Successful Treatment of Spontaneous Splenic Rupture in a Patient with Polyarteritis Nodosa
Firdevs Ulutas*, Ugur Karasu, Serdar Kaymaz, Veli Cobankara
Department of Rheumatology, Internal Medicine, Pamukkale University, Denizli, Turkey
*Corresponding Author: Firdevs Ulutas, Department of Rheumatology, Internal Medicine, Pamukkale University, Denizli, Turkey
Received: 12 December 2019; Accepted: 06 January 2019; Published: 09 January 2019
Article Information
Citation:
Firdevs Ulutas, Ugur Karasu, Serdar Kaymaz, Veli Cobankara. Successful Treatment of Spontaneous Splenic Rupture in a Patient with Polyarteritis Nodosa. Fortune Journal of Rheumatology 2 (2020): 013-015.
View / Download Pdf Share at FacebookAbstract
Polyarteritis nodosa (PAN) is a rare necrotizing vasculitis presenting with multiple organ manifestations. We herein describe a case presented with splenic subcapsular hematoma, after nontraumatic splenic rupture and successfully treated with a combination of pulse steroids and cyclophosphamide therapy.
Keywords
Polyarteritis nodosa, Splenic rupture, Gastrointestinal
Polyarteritis nodosa articles, Splenic rupture articles, Gastrointestinal articles
Polyarteritis nodosa articles Polyarteritis nodosa Research articles Polyarteritis nodosa review articles Polyarteritis nodosa PubMed articles Polyarteritis nodosa PubMed Central articles Polyarteritis nodosa 2023 articles Polyarteritis nodosa 2024 articles Polyarteritis nodosa Scopus articles Polyarteritis nodosa impact factor journals Polyarteritis nodosa Scopus journals Polyarteritis nodosa PubMed journals Polyarteritis nodosa medical journals Polyarteritis nodosa free journals Polyarteritis nodosa best journals Polyarteritis nodosa top journals Polyarteritis nodosa free medical journals Polyarteritis nodosa famous journals Polyarteritis nodosa Google Scholar indexed journals Splenic rupture articles Splenic rupture Research articles Splenic rupture review articles Splenic rupture PubMed articles Splenic rupture PubMed Central articles Splenic rupture 2023 articles Splenic rupture 2024 articles Splenic rupture Scopus articles Splenic rupture impact factor journals Splenic rupture Scopus journals Splenic rupture PubMed journals Splenic rupture medical journals Splenic rupture free journals Splenic rupture best journals Splenic rupture top journals Splenic rupture free medical journals Splenic rupture famous journals Splenic rupture Google Scholar indexed journals Gastrointestinal articles Gastrointestinal Research articles Gastrointestinal review articles Gastrointestinal PubMed articles Gastrointestinal PubMed Central articles Gastrointestinal 2023 articles Gastrointestinal 2024 articles Gastrointestinal Scopus articles Gastrointestinal impact factor journals Gastrointestinal Scopus journals Gastrointestinal PubMed journals Gastrointestinal medical journals Gastrointestinal free journals Gastrointestinal best journals Gastrointestinal top journals Gastrointestinal free medical journals Gastrointestinal famous journals Gastrointestinal Google Scholar indexed journals splenic subcapsular hematoma articles splenic subcapsular hematoma Research articles splenic subcapsular hematoma review articles splenic subcapsular hematoma PubMed articles splenic subcapsular hematoma PubMed Central articles splenic subcapsular hematoma 2023 articles splenic subcapsular hematoma 2024 articles splenic subcapsular hematoma Scopus articles splenic subcapsular hematoma impact factor journals splenic subcapsular hematoma Scopus journals splenic subcapsular hematoma PubMed journals splenic subcapsular hematoma medical journals splenic subcapsular hematoma free journals splenic subcapsular hematoma best journals splenic subcapsular hematoma top journals splenic subcapsular hematoma free medical journals splenic subcapsular hematoma famous journals splenic subcapsular hematoma Google Scholar indexed journals cyclophosphamide therapy articles cyclophosphamide therapy Research articles cyclophosphamide therapy review articles cyclophosphamide therapy PubMed articles cyclophosphamide therapy PubMed Central articles cyclophosphamide therapy 2023 articles cyclophosphamide therapy 2024 articles cyclophosphamide therapy Scopus articles cyclophosphamide therapy impact factor journals cyclophosphamide therapy Scopus journals cyclophosphamide therapy PubMed journals cyclophosphamide therapy medical journals cyclophosphamide therapy free journals cyclophosphamide therapy best journals cyclophosphamide therapy top journals cyclophosphamide therapy free medical journals cyclophosphamide therapy famous journals cyclophosphamide therapy Google Scholar indexed journals nontraumatic splenic rupture articles nontraumatic splenic rupture Research articles nontraumatic splenic rupture review articles nontraumatic splenic rupture PubMed articles nontraumatic splenic rupture PubMed Central articles nontraumatic splenic rupture 2023 articles nontraumatic splenic rupture 2024 articles nontraumatic splenic rupture Scopus articles nontraumatic splenic rupture impact factor journals nontraumatic splenic rupture Scopus journals nontraumatic splenic rupture PubMed journals nontraumatic splenic rupture medical journals nontraumatic splenic rupture free journals nontraumatic splenic rupture best journals nontraumatic splenic rupture top journals nontraumatic splenic rupture free medical journals nontraumatic splenic rupture famous journals nontraumatic splenic rupture Google Scholar indexed journals obstructive cerebrovascular disease articles obstructive cerebrovascular disease Research articles obstructive cerebrovascular disease review articles obstructive cerebrovascular disease PubMed articles obstructive cerebrovascular disease PubMed Central articles obstructive cerebrovascular disease 2023 articles obstructive cerebrovascular disease 2024 articles obstructive cerebrovascular disease Scopus articles obstructive cerebrovascular disease impact factor journals obstructive cerebrovascular disease Scopus journals obstructive cerebrovascular disease PubMed journals obstructive cerebrovascular disease medical journals obstructive cerebrovascular disease free journals obstructive cerebrovascular disease best journals obstructive cerebrovascular disease top journals obstructive cerebrovascular disease free medical journals obstructive cerebrovascular disease famous journals obstructive cerebrovascular disease Google Scholar indexed journals tenderness articles tenderness Research articles tenderness review articles tenderness PubMed articles tenderness PubMed Central articles tenderness 2023 articles tenderness 2024 articles tenderness Scopus articles tenderness impact factor journals tenderness Scopus journals tenderness PubMed journals tenderness medical journals tenderness free journals tenderness best journals tenderness top journals tenderness free medical journals tenderness famous journals tenderness Google Scholar indexed journals Rheumatology articles Rheumatology Research articles Rheumatology review articles Rheumatology PubMed articles Rheumatology PubMed Central articles Rheumatology 2023 articles Rheumatology 2024 articles Rheumatology Scopus articles Rheumatology impact factor journals Rheumatology Scopus journals Rheumatology PubMed journals Rheumatology medical journals Rheumatology free journals Rheumatology best journals Rheumatology top journals Rheumatology free medical journals Rheumatology famous journals Rheumatology Google Scholar indexed journals gastrointestinal tract articles gastrointestinal tract Research articles gastrointestinal tract review articles gastrointestinal tract PubMed articles gastrointestinal tract PubMed Central articles gastrointestinal tract 2023 articles gastrointestinal tract 2024 articles gastrointestinal tract Scopus articles gastrointestinal tract impact factor journals gastrointestinal tract Scopus journals gastrointestinal tract PubMed journals gastrointestinal tract medical journals gastrointestinal tract free journals gastrointestinal tract best journals gastrointestinal tract top journals gastrointestinal tract free medical journals gastrointestinal tract famous journals gastrointestinal tract Google Scholar indexed journals
Article Details
1. Introduction
Polyarteritis nodosa (PAN) is a rare syndrome, predominantly affects medium sized arteries in various organs [1]. The skin and peripheral nervous system are the most frequently involved target organs followed by the gastrointestinal tract and the kidneys which indicate a poor prognosis [1]. Our case presented with pure cutaneous manifestations seven years ago, systemic involvement was excluded by MR angiography in that period. But now he presented with gastrointestinal involvement. We emphasized the presentation and treatment of this case.
2. Case Report
A 43 year old man, with obstructive cerebrovascular disease history, was diagnosed cutaneous PAN with a biopsy on erythema nodosum-like skin lesions seven years ago. Pathological findings were fibrinoid necrotic vasculitis of middle and small arteries. The patient was followed in remission, treatment with steroids and methotrexate. He presents with sudden onset, severe, diffuse abdominal pain of 18 hours’ duration and evaluated in the emergency room, diagnosed with spontaneous splenic rupture and acute abdomen. Abdominal examination revealed severe tenderness in the upper umbilical region with rebound tenderness. Diagnostic imaging with computed tomography showed 1 cm liver hemangioma, hepatic artery aneurism and perisplenic high-density fluid in hemorrhagic characteristics. Investigations revealed elevated C reactive protein, erythrocyte sedimentation rate, neutrophilic leukocytosis. Considering the main organ involvement, treatment with both pulse steroids (methylprednisolone 1000 mg/day, three days followed by 64 mg/day) and cyclophosphamide (1000 mg/month six times) was administered, leading to significant clinical improvement. In the first month of the treatment, we observed that the hematoma was organized in images and the patient is stable. After six cycles of cyclophosphamide treatment, six months after disease onset; PAN was inactive. Long term maintenance therapy of the patient continued with oral azathioprine. Today the patient is in good clinical condition with no abdominal pain, no fatigue, no skin rash. Acute phase reactants returned to the normal range.
3. Discussion
Patients diagnosed polyarteritis nodosa (PAN), may present with cutaneous and life threatening multiple organ manifestations such as gastrointestinal, cardiac, neurologic system involvement [2]. Histopathology showed the inflammation and fibrinoid necrosis in the entire arterial wall, which is a typical feature of PAN. Also deposition of polymorphonuclear leucocytes and antigen-antibody complexes in the arterial wall weakens the vessels, prepares the ground for aneurysm formation and eventual rupture [3]. Gastrointestinal (GI) involvement with ischemia and hemorrhage can cause acute abdomen. Surgery may be necessary due to bowel perforation or massive G?S bleeding [4]. High mortality rates can be seen in patients presenting with acute abdomen despite combined medical and surgical treatment [5]. Limited form of the disease, cutaneous PAN is more frequent than systemic disease, typically have benign clinical course and its etiology is unknown. Progressing of cutaneous form to the systemic PAN is rarely seen [6]. Treatment is dependent on the severity of disease which includes corticosteroids with the addition of immunosuppressive agents. Combination of high dose steroids and cyclophosphamide treatment was recommended for remission induction in patients with moderate to severe systemic vasculitides (any evidence of renal insufficiency, gastrointestinal, cardiac, or neurologic involvement) followed by long term maintenance therapy with steroid sparing drugs such as azathiopurine, mycophenolate mofetil and methotrexate [7].
4. Conclusion
Polyarteritis nodosa (PAN) may present with life threatening organ manifestations. We describe the case of a patient with PAN presented with bleeding due to splenic rupture, successfully treated with a combination of pulse steroids and cyclophosphamide therapy. Failures of response to immunosuppressive medications and radiological intervention can lead to high mortality or permanent organ damage. Timely diagnosis and aggressive treatment are keys to a successful outcome.
References
- De Virgilio A, Greco A, Magliulo G, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev 15 (2016): 564-570.
- Levine SM, Hellmann DB, Stone JH. Gastrointestinal involvement in polyarteritis nodosa (1986-2000): presentation and outcomes in 24 patients. Am J Med 112 (2002): 386-391.
- Lie JT. Illustrated histopathologic classification criteria for selected vasculitis syndromes. American College of Rheumatology Subcommittee on Classification of Vasculitis. Arthritis Rheum 33 (1990): 1074-1087.
- De Carpi JM, Castejón E, Masiques L, et al. Gastrointestinal involvement in pediatric polyarteritis nodosa. J Pediatr Gastroenterol Nutr 44 (2007): 274-278.
- Mocan H, Mocan M, ?en Y, et al. Fatal polyarteritis nodosa with massive mesenteric necrosis in a child. Clin Rheumatol 18 (1999): 88-90.
- Ngan Do BS, Sarah Ringold MD, Heather Brandling, et al. Cutaneous polyarteritis in pediatric patients successfully treated with TNF-alpha inhibitor and methotrexat: Case series and literature review. Pediatric Dermatology 36 (2019): 932-935.
- Eleftheriou D, Dillon MJ, Tullus K, et al. Systemic polyarteritis nodosa in the young: a single?center experience over thirty?two years. Arthritis Rheum 65 (2013): 2476-2485.
Impact Factor: * 1.7
Acceptance Rate: 74.36% 
Time to first decision: 10.4 days 
Time from article received to acceptance: 2-3 weeks 