Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but ...Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.展开更多
We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pa...We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pain, nausea and vomiting. Radiography work up was negative for free air although ultrasound examination showed extraluminal intra-abdominal fluid. Exploratory laparotomy showed perforation of the jejunum. The bowel was vital except for this small segment of jejunum. A 5-cm long segment of jejunum was resected which revealed ulcerative inflammation accompanied by occluded arteries of the small intestine. Although intestinal perforation in Wegener's granulomatosis is uncommon, several cases have been previously reported. Intestinal involvement in the early phase of the disease is even more uncommon. This case combined with prev iously reported cases emphasizes the possibility of gastrointestinal manifestation early in Wegener's disease.展开更多
A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener&#...A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener's granulomatosis(WG) involving lungs and sinuses is reported. The patient survived for 12 years after the initial diagnosis of systemic disease. There was perforation of left globe following trauma and no perforation of the right globe till the last follow up of the patient.展开更多
A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of ...A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.展开更多
Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most co...Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most commonly affected organs include upper and lower respiratory tract, kidneys, eyes, nervous system and skin. Kidneys’ involvement has a central position in the classification, diagnosis, treatment and prognosis of patients with WG, and is characterized by the presence of necrotic glomerulonephritis and clinical manifestations that vary from microscopic hematuria to acute renal insufficiency. We describe a case report of a ten year old boy presenting with microscopic hematuria of glomerular origin and a medical history of orbital pseudotumor two years before his hospitalization due to renal symptoms. Renal biopsy revealed lesions of pauci-immune glome-rulonephritis and findings of granulomatous inflam-mation and necrotizing vasculitis. Serum was positive for p-ANCA antibodies (perinuclear staining pattern ANCA antibodies). These findings led to the diagnosis of WG of generalized form (according to EULAR/ PRINTO/PRES criteria). The patient has been treated with aggressive immunotherapy with the use of ster-oids, cyclophosphamide and mycophenolate mofetil. Disease remission has been established and retained one year after initial diagnosis. Orbital pseudotumor, which is a diagnosis of exclusion, has been the initial disease’s clinical manifestation, even though at that time neither the ocular biopsy nor the immunologic workup had been indicative in terms of WG. Although WG is very rare in children, this disease should always been included in the differential diagnosis in patients with similar clinical manifestations and clinicians should emphasize on the recognition of granulomatous vasculitis in biopsies as well as on repeated tests for ANCA antibodies’ detection in serum. High morbidity and mortality rates [1] of this clinical entity necessitates the early recognition of atypical disease’s forms and the close follow up in cases of uncertain initial diagnosis.展开更多
AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRIS...AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRISMA guidelines.We searched Medline,LILACS,Virtual Health Library,and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range(<18 years),with no language limitations.All relevant articles were accessed in full text.The manual search included references of retrieved articles.We extracted data on patients’characteristics,disease characteristics,association with other diseases,and treatment.We analyzed the data and reported the results in tables and text.RESULTS:We retrieved 173 reports of OFG in children.Mean age at onset was 11.1±3.8 years(range:2.0-18years).Prevalence in males was significant higher than in females(P<0.001),with a male:female ratio of 2:1.Gastrointestinal signs or symptoms were present in 26.0%of children at the time of OFG diagnosis.Overall,70/173(40.4%)children received a concomitant diagnosis of Crohn’s disease.In about half(51.4%)of the cases the onset of OFG anticipated the diagnosis of Crohn’s disease,with a mean time between the two diagnoses of 13.1±11.6 mo(range:3-36 mo).Overall,21/173(12.1%)of the children with OFG had perianal disease,while 11/173(6.4%)had a family history of Crohn’s disease.Both perianal disease and a family history of Crohn’s disease were significantly associated with a higher risk of Crohn’s disease diagnosis in children with OFG[relative risk(RR)=3.10,95%confidence interval(CI):2.46-3.90;RR=2.74,95%CI:2.24-3.36,P<0.0001 for both).Treatment of OFG included steroids(70.8%of children)and other immunosuppressive drugs(42.7%),such as azathioprine,thalidomide and infliximab.CONCLUSION:High prevalence of Crohn’s disease in children with OFG suggests that OFG may be a subtype of Crohn’s disease.展开更多
BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel...BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel lesion in GPA patients has been scarcely reported,and it can cause confusion in the diagnosis.CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior.An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe.Both nodules showed central necrosis.Also,there was a periaortic mass occluding the branching porting of the subclavian artery.He had positive anti-neutrophil cytoplasmic antibodies(ANCAs),but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative.The patient also developed symptoms of subclavian vein syndrome during the follow-up.Wedge resection of the lung revealed necrotizing vasculitis,destructive parenchymal abscess and surrounding granuloma,and therefore diagnosed of GPA.The patient started on methotrexate and steroid therapy with a relief of symptomatic.CONCLUSION Here,we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome,which has never been previously described.This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.展开更多
Anogenital granulomatosis (AGG) is a rare chronic idiopathic disorder presenting as a painless swelling of the vulva, labia, penis, and/or anogenital area. Some AGG cases have been diagnosed along with Crohn’s diseas...Anogenital granulomatosis (AGG) is a rare chronic idiopathic disorder presenting as a painless swelling of the vulva, labia, penis, and/or anogenital area. Some AGG cases have been diagnosed along with Crohn’s disease (CD). We report a case of AGG that led to diagnosis of underlying CD, namely Cutaneous Crohn’s disease (CCD). We guess that CCD appeared in the anogenital region is equal to AGG with CD.展开更多
SUMMARY Wegener’s granulomatosisis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasciculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic in...SUMMARY Wegener’s granulomatosisis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasciculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic involvement may occasionally be the initial symptom of WG. A case report of WG was described, which first presented as facial nerve palsy. The combination of facial nerve palsy, sensorineural hearing loss and otitis media was unusual. We should raise suspicion. Early diagnosis is vital if unnecessary surgical exploration is to be avoided. The diagnosis of a WG is made clinically based on clinical findings, histologic confirmation and titres of cytoplasmic pattern antineutrophil cytoplasmic autoantibodies (c-ANCA)/ Anti-proteinase 3 (Anti-PR3). Immunosuppressive therapy with steroids, cyclophosphamide (CTX) is required for relief. A delay in diagnosis may lead to devastating sequelae, such as facial nerve palsy and hearing loss. WG is a challenging disease for otorhinolaryngologist.展开更多
文摘Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.
文摘We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pain, nausea and vomiting. Radiography work up was negative for free air although ultrasound examination showed extraluminal intra-abdominal fluid. Exploratory laparotomy showed perforation of the jejunum. The bowel was vital except for this small segment of jejunum. A 5-cm long segment of jejunum was resected which revealed ulcerative inflammation accompanied by occluded arteries of the small intestine. Although intestinal perforation in Wegener's granulomatosis is uncommon, several cases have been previously reported. Intestinal involvement in the early phase of the disease is even more uncommon. This case combined with prev iously reported cases emphasizes the possibility of gastrointestinal manifestation early in Wegener's disease.
文摘A rare case of bilateral scleromalacia perforans, bilateral peripheral corneal thinning (contact lens cornea) and unilateral orbital inflammatory disease in a 50 year old female patient with an indolent form Wegener's granulomatosis(WG) involving lungs and sinuses is reported. The patient survived for 12 years after the initial diagnosis of systemic disease. There was perforation of left globe following trauma and no perforation of the right globe till the last follow up of the patient.
文摘A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.
文摘Wegener granulomatosis (WG) is a type of vasculitis characterized by the presence of anti-neutrophil cy-toplasmic antibodies (ANCA) and inflammation of small and medium sized vessels with granulomas formation. Most commonly affected organs include upper and lower respiratory tract, kidneys, eyes, nervous system and skin. Kidneys’ involvement has a central position in the classification, diagnosis, treatment and prognosis of patients with WG, and is characterized by the presence of necrotic glomerulonephritis and clinical manifestations that vary from microscopic hematuria to acute renal insufficiency. We describe a case report of a ten year old boy presenting with microscopic hematuria of glomerular origin and a medical history of orbital pseudotumor two years before his hospitalization due to renal symptoms. Renal biopsy revealed lesions of pauci-immune glome-rulonephritis and findings of granulomatous inflam-mation and necrotizing vasculitis. Serum was positive for p-ANCA antibodies (perinuclear staining pattern ANCA antibodies). These findings led to the diagnosis of WG of generalized form (according to EULAR/ PRINTO/PRES criteria). The patient has been treated with aggressive immunotherapy with the use of ster-oids, cyclophosphamide and mycophenolate mofetil. Disease remission has been established and retained one year after initial diagnosis. Orbital pseudotumor, which is a diagnosis of exclusion, has been the initial disease’s clinical manifestation, even though at that time neither the ocular biopsy nor the immunologic workup had been indicative in terms of WG. Although WG is very rare in children, this disease should always been included in the differential diagnosis in patients with similar clinical manifestations and clinicians should emphasize on the recognition of granulomatous vasculitis in biopsies as well as on repeated tests for ANCA antibodies’ detection in serum. High morbidity and mortality rates [1] of this clinical entity necessitates the early recognition of atypical disease’s forms and the close follow up in cases of uncertain initial diagnosis.
文摘AIM:To review pediatric cases of orofacial granulomatosis(OFG),report disease characteristics,and explore the association between OFG and Crohn’s disease.METHODS:We conducted a systematic review according to the PRISMA guidelines.We searched Medline,LILACS,Virtual Health Library,and Web of Knowledge in September 2013 for cases of OFG in the pediatric age range(<18 years),with no language limitations.All relevant articles were accessed in full text.The manual search included references of retrieved articles.We extracted data on patients’characteristics,disease characteristics,association with other diseases,and treatment.We analyzed the data and reported the results in tables and text.RESULTS:We retrieved 173 reports of OFG in children.Mean age at onset was 11.1±3.8 years(range:2.0-18years).Prevalence in males was significant higher than in females(P<0.001),with a male:female ratio of 2:1.Gastrointestinal signs or symptoms were present in 26.0%of children at the time of OFG diagnosis.Overall,70/173(40.4%)children received a concomitant diagnosis of Crohn’s disease.In about half(51.4%)of the cases the onset of OFG anticipated the diagnosis of Crohn’s disease,with a mean time between the two diagnoses of 13.1±11.6 mo(range:3-36 mo).Overall,21/173(12.1%)of the children with OFG had perianal disease,while 11/173(6.4%)had a family history of Crohn’s disease.Both perianal disease and a family history of Crohn’s disease were significantly associated with a higher risk of Crohn’s disease diagnosis in children with OFG[relative risk(RR)=3.10,95%confidence interval(CI):2.46-3.90;RR=2.74,95%CI:2.24-3.36,P<0.0001 for both).Treatment of OFG included steroids(70.8%of children)and other immunosuppressive drugs(42.7%),such as azathioprine,thalidomide and infliximab.CONCLUSION:High prevalence of Crohn’s disease in children with OFG suggests that OFG may be a subtype of Crohn’s disease.
文摘BACKGROUND Granulomatosis with polyangiitis(GPA)is a rare autoimmune disease that involves small-to-medium-sized vessels and forms necrotizing vasculitis with granulomatous inflammation.The formation of a large vessel lesion in GPA patients has been scarcely reported,and it can cause confusion in the diagnosis.CASE SUMMARY A 27-year-old man presented with mild left-sided pleuritic chest pain that started one year prior.An imaging study revealed up to 2.5 cm-sized two irregular nodular consolidation nodule in the left lower lobe.Both nodules showed central necrosis.Also,there was a periaortic mass occluding the branching porting of the subclavian artery.He had positive anti-neutrophil cytoplasmic antibodies(ANCAs),but myeloperoxidase-ANCAs and proteinase 3-ANCAs were negative.The patient also developed symptoms of subclavian vein syndrome during the follow-up.Wedge resection of the lung revealed necrotizing vasculitis,destructive parenchymal abscess and surrounding granuloma,and therefore diagnosed of GPA.The patient started on methotrexate and steroid therapy with a relief of symptomatic.CONCLUSION Here,we present an unusual manifestation of GPA with periaortitis and consequent subclavian steal syndrome,which has never been previously described.This case alerts us that we should include GPA in the differential diagnosis of large vessel vasculitis as well as subclavian steal syndrome.
文摘Anogenital granulomatosis (AGG) is a rare chronic idiopathic disorder presenting as a painless swelling of the vulva, labia, penis, and/or anogenital area. Some AGG cases have been diagnosed along with Crohn’s disease (CD). We report a case of AGG that led to diagnosis of underlying CD, namely Cutaneous Crohn’s disease (CCD). We guess that CCD appeared in the anogenital region is equal to AGG with CD.
文摘SUMMARY Wegener’s granulomatosisis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasciculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic involvement may occasionally be the initial symptom of WG. A case report of WG was described, which first presented as facial nerve palsy. The combination of facial nerve palsy, sensorineural hearing loss and otitis media was unusual. We should raise suspicion. Early diagnosis is vital if unnecessary surgical exploration is to be avoided. The diagnosis of a WG is made clinically based on clinical findings, histologic confirmation and titres of cytoplasmic pattern antineutrophil cytoplasmic autoantibodies (c-ANCA)/ Anti-proteinase 3 (Anti-PR3). Immunosuppressive therapy with steroids, cyclophosphamide (CTX) is required for relief. A delay in diagnosis may lead to devastating sequelae, such as facial nerve palsy and hearing loss. WG is a challenging disease for otorhinolaryngologist.
