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Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling
Accepted Manuscript Title: Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling Author: Subir Bhatia, Loren P. Herrera Hernandez, Amrit K. Kamboj, Katie M. Rieck PII: DOI: Reference:
S0002-9343(17)30941-5 https://doi.org/doi:10.1016/j.amjmed.2017.08.040 AJM 14287
To appear in:
The American Journal of Medicine
Please cite this article as: Subir Bhatia, Loren P. Herrera Hernandez, Amrit K. Kamboj, Katie M. Rieck, Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling, The American Journal of Medicine (2017), https://doi.org/doi:10.1016/j.amjmed.2017.08.040. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling Subir Bhatia MD1, Loren P. Herrera Hernandez MD2, Amrit K. Kamboj, MD1, Katie M. Rieck MD, MHA3 1
Department of Internal Medicine, Mayo Clinic, Rochester, MN Department of Pathology, Mayo Clinic, Rochester, MN 3 Division of Hospital Internal Medicine, Mayo Clinic, Rochester, MN 2
Article Type: Clinical Communications to the Editor Conflicts of Interests: None Author involvement with manuscript: SB, LHH, AK- Concept, literature review, and drafting of manuscript; KR- drafting and critical review of manuscript Corresponding author: Katie Rieck, MD Mayo Clinic 200 First Street SW Rochester, Minnesota 55905 [email protected] Manuscript word count including references: 606 words Figures: 1 Funding source: None
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To the Editor: A previously healthy 52-year-old man presented for further evaluation of eight months of progressively worsening scrotal swelling and testicular pain. Physical examination showed normal vital signs and a severely edematous, erythematous, and tender scrotum. Initial labs revealed leukocytosis (21.8 * 10^9/L) and elevated Creactive protein (CRP) (167 mg/L). Scrotal ultrasound was concerning for Fournier’s gangrene of the scrotum with left testicular ischemia. He then underwent an emergent left orchiectomy with debridement and was placed on broad-spectrum antibiotics. Despite this, his scrotal swelling continued and repeat scrotal ultrasound showed an ischemic right testicle; he then underwent right orchiectomy. Testing for myeloperoxidase antibody, proteinase 3 (PR3), anti-double stranded DNA, complement 3, complement 4, and hepatitis B and C serologies were unremarkable. Pathology of the both testis and scrotum revealed acute and chronic necrotizing vasculitis with morphologic features consistent with polyarteritis nodosa, confirming the diagnosis (Figure 1). To assess for further involvement of polyarteritis nodosa, CT angiogram of the abdomen and pelvis was performed but was without evidence of more extensive vasculitis. He was initiated on high dose steroids and cyclophosphamide. He required multiple debridements due to significantly worsening and advancing penile, bladder, and scrotal necrosis. After repeated inspection and debridement revealed stable disease activity and receipt of a partial thickness skin graft, he was discharged with prednisone and cyclophosphamide, two months after his initial presentation. On follow-up at four months, he showed no signs of disease recurrence and the surgical site was completely
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healed. He successfully completed 6 months treatment of cyclophosphamide and prednisone and was then transitioned to oral methotrexate and continued a prednisone taper. He continued to do well at 8 months follow-up.
Discussion Polyarteritis nodosa is a type of systemic necrotizing vasculitis that affects medium-sized arteries, with occasional involvement of the small arteries. It can affect any organ in the body with the most commonly affected organs being the kidney, heart, liver, and gastrointestinal tract. While testicular involvement in polyarteritis nodosa is recognized in nearly all cases at autopsy, only a minority of patients with polyarteritis nodosa have clinically significant testicular involvement1,2. To our knowledge, our case highlights the only known case of polyarteritis nodosa to present with isolated testicular involvement resulting in extensive bilateral necrosis. Our case was also unique in that the patient did not develop any other systemic manifestations and the disease activity was highly aggressive, resulting in extensive necrosis of the penis, bladder, and scrotum, and required not only surgical treatment but systemic glucocorticoid and cytotoxic agents as well. While patients with moderate to severe polyarteritis nodosa typically require glucocorticoids and cyclophosphamide, there is no consensus on the treatment of isolated disease3. Current literature suggests treatment beyond surgical excision is usually not necessary in those cases4. Though he required both surgical debridement and treatment with systemic glucocorticoids and cyclophosphamide, our patient eventually responded appropriately and healed well.
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References: 1. Persellin ST, Menke DM. Isolated polyarteritis nodosa of the male reproductive system. The Journal of rheumatology 1992;19:985-988. 2. Warfield AT, Lee SJ, Phillips SM, Pall AA. Isolated testicular vasculitis mimicking a testicular neoplasm. Journal of clinical pathology 1994;47:1121-1123. 3. Bourgarit A, Le Toumelin P, Pagnoux C, Cohen P, Mahr A, Le Guern V, Mouthon L, Guillevin L. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine 2005;84:323-330. 4. Hernandez-Rodriguez J, Hoffman GS. Updating single-organ vasculitis. Current opinion in rheumatology 2012;24:38-45.
Figure 1: A. Paratesticular blood vessels with necrotizing arteritis and partial thrombosis. B. Partially thrombosed blood vessel with recanalization. C. Seminiferous tubules within area of infarction.
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S0002-9343(17)30941-5 https://doi.org/doi:10.1016/j.amjmed.2017.08.040 AJM 14287
To appear in:
The American Journal of Medicine
Please cite this article as: Subir Bhatia, Loren P. Herrera Hernandez, Amrit K. Kamboj, Katie M. Rieck, Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling, The American Journal of Medicine (2017), https://doi.org/doi:10.1016/j.amjmed.2017.08.040. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Isolated Polyarteritis Nodosa Presenting as Bilateral Testicular Swelling Subir Bhatia MD1, Loren P. Herrera Hernandez MD2, Amrit K. Kamboj, MD1, Katie M. Rieck MD, MHA3 1
Department of Internal Medicine, Mayo Clinic, Rochester, MN Department of Pathology, Mayo Clinic, Rochester, MN 3 Division of Hospital Internal Medicine, Mayo Clinic, Rochester, MN 2
Article Type: Clinical Communications to the Editor Conflicts of Interests: None Author involvement with manuscript: SB, LHH, AK- Concept, literature review, and drafting of manuscript; KR- drafting and critical review of manuscript Corresponding author: Katie Rieck, MD Mayo Clinic 200 First Street SW Rochester, Minnesota 55905 [email protected] Manuscript word count including references: 606 words Figures: 1 Funding source: None
Page 1 of 6
To the Editor: A previously healthy 52-year-old man presented for further evaluation of eight months of progressively worsening scrotal swelling and testicular pain. Physical examination showed normal vital signs and a severely edematous, erythematous, and tender scrotum. Initial labs revealed leukocytosis (21.8 * 10^9/L) and elevated Creactive protein (CRP) (167 mg/L). Scrotal ultrasound was concerning for Fournier’s gangrene of the scrotum with left testicular ischemia. He then underwent an emergent left orchiectomy with debridement and was placed on broad-spectrum antibiotics. Despite this, his scrotal swelling continued and repeat scrotal ultrasound showed an ischemic right testicle; he then underwent right orchiectomy. Testing for myeloperoxidase antibody, proteinase 3 (PR3), anti-double stranded DNA, complement 3, complement 4, and hepatitis B and C serologies were unremarkable. Pathology of the both testis and scrotum revealed acute and chronic necrotizing vasculitis with morphologic features consistent with polyarteritis nodosa, confirming the diagnosis (Figure 1). To assess for further involvement of polyarteritis nodosa, CT angiogram of the abdomen and pelvis was performed but was without evidence of more extensive vasculitis. He was initiated on high dose steroids and cyclophosphamide. He required multiple debridements due to significantly worsening and advancing penile, bladder, and scrotal necrosis. After repeated inspection and debridement revealed stable disease activity and receipt of a partial thickness skin graft, he was discharged with prednisone and cyclophosphamide, two months after his initial presentation. On follow-up at four months, he showed no signs of disease recurrence and the surgical site was completely
Page 2 of 6
healed. He successfully completed 6 months treatment of cyclophosphamide and prednisone and was then transitioned to oral methotrexate and continued a prednisone taper. He continued to do well at 8 months follow-up.
Discussion Polyarteritis nodosa is a type of systemic necrotizing vasculitis that affects medium-sized arteries, with occasional involvement of the small arteries. It can affect any organ in the body with the most commonly affected organs being the kidney, heart, liver, and gastrointestinal tract. While testicular involvement in polyarteritis nodosa is recognized in nearly all cases at autopsy, only a minority of patients with polyarteritis nodosa have clinically significant testicular involvement1,2. To our knowledge, our case highlights the only known case of polyarteritis nodosa to present with isolated testicular involvement resulting in extensive bilateral necrosis. Our case was also unique in that the patient did not develop any other systemic manifestations and the disease activity was highly aggressive, resulting in extensive necrosis of the penis, bladder, and scrotum, and required not only surgical treatment but systemic glucocorticoid and cytotoxic agents as well. While patients with moderate to severe polyarteritis nodosa typically require glucocorticoids and cyclophosphamide, there is no consensus on the treatment of isolated disease3. Current literature suggests treatment beyond surgical excision is usually not necessary in those cases4. Though he required both surgical debridement and treatment with systemic glucocorticoids and cyclophosphamide, our patient eventually responded appropriately and healed well.
Page 3 of 6
References: 1. Persellin ST, Menke DM. Isolated polyarteritis nodosa of the male reproductive system. The Journal of rheumatology 1992;19:985-988. 2. Warfield AT, Lee SJ, Phillips SM, Pall AA. Isolated testicular vasculitis mimicking a testicular neoplasm. Journal of clinical pathology 1994;47:1121-1123. 3. Bourgarit A, Le Toumelin P, Pagnoux C, Cohen P, Mahr A, Le Guern V, Mouthon L, Guillevin L. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine 2005;84:323-330. 4. Hernandez-Rodriguez J, Hoffman GS. Updating single-organ vasculitis. Current opinion in rheumatology 2012;24:38-45.
Figure 1: A. Paratesticular blood vessels with necrotizing arteritis and partial thrombosis. B. Partially thrombosed blood vessel with recanalization. C. Seminiferous tubules within area of infarction.
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