A Case of Sinobronchial Syndrome Complicated with Myeloperoxidase Antineutrophil Cytoplasmic Antibody Associated Vasculitis: Review of the Literature
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1 CASE REPORT A Case of Sinobronchial Syndrome Complicated with Myeloperoxidase Antineutrophil Cytoplasmic Antibody Associated Vasculitis: Review of the Literature Toru Kadowaki 1, Shuichi Yano 1, Ichiro Yamadori 2, Kunio Araki 3, Masahiro Kimura 1, Kiryo Wakabayashi 1, Kanako Kobayashi 1, Shigenori Ishikawa 1 and Toshikazu Ikeda 1 Abstract We report a case of long-standing sinobronchial syndrome complicated by microscopic polyangiitis (MPA) during the clinical course. The patient showed a mild elevation of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) 17 months prior to the diagnosis of MPA. Subsequently, her MPO-ANCA level gradually became more elevated, and finally her MPO-ANCA level peaked when purpura appeared. Histologic examination of the skin biopsy was consistent with leukocytoclastic vasculitis. Based on the pathological and clinical findings, a diagnosis of MPA was made. Corticosteroid therapy finally led to a remission of MPA with normalized MPO-ANCA titers. Key words: sinobronchial syndrome (SBS), myeloperoxidase antineutrophil cytoplasmic antibody (MPO- ANCA), chronic airway diseases (CAD), microscopic polyangiitis (MPA) (Intern Med 51: , 2012) () Introduction Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) is used as a serologic marker for the most common type of crescentic glomerulonephritis and for microscopic polyangiitis (MPA) (1). An association between chronic airway diseases (CAD) such as bronchiectasis (), diffuse panbronchiolitis (DPB) and sinobronchial syndrome (SBS), and vasculitis has been reported. To our knowledge, there have been 22 reported cases of CAD complicating systemic vasculitis (2-14). In these reported cases, CAD preceded the onset of vasculitis. We report herein a case of SBS complicated by MPA during the clinical course. We were able to observe the development of MPA with the elevation of MPO-ANCA level in a patient with SBS. In addition, we reviewed the reported cases of systemic vasculitis complicated by CAD. Case Report A 70-year-old woman was admitted to our hospital in mid-january 2009 for the treatment of acute exacerbation of. was diagnosed when the patient was in her fifties, and she had received follow-up care at our hospital. Recently, SBS was diagnosed because was complicated by chronic sinusitis, but there was no evidence of immunologic abnormality. Chest X-ray and computed tomography of the chest showed cylindrical and cystic in bilateral lungs, predominantly in the left lower lobe (ig. 1). Six months prior to admission, MPO-ANCA was elevated to 24 EU (normal <20 EU) without elevation of proteinase-3 antineutrophil cytoplasmic antibody. Based on the elevated MPO- ANCA level, vasculitic syndrome was suspected. However, the clinical symptoms and results of physical examination and laboratory tests did not meet the criteria for a diagnosis Department of Pulmonary Medicine, National Hospital Organization Matsue Medical Center, Japan, Department of Pathology, National Hospital Organization Okayama Medical Center, Japan and Department of Pulmonary Surgery, National Hospital Organization Matsue Medical Center, Japan Received for publication June 5, 2011; Accepted for publication December 12, 2011 Correspondence to Dr. Toru Kadowaki, toruyan@matsue.hosp.go.jp 763
2 igure1.high-resolution computed tomography scans of the chest obtained upon admission showed cylindrical, varicose, and cystic bronchiectasis which is dominant in the left lung base. igure2.clinical course. of any vasculitic syndrome at that time. Laboratory tests on admission revealed that the white blood cell count and C- reactive protein (CRP) level were elevated to 8,900/μL and 9.03 mg/dl, respectively. Sputum culture revealed the presence of Pseudomonas aeruginosa. Antibiotic therapy was effective, and the infection was resolved. However, the patient s MPO-ANCA level was markedly elevated to 109 EU without any clinical symptoms of vasculitic syndrome. Three months after admission, in mid-april 2009, she coughed up massive blood of 200 ml, and was emergently admitted to our hospital. Hemostatics and antibiotics effectively resolved her symptom. MPO-ANCA level at that time was still high (101 EU). Renal biopsy was done to diagnose her disease, and the findings were consistent with IgA nephropathy. Serum creatinine level was 0.80 mg/dl, and the urine gave a ++ test for blood and a + test for protein. Aspergillus antigen was positive so antifungal therapy (voriconazole) was started after this episode. After a year of the administration of voriconazole, aspergillus antigen turned to be negative. Consequently, she was followed as a patient with MPO-ANCA positive SBS. Purpura suddenly appeared on her lower limbs three months after the second admission and was associated with a further elevation of MPO-ANCA level (146 EU) (ig. 2). At that time proteinuria and hematuria also developed. The onset of ANCA-associated vasculitis was highly suspected, therefore a skin biopsy of the lower limb has been performed. Histology of the skin biopsy showed an infiltration of neutrophils and lymphocytes around the small arterioles and capillaries in the dermis and fragmentation of nuclei of these inflammatory cells, leading to the diagnosis of leukocytoclastic vasculitis (ig. 3). According to pathological and clinical findings, a diagnosis of MPA was made. Prednisolone (1 mg/kg) was started and tapered down as the purpura 764
3 igure3.histology of the skin showing leukocytoclastic vasculitis. Arterioles and capillaries in the dermis are surrounded by acute inflammatory cells with karyorrhexis (Hematoxylin and Eosin staining, 200). and MPO-ANCA levels were gradually resolved. After the administration of corticosteroids, her hematuria and proteinuria were also ameliorated. Moreover, her occasional hemoptysis was resolved and the MPO-ANCA level normalized after 8 months of administration of prednisolone (ig. 2). Discussion The possibility of the development of vasculitis should be considered during the management of patients with a longstanding CAD. The present case was clinically significant in that we could observe the development of MPA with the elevation of MPO-ANCA in a patient with SBS. Mild elevation of MPO-ANCA has been reported in patients with chronic bronchial suppuration (12, 15, 16). In one such study, 4 of 30 patients with DPB were positive for MPO- ANCA (12). Three of the four patients with DPB but without vasculitis showed a low titer of MPO-ANCA (10-60 EU) (12). The other DPB patient who had vasculitis showed a high titer of MPO-ANCA (442 EU). Indeed, the present case had shown a mild elevation of MPO-ANCA (24 EU) 17 months prior to the diagnosis of MPA. Then, the MPO- ANCA level became gradually elevated, and finally the MPO-ANCA level peaked (146 EU) simultaneously with the appearance of purpura. To date, 22 cases of vasculitis with preceding CAD have been reported (Table 1) (2-14), however, all of the cases were found to be elevated ANCA on the onset of vasculitis. Thus, long-standing CAD followed by onset of ANCAassociated vasculitis strongly supports the ANCA-cytokine sequence hypothesis (6). CAD could cause the neutrophil destruction, which is followed by ANCA elevation (3). Moreover, ANCA leads to activation of neutrophils and degranulation by inducing inflammatory cytokines and this finally results in vascular damage. To date, there have been 22 reported cases of vasculitis following a long-standing history of CAD: 18 as, 3 as DPB, and 1 as SBS (Table 1) (2-14). In 15 patients for whom ANCA was measured, 11 patients showed positive p-anca or MPO-ANCA whereas only 2 patients showed positive c-anca. One possible explanation for these findings is the causative pathogens of chronic airway suppuration. Bacterial infections also have been associated with the initiation and relapse of Wegener s granulomatosis (WG). Among these bacteria, Staphylococcus aureus seems to have the strongest association with WG (17). On the other hand, according to the review of the 22 reported cases, Gram-negative bacilli such as Haemophilus influenzae and Pseudomonas aeruginosa were the common causative pathogens (Table 1). Kain et al recently found that 93% of patients with active ANCA associated necrotizing crescentic glomerulonephritis, which often shows positive MPO-ANCA, had autoantibodies to human lysosomal membrane protein 2 (LAMP-2), and a major epitope of LAMP-2 is homologous to the bacterial adhesion protein imh, which is found in Gram-negative bacteria. Actually, rats immunized with imh developed pauciimmune necrotizing crescentic glomerulonephritis in conjunction with antibodies to rat LAMP-2 and human LAMP- 2 (17). In patients with preceding CAD followed by ANCA associated vasculitis, Gram-negative bacilli are the most common causative pathogens. Consequently, long-standing airway infection might associate with the onset of p-anca (MPO-ANCA) associated vasculitis. Recently, Takahashi et al reported a radiologic review of 26 patients with MPA. This review revealed the presence of in 9 patients (35%), 4 of whom had long-standing histories (from 13 to 31 years) prior to the onset of MPA (13). Accordingly, in patients with a long-standing history of CAD or bronchiectatic changes in chest radiographic examination, careful attention should be paid for the development of vasculitis in future. Balancing the efficacy of immunosuppression with its adverse effects is very important for the treatment of vasculitis complicated by CAD. In 86% (12 of 14) of reported cases that were treated for vasculitis, cyclophosphamide (CPA) or azathioprine was used in combination with corticosteroids (CS) (Table 1). Most of these patients improved after the induction of immunosuppressive therapy. In one case, however, CPA was discontinued due to the exacerbation of (8). In the present case, the clinical subtype was considered to be early systemic, and in such a condition, CS in combination with daily oral CPA is reported to be effective (17). Because the patient had taken voriconazole for pulmonary aspergillosis and sputum culture had consistently revealed the presence of Pseudomonas aeruginosa, we initiated therapy with prednisolone (1 mg/kg) but without CPA to avoid extreme immunosuppression. CS monotherapy finally led to a remission of MPA. Reported cases showed good prognosis for treatment of systemic vasculitis with CAD (Table 1). Likewise, Takahashi et al. suggested a better prognosis in patients with MPA with CAD than in those without CAD (13). In that study, only one patient (10%) with CAD died within 90 days of the onset of MPA, while 765
4 Table1.Reported Cases of Vasculitis with Preceding Chronic Airway Diseases Ref. No. Year Age Sex Preceding respiratory disease Duration at the onset of vasculitis ANCA detected Isolated organism from sputum Treatment for vasculitis CS CPA AZP Clinical course after treatment M 18 years P.aeruginosa Improved 57 M 26 years H.influenzae Improved 59 M 38 years H.influenzae Improved years H.influenzae (+) (-) (-) Improved M 10 years C-ANCA Unknown (+) (+) (+) Once improved, but relapsed years P-ANCA (+) (+) (-) Improved M 17 years (-) H.influenzae (+) (+) (-) Improved P.aeruginosa M Since childhood MPO-ANCA (+) (+) (+) Improved 73 M Since childhood C-ANCA (+) (-) (+) Improved years MPO-ANCA (+) (+) (-) Improved years MPO -ANCA Pseudomonas spp. (+) (+) (-) Improved M DPB 11 years P-ANCA S.pneumoniae (+) (+) (-) Died of the disease H.influenzae MPO-ANCA Pseudomonas spp. (+) (-) (+) Improved 63 M MPO-ANCA H.influenzae (+) (+) (-) Improved DPB 18 years MPO-ANCA P.aeruginosa (+) (+) (-) Improved SBS 36 years MPO-ANCA H.influenzae P.aeruginosa (+) (-) (-) Died of severe pneumonia DPB 13 years MPO-ANCA BPI-ANCA M 20 years 31 years 13 years 15 years Stenotrophomonas maltophilia (+) (+) (-) Once improved, but relapsed years BPI-ANCA P.aeruginosa (+) (+) (-) Improved Present case 70 SBS About 40 years MPO-ANCA P.aeruginosa (+) (-) (-) Improved ; bronchiectasis, DPB; diffuse panbronchiolitis, SBS; Sinobronchial syndrome, CS; Corticosteroid, CPA; Cyclophosphamide, AZP; Azathioprine,C-ANCA; Cytoplasmic antineutrophil cytoplasmic antibody, P-ANCA; Perinuclear antineutrophil cytoplasmic antibody, MPO-ANCA; Myeloperoxidase antineutrophil cytoplasmic antibody, BPI-ANCA; Bactericidal/permeability-increasing antineutrophil cytoplasmic antibody, : Not documented Started with intravenous methylprednisolone, then tapered to oral prednisolone, Started with prednisolone, Used after relapse, CPA replaced by AZP after normalization of ANCA, Discontinued due to exacerbation of, AZP replaced cyclosporine after renal transplantation This study showed the high frequency of positive MPO-ANCA in patients with preceding chronic airway disease (87.5%), however, details of each patient are not shown. Immunoglobulin therapy was combined with CS and CPA. 7 patients (43.8%) without CAD died. Avoiding an exacerbation of CAD with immunosuppressive therapy remains a therapeutic challenge in these patients. It is unknown whether CS monotherapy or CS combined with CPA is more efficacious for the treatment of systemic vasculitis with CAD. urther accumulation of cases will be necessary to clarify this point. The authors state that they have no Conflict of Interest (COI). References 1. alk RJ, Jennette JC. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxydase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. N Engl J Med 318: , Hilton AM, Hasleton PS, Bradlow A, Leahy BC, Cooper KM, Moore M. Cutaneous vasculitis and immune complexes in severe bronchiectasis. Thorax 39: , Sitara D, Hoffbrand BI. Chronic bronchial suppuration and antineutrophil cytoplasmic antibody (ANCA) positive systemic vasculitis. Postgrad Med J 66: , Tanaka E, Tada K, Amitani R, Kuze. Systemic hypersensitivity vasculitis associated with bronchiectasis. Chest 102: , McKane WS, Velasco N, arrington K. ANCA-positive crescentic glomerulonephritis in chronic bronchiectasis. Nephrol Dial Transplant 10: , Bruce IN, McAteer JA, Gardiner PV, Mcarland RJ, Sloan JM, Bell AL. Chronic suppurative lung disease with associated vasculitis. Postgrad Med J 71: 24-27, Saku N, Sugiyama Y, Kitamura S, ujii T, Saitoh K. Diffuse panbronchiolitis with P-ANCA-positive arteritis and necrotizing glomerulitis. Nihon Kyobu Shikkan Gakkai Zasshi 34: , 1996 (in Japanese, Abstract in English). 8. Pai P, Rustom R, Bone JM, Bell GM. Clinical bronchiectasis and anti-myeloperoxidase antibody related rapidly progressive necrotizing glomerulonephritis. Clin Nephrology 49: , Miyamoto D, Ichinose Y, Kikawada M, et al. Diffuse panbronchiolitis with myeloperoxidase-specific antineutrophil cytoplasmic antibody-related vasculitis. Nihon Kokyuki Gakkai Zasshi 36: , 1998 (in Japanese, Abstract in English). 766
5 10. Nagata T, Hara K, Oka T, et al. Microscopic polyangiitis associated with sinobronchial syndrome. Intern Med 40: , Park J, Banno S, Sugiura Y, et al. Microscopic polyangiitis associated with diffuse panbronchiolitis. Intern Med 43: , Sugiyama Y, Kitamura S. Antineutrophil cytoplasmic antibodies in diffuse panbronchiolitis. Respiration 66: , Takahashi K, Hayashi S, Ushiyama O, et al. Development of microscopic polyangiitis in patients with chronic airway disease. Lung 183: , Benucci M, Nenci G, Taccetti G, et al. Bronchiectasis worsening by p-anca (bactericidal/permeability-increasing protein) positive vasculitis. A case report and review of the literature. Ann Ital Med Int 20: , 2005 (in Italian, Abstract in English). 15. Matsuyama W, Wakimoto J, Watanabe A, et al. Bronchiectasis with myeloperoxidase antineutrophil cytoplasmic antibody and bactericidal/permeability-increasing protein antineutrophil cytoplasmic antibody. Intern Med 38: , Kadowaki T, Hamada H, Ito R, et al. Myeloperoxidase antineutrophil cytoplasmic antibody-positive bronchiectasis in an elderly patient. Geriatr Gerontol Int 7: , Chen M, Kallenberg CG. ANCA-associated vasculitides--advances in pathogenesis and treatment. Nat Rev Rheumatol 6: , The Japanese Society of Internal Medicine 767
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