Juvenile polymyositis associated with ureteral necrosis: a diagnostic and therapeutic dilemma—case report and review of the literature

Haviv, Ruby; Zehavi, Tania; Pomeranz, Avishalom; Leibovitch, Ilan; Neheman, Amos; Uziel, Yosef

doi:10.1007/s10067-019-04576-4

Cited by 4 publications

(5 citation statements)

References 13 publications

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“…These ndings may be related to vasculitis involving the urinary system [21]. Ruby Haviv [22] reported a case of JDM with right ureteral obstruction secondary to necrosis. The histopathology report of the resected ureter revealed moderate, acute, and chronic in ammatory in ltrates within the ureter wall, urothelial erosions, super cial granulation tissue, degenerated smooth muscle bres within the muscularis propria, some of which were in ltrated with lymphocytes and plasma cells, and multiple calci cations within the lamina propria.…”

Section: Results Of the Literature Reviewmentioning

confidence: 99%

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

Zhou³

et al. 2020

Preprint

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Background: To summarize the characteristics of gastrointestinal (GI) perforation in anti-NXP2 antibody-associated juvenile dermatomyositis (JDM). Methods: Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed. Results: Five patients, including 4 females and 1 male, were involved in the study. The average age of the 5 patients was 5.74 years (5.74 ±2.82). Myositis-specific antibody (MAS) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients, which was successful in only one patient through fistulization. Four patients died (4/5, 80%), and 1 patient was completely relieved after 2 years of treatment. Combined with 16 patients described in the literature, the onset symptom was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most often occurred in the duodenum, although perforations at multiple sites or recurrent perforations also occurred. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions: All perforation cases subjected to MAS analysis were anti-NXP2 antibody positive. The onset symptom was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations complicated early diagnosis. GI perforation is a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

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Section: Results Of the Literature Reviewmentioning

confidence: 99%

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

Zhou³

et al. 2020

Preprint

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“…Morita et al [23] reported a case of dermatomyositis who developed duodenal perforation as well as ureteral stenosis, with biopsy of stenotic ureter showing calcification with granulomatous tissue without any vasculitic lesion. Ruby Haviv et al [24] reported a case of juvenile polymyositis (JPM) with right ureteral obstruction secondary to necrosis. The histopathology report of the resected ureter revealed moderate, acute, and chronic inflammatory infiltrates within the ureter wall, urothelial erosions, superficial granulation tissue, and degenerated smooth muscle fibres within the muscularis propria; some of which were infiltrated with lymphocytes and plasma cells, and multiple calcifications within the lamina propria.…”

Section: Discussionmentioning

confidence: 99%

“…Ruby Haviv et al considered calcified ureteral necrosis to be a feature of visceral vasculopathy, related to both JDM and JPM. The mechanism is obscure, and the presence of calcifications within visceral tissues might suggest the pathogenesis of the typical calcinosis related to these diseases [24]. Considering that NXP-2 is a type prone to calcification, it was not clear whether ureteral calculi and ureteral stenosis in P1 and P2 were related to vasculopathy with calcification, which needs further studies of visceral calcinosis.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature

Zhou

et al. 2021

Pediatr Rheumatol

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Background To summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile dermatomyositis (JDM). Methods Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed. Results Five patients, including four females and one male, were included in the study. The age of onset of these patients ranged from 3.3 to 9.5 years with the median age of 5.0 years. When these patients were complicated by GI perforation, childhood myositis assessment score (CMAS) ranged from 1 to 5 with the median score of 2. Myositis-specific antibody (MSA) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients. One patient failed to undergo a repair as the perforation was high in position. For the other three patients, perforation repair was successful, of which two patients failed due to recurrent perforation. At 24 months postoperative follow-up, one patient was in complete remission on prednisone (Pred) and methotrexate (MTX) treatment, and her ureteral stricture had disappeared. The other four patients died. Adding these cases with 16 other patients described in the literature, the symptom at onset was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most commonly occurred in the duodenum, although it also occurred at multiple sites or was recurrent. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions All the five perforation cases in our study subjected to MSA analysis were anti-NXP2 antibody positive. The symptom at onset was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations prevented early diagnosis. GI perforation may be a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

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“…Morita et al [23] reported a case of dermatomyositis who developed duodenal perforation as well as ureteral stenosis, with biopsy of stenotic ureter showing calci cation with granulomatous tissue without any vasculitic lesion. Ruby Haviv et al [24] reported a case of juvenile polymyositis (JPM ) with right ureteral obstruction secondary to necrosis. The histopathology report of the resected ureter revealed moderate, acute, and chronic in ammatory in ltrates within the ureter wall, urothelial erosions, super cial granulation tissue, and degenerated smooth muscle bres within the muscularis propria; some of which were in ltrated with lymphocytes and plasma cells, and multiple calci cations within the lamina propria.…”

Section: Discussionmentioning

confidence: 99%

“…Ruby Haviv et al considered calci ed ureteral necrosis to be a feature of visceral vasculopathy, related to both JDM and JPM. The mechanism is obscure, and the presence of calci cations within visceral tissues might suggest the pathogenesis of the typical calcinosis related to these diseases [24]. Considering that NXP-2 is a type prone to calci cation, it was not clear whether ureteral calculi and ureteral stenosis in P1 and P2 were related to vasculopathy with calci cation, which needs further studies of visceral calcinosis.…”

Section: Discussionmentioning

confidence: 99%

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

Zhou³

et al. 2020

Preprint

View full text Add to dashboard Cite

Background: To summarize the characteristics of gastrointestinal (GI) perforation in anti-nuclear matrix protein 2 (NXP2) antibody-associated juvenile dermatomyositis (JDM). Methods: Five patients with GI perforation from a JDM cohort of 120 cases are described. Relevant literature was reviewed.Results: Five patients, including four females and one male, were included in the study. The age of onset of these patients ranged from 3.3 to 9.5 years with the median age of 5.0 years. When these patients were complicated by GI perforation, childhood myositis assessment score (CMAS) ranged from 1 to 5 with the median score of 2. Myositis-specific antibody (MSA) spectrum analysis indicated that the five patients were anti-NXP2 antibody positive. The initial symptoms of GI perforation were progressive abdominal pain and intermittent fever. Two patients also presented with ureteral calculus with hydronephrosis and ureteral stricture. Surgery was performed in four patients. One patient failed to undergo a repair as the perforation was high in position. For the other three patients, perforation repair was successful, of which two patients failed due to recurrent perforation. At 24 months postoperative follow-up, one patient was in complete remission on prednisone (Pred) and methotrexate(MTX) treatment, and her ureteral stricture had disappeared. The other four patients died. Adding these cases with 16 other patients described in the literature, the symptom at onset was progressive abdominal pain, which often occurred within 10 months after JDM was diagnosed. Perforation most commonly occurred in the duodenum, although it also occurred at multiple sites or was recurrent. The mortality rate of GI perforation in JDM was 38% (8/21). Conclusions: All the five perforation cases in our study subjected to MSA analysis were anti-NXP2 antibody positive. The symptom at onset was abdominal pain. The most common site of perforation was the duodenum in the retroperitoneum, and the lack of acute abdominal manifestations prevented early diagnosis. GI perforation may be a fatal complication in JDM, and early diagnosis is very important. More research is needed to determine the pathogenesis and predictive factors of GI perforation in JDM.

show abstract

Juvenile polymyositis associated with ureteral necrosis: a diagnostic and therapeutic dilemma—case report and review of the literature

Cited by 4 publications

References 13 publications

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: case reports and a review of the literature

Gastrointestinal perforation in anti-NXP2 antibody-associated juvenile dermatomyositis: Case reports and a review of the literature

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