular basement On 21 May 2014, the patient presented to the membrane disease [3,4], scleroderma [5], systemic lupus ophthalmologist with defective vision in the right erythematosus [6], and membranous glomerulonephritis eye with appearance of red eye. The ophthalmology [7,8]. Knowledge of these overlap syndromes is important report revealed nodular scleritis, anterior uveitis, and in early recognition of potential complications and papilledema. Fluorescein angiography and optical differences in clinical courses and management pathways. Draibe and Salama [9] noticed the development of This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which AAV in a group of patients with rheumatoid arthritis allows others to remix, tweak, and build upon the work (RA), where there was a delay in presentation with noncommercially, as long as the author is credited and the new vasculitic symptoms following the diagnosis of RA. creations are licensed under the identical terms. © 2017 Egyptian Journal of Bronchology | Published by Wolters Kluwer - Medknow DOI: 10.4103/1687-8426.198995 Granulomatosis with polyangiitis Sileem and Said 57 coherence tomography revealed leakage at the macula min, and O2 saturation was 87%. On local physical and optic nerve head, as shown in Fig. 2. examination, there was diminished vesicular breath- sound intensity and bilateral fine late inspiratory Methylprednisolone 100 mg/day, topical steroid, and crackles. Chest radiography revealed bilateral micro- cycloplegic mydriatics were prescribed. The steroid was nodular infiltrates with predominance of left upper and tapered to 20 mg within 2 weeks with reasonable middle lung zones. response as regards eye redness and vision improvement. He was admitted to the ICU, with a provisional After 2 weeks, he relapsed with defective vision, with diagnosis of atypical pneumonia, with referral to a optical coherence tomography evidence of cystoid chest physician. macular edema, and attack of hematuria and decline in renal function (creatinine was 3.2 mg/dl with active His arterial blood gases, on 5 l/min humidified oxygen urinary sediment). He was injected with intravitreal face mask, were as follows: pH 7.5, PaCO2 22.9, PaO2 − triamcinolone acetonide with recommendation to 56.4, HCO3 21.6, BE 3.3, O2 saturation 89%. d- continue systemic steroid at 20 mg/day for 1 month. dimer was negative, CRP was high (270 mg/l), He showed improvement as regards his ocular and complete blood count was normal, blood urea was urinary complaints as well as renal function, and urine 68 mg/dl, serum creatinine was 3.09 mg/dl, and uric analysis revealed normal levels. acid was 9.1 mg/dl, with normal serum electrolytes. On 26 July 2014, he presented at the ER with fever, dry High-resolution chest CT without contrast revealed cough, shortness of breath, and respiratory distress. His evidence of interstitial thickening involving scattered blood pressure was 140/100, pulse was 120 beats/min, areas of both lung fields with preserved lung volumes; temperature was 38°C, respiratory rate was 32 breaths/ upper and mid zones were prominently involved; the interstitial prominence was more centrally located; Figure 1 there were mild reticular opacities in both apices and patchy areas of ground-glass haziness in the perihilar region and upper lung zones bilaterally. Bilateral pleural reactions were also noted, as shown in Fig. 3. Figure 3 Boggy swelling in proximal interphalangeal and metacarpophalan- geal joints. Figure 2 Optical coherence tomography (OCT) revealed leakage at the mac- ula and optic nerve head. High-resolution chest computed tomography (CT) without contrast. 58 Egyptian Journal of Bronchology, Vol. 11 No. 1, January-March 2017 He was diagnosed as a case of acute interstitial pleural effusion, and no pulmonary fibrosis or pneumonitis with respiratory failure type I in case of nodularity, as shown in Fig. 4. RA. Methotrexate was held and treatment started in the form of oxygen therapy, methylprednisolone The patient was advised to continue on 30 mg/ 250 mg intravenously twice daily, clarithromycin day and oral N-acetylcysteine 600 mg twice daily 500 mg oral twice daily, and N-acetylcysteine 600 mg for 1 month. Thereafter, the steroid was tapered twice daily. and the patient was advised to consult a rheuma- tologist. The steroid was tapered to 10 mg/day Previous medications continued for 5 days, with daily and stopped abruptly by the patient after 2 improvement of patient’s general condition and months. laboratory investigations; especially serum creatinine and CRP returned to normal values. The patient was On 10 December 2014, the patient presented at discharged on 60 mg prednisolone and pantoprazole the chest clinic complaining of dry infrequent cough, 40 mg, clarithromycin 500 mg tablet twice daily for 7 low-grade fever, nasal dryness, loss of appetite, and days, with weekly follow-up. fatigue; his clinical examination was unremarkable apart from flushed face. He had sinus tachycardia The patient was asymptomatic during the weekly visits (114 beats/min), O2 saturation 95%, and respiratory for 1 month, and the steroid dose was gradually tapered rate 18 breaths/min. the next month to 40 mg daily. Chest rsdiography revealed bilateral pulmonary High-resolution chest CT without contrast after nodules; they were multiple on the left upper and 6 weeks revealed significant reduction in the mid lung zone, and solitary on the right lower lung interstitial thickening and ground-glass haziness, no zone, as shown in Fig. 5. Figure 4 High-resolution
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