Abstract

Diagnosing Behcet’s disease (BD) in a patient already diagnosed with Castelman’s disease (CD) is rare. There are only a few cases reported in the literature and all of them were diagnosed as BD prior to the patient experiencing symptoms and signs of CD. We present a patient who was initially diagnosed as having CD. However, after being managed with chemotherapy, specifically after the fourth cycle, the patient was found to have an incidental finding of pulmonary artery aneurysm, which led to the diagnosis of BD. For that, he received the appropriate management of high-dose steroid, azathioprine, and oral anticoagulant. Currently, the patient is doing well, and the latest computed tomography scan showed complete resolution of his pulmonary aneurysm. We suggest taking a thorough history from all patients with BD symptoms and signs, especially in CD patients as they may overlap, for early diagnosis and to prevent complications.

Highlights

  • The presence of Behçet’s disease (BD) with Castleman’s disease (CD) is quite rare, as only a few cases have been reported worldwide, all of which were diagnosed as Behcet’s disease (BD) prior to the patient experiencing manifestations of Castelman’s disease (CD)

  • After being managed with chemotherapy, after the fourth cycle, the patient was found to have an incidental finding of pulmonary artery aneurysm, which led to the diagnosis of BD

  • We suggest taking a thorough history from all patients with BD symptoms and signs, especially in CD patients as they may overlap, for early diagnosis and to prevent complications

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Summary

Introduction

The presence of Behçet’s disease (BD) with Castleman’s disease (CD) is quite rare, as only a few cases have been reported worldwide, all of which were diagnosed as BD prior to the patient experiencing manifestations of CD. Laboratory investigations showed a normal cell count and that his inflammatory markers were not elevated (C-reactive protein and erythrocyte sedimentation rate) His autoimmune profile was negative (antinuclear antibodies, anti-DNA, anti-Sjögren’ s-syndrome-related antigen A, anti-cyclic citrullinated peptides, anticardiolipin antibodies, lupus anticoagulant), and his human leukocyte antigen-B51 was negative. The patient was diagnosed as having BD by fulfilling the International Criteria for Behcet’s disease (ICBD) and the International Study Group (ISG) with a pulmonary aneurysm He was hospitalized and started on hydrocortisone 100 mg intravenously every six hours, azathioprine 50 mg orally daily, and enoxaparin 80 mg twice daily subcutaneously based on his weight. A repeated chest CT scan (done five months after the previous scan) showed marked improvement of his pulmonary aneurysm (Figure 2) He is following up with the rheumatology outpatient department and doing well, with no recurrence of ulcers and no signs of active disease. He is being maintained on prednisone 2.5 mg orally daily, azathioprine 150 mg orally daily, colchicine 500 mcg orally daily, and rivaroxaban 20 mg orally daily

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Davatchi F
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