|Year : 2019 | Volume
| Issue : 6 | Page : 383-384
Multiorgan involvement with severe gastrointestinal system manifestations in a patient with granulomatosis with polyangiitis
Ashish Kataria1, Sandeep Magoon2
1 Division of Nephrology, Department of Medicine, University at Buffalo, Buffalo, NY, USA
2 Division of Nephrology, Department of Medicine, Eastern Virginia Medical School, Norfolk, VA, USA
|Date of Web Publication||17-Sep-2019|
Dr. Ashish Kataria
Division of Nephrology, University at Buffalo, NY
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kataria A, Magoon S. Multiorgan involvement with severe gastrointestinal system manifestations in a patient with granulomatosis with polyangiitis. Indian J Nephrol 2019;29:383-4
|How to cite this URL:|
Kataria A, Magoon S. Multiorgan involvement with severe gastrointestinal system manifestations in a patient with granulomatosis with polyangiitis. Indian J Nephrol [serial online] 2019 [cited 2020 Jun 2];29:383-4. Available from: http://www.indianjnephrol.org/text.asp?2019/29/6/383/266994
Granulomatosis with polyangiitis (GPA) is a systemic vasculitis affecting small to medium-sized blood vessels of various organ systems with necrotizing and granulomatous inflammation. It most commonly involves the respiratory tract and kidneys, and involvement of other organ systems is less common. When kidneys are affected, it has high predilection to cause end-stage renal disease (ESRD). Gastrointestinal (GI) involvement happens rarely in GPA but when affected, there is high risk of morbidity and mortality from intestinal perforation and bleeding.
In the current issue of the journal, Bagai et al. describe a case of 50-year-old male with GPA involving multiple organ systems and severe GI manifestations. He initially presented with ear-nose and throat (ENT) manifestations but progressed to have diffuse multisystemic involvement affecting the skin, lungs, GI system, and kidneys. The diagnosis was made by skin biopsy, surgical pathology of the resected bowel, and a positive c-ANCA (antineutrophil cytoplasmic antibody) titer. GI manifestations included severe lower GI bleeding, development of terminal ileal stricture and ulceration, multiple colonic ulcers, and multiple small bowel telangiectasias. He was treated with intravenous (IV) steroids, cyclophosphamide, hemodialysis, and plasmapheresis. Surgical resection was also attempted but the patient eventually died.
GI involvement has been described in most systemic small vessel vasculitides such as GPA, microscopic polyangiitis, eosinophilic granulomatous with polyangiitis, Henoch Schonlein purpura, and cryoglobulinemic vasculitis, although with variable frequencies. GI manifestations are often indistinguishable from those of inflammatory bowel disease, and the patient may present with GI bleeding, ulceration or perforation. Often GI symptoms are the first manifestations that predate involvement of other organ systems and directly correlate with the adverse patient outcomes.
The authors highlight the catastrophic GI involvement in ANCA vasculitis. Although their patient's clinical and laboratory features fit the diagnostic criteria for GPA, other multi-system small vessel vasculitides can have a very similar presentation. Patients with GPA commonly present with ENT or respiratory manifestations (up to 90%). Renal involvement is seen in 18% at the onset and in over 85% eventually happens during the course of the illness. In a recent study by Sharma et al., GI involvement in GPA was seen in 12.3% of patients  and a presence of GI or renal involvement was noted to predict a worse outcome in the multivariate analysis. In another study, GI involvement was noted in 9 of the 34 (26%) patients with GPA during the course of illness. GI bleeding or perforation was seen in six patients and two required surgical intervention.
Clinical features in conjunction with laboratory parameters are very important in making a specific diagnosis. Patient in the current report had severe acute kidney injury, active urine sediment along with GI bleeding and perforation. Pathology of the resected bowel showed necrotizing inflammation, commonly seen in other vasculitides. Skin biopsy confirmed the presence of small vessel vasculitis and a positive c-ANCA clinched the diagnosis of GPA. The patient was treated with IV steroids, cyclophosphamide, and plasmapheresis keeping in line with the current recommendations for organ-threatening ANCA vasculitis. An exploratory laparotomy led to surgical resection of the involved small bowel. Despite potent immunosuppressive medications and extensive surgical resection, the patient could not be saved and succumbed to the complications of renal failure, GI bleeding, and perforation.
In conclusion, GI involvement in GPA is uncommon but not unknown and can have a catastrophic presentation. High clinical suspicion with early aggressive immunosuppressive therapy and timely surgical intervention remains the cornerstone of management.
| References|| |
Bagai S, Sharma A, Gupta R, Kumar V, Rathi M, Kohli HS, et al. Gastrointestinal involvement in granulomatosis with polyangiitis: Case report and review. Indian J Nephrol 2019;29:415-8. [Full text]
Pagnoux C, Mahr A, Cohen P, Guillevin L. Presentation and outcome of gastrointestinal involvement in systemic necrotizing vasculitides: Analysis of 62 patients with polyarteritis nodosa, microscopic polyangiitis, Wegener granulomatosis, Churg-Strauss syndrome, or rheumatoid arthritis-associated vasculitis. Medicine (Baltimore) 2005;84:115-28.
Schneider A, Menzel J, Gaubitz M, Keller R, Lügering N, Domschke W, et al
. Colitis as the initial presentations of Wegener's granulomatosis. J Intern Med 1997;242:513-7.
Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, et al
. The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. Arthritis Rheum 1990;33:1101-7.
Jennette JC, Falk RJ. Small-vessel vasculitis. N Engl J Med 1997;337:1512-23.
Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD. Wegener granulomatosis: An analysis of 158 patients. Ann Intern Med 1992;116:488-98.
Sharma A, Naidu GSRSNK, Rathi M, Verma R, Modi M, Pinto B, et al
. Clinical features and long-term outcomes of 105 granulomatosis with polyangiitis patients: A single center experience from north India. Int J Rheum Dis 2018;21:278-84.
Masiak A, Zdrojewski Ł, Zdrojewski Z, Bułło-Piontecka B, Rutkowski B. Gastrointestinal tract involvement in granulomatosis with polyangiitis. Prz Gastroenterol 2016;11:270-5.
Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al
. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016;75:1583-94.