Breast mass as the primary presentation of Wegener’s granulomatosis: a case report

A case report of a 27-year-old African-American female with a history of bilateral breast masses is presented. Primary breast cancer or metastatic disease was the presumed etiology. On work-up, however, fine-needle aspiration biopsy followed by open excisional biopsy revealed the diagnosis of Wegeners granulomatosis. Initial presentation of this necrotizing granulomatous vasculitis in the breast is exceedingly rare, and this case highlights the importance of maintaining a degree of suspicion for this clinical entity in the evaluation of a breast mass.     

Bilateral multicystic kidneys – an unusual case

Multicystic dysplasia of the kidneys is a condition whose prognosis is good as it usually presents unilaterally. Bilateral cases are usually fatal in utero. We report a case of bilateral multicystic dysplasia of the kidneys where the lower moiety of the right kidney was spared cystic change. The patient had normal renal function and, following conservative management, remains alive and well 6 months later.     

Pulmonary renal syndrome associated with Wegener’s granulomatosis: a case report and review of literature

Pulmonary renal syndromes are rare but serious complications of systemic vasculitis. The majority of these cases are related to ANCA-associated vasculitis. These syndromes represent a combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Here we report a case of 21-year-old female with a clinical picture of community-acquired pneumonia that rapidly evolved to pulmonary hemorrhage and acute renal failure. Combined pulse steroid treatment, immunosuppressive treatment, and plasmapheresis were instituted and resulted in the improvement of the patient. The diagnosis of Wegener’s granulomatosis was established based on the clinical and serological findings (positive cANCA). Here we review the relevant literature on pulmonary renal syndromes associated with Wegener’s granulomatosis and discuss their diverse clinicopathologic features.     

Limited Wegener＇s granulomatosis of the epididymis and testis

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.     

A case of Wegener’s granulomatosis with pulmonary bleeding successfully treated with double filtration plasmapheresis (DFPP)

We report a case of a 41-year-old Japanese man who presented with rapidly progressive glomerulonephritis, chronic sinusitis, and positive cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA). Renal biopsy showed crescentic glomerulonephritis, and he was diagnosed as having Wegeners granulomatosis. During the clinical course, he suffered from pulmonary bleeding, and combination therapy of steroid, immunosuppressant, and double filtration plasmapheresis (DFPP) was started. He rapidly entered remission after assistance through DFPP, suggesting the potential efficacy of DFPP for Wegeners granulomatosis, especially with pulmonary bleeding.     

Wegener’s granulomatosis with massive gastrointestinal hemorrhage due to jejunal and colonic involvement: Report of a case

Wegener’s granulomatosis (WG) is a systemic necrotizing vasculitis of unknown etiology characterized mainly by the involvement of the upper airways, lungs, and kidneys. Although most organ systems can be involved, gastrointestinal involvement in WG is notably uncommon. We herein present the case of a WG patient who developed two massive gastrointestinal hemorrhages treated respectively by surgery and angiographic embolization of the bleeding artery. The present case indicates that gastrointestinal manifestations might thus be considered in the natural history of WG.     

Aneurysmal subarachnoid hemorrhage in a patient with Wegener’s granulomatosis

A 34-year-old Japanese man admitted to hospital with pneumonia had previously undergone surgery for paranasal sinusitis and also for the clipping of an aneurysm at the origin of the anterior choroidal artery after subarachnoid hemorrhage. Laboratory tests performed at the present admission showed renal insufficiency and serological findings of raised proteinase 3 antineutrophil cytoplasmic antibody level. A renal biopsy was performed that showed diffuse necrotizing glomerulonephritis with fibrocellular crescents. The diagnosis of Wegeners granulomatosis(WG) was confirmed on the basis of the clinical picture, laboratory findings, and biopsies of renal tissues. The disease responded to prednisolone and cyclophosphamide. The association of WG with a ruptured intracranial aneurysm is rare and has not previously been confirmed.     

Unexpectedly difficult intubation caused by subglottic stenosis in Wegener’s granulomatosis

A 76-year-old woman was scheduled to undergo abdominal aortic repair for progressive abdominal aortic aneurysm. After inducing general anesthesia, the 7.5-mm internal diameter (ID) tracheal tube could not be advanced below the level of the vocal cords because of resistance, and intubation was re-attempted several times using smaller tubes. An otolaryngologist was consulted and subglottic stenosis of unknown origin was suggested. The aortic repair was cancelled and tracheostomy was performed instead. She was diagnosed with Wegener’s granulomatosis 46 days after the operation because she developed symptoms of renal dysfunction, hemoptysis, gastrointestinal bleeding, and presence of anti-neutrophil cytoplasmic autoantibodies (c-ANCA). The patient was treated with steroids but died 89 days after the operation because of pulmonary bleeding and renal dysfunction. Tracheal stenosis is a rare presenting feature of Wegener’s granulomatosis that usually occurs late in the disease; however, anesthesiologists around the world need to bear in mind that the disease can present airway symptoms and can be the cause of airway obstruction.     

A case of an atypical femoral fracture associated with bacterial biofilm—pathogen or bystander?

We report a case of an 86-year-old woman with an atypical femoral fracture (AFF) who was treated with intramedullary nailing followed by lateral femoral plating. She developed a second femoral shaft fracture distal to the intramedullary nail which required a second operation. Biopsy of the periosteum overlying the site of the initial proximal AFF was sent for pathogen analysis. Using the Ibis T5000 platform and the BAC plate assay, a polymicrobial infection was diagnosed consisting of Bifidobacterium subtile and Pseudomonas mendocina. This raises the possibility that bacterial infections may play some role in atypical fractures of the femur.     

Granulomatosis with polyangiitis (Wegener granulomatosis): A proteinase-3 driven disease?

Granulomatosis with polyangiitis (GPA, Wegener granulomatosis) is a systemic autoimmune vasculitis that affects small arteries, arterioles, and capillaries, most notably in the kidneys and lungs. In this disease, proteinase-3 (PR3), produced by neutrophils, is targeted by antineutrophil cytoplasmic antibodies (ANCA). Recent work by our group has shown how PR3 impairs the resolution of inflammation and deregulates the immune system. Normally, the clearance of activated neutrophils triggers an anti-inflammatory, pro-resolution process. In patients with GPA, however, macrophages phagocytose apoptotic neutrophils then release massive amounts of pro-inflammatory mediators, notably interleukin-1, thereby generating a pro-inflammatory microenvironment conducive to autoimmunity. This deregulation of immune processes is accompanied with activation of plasmacytoid dendritic cells and with polarization of T-helper-2 (Th2), Th9, and Th17 cells. These recent data highlight the dual role of PR3, both auto-antigenic and auto-inflammatory, thus potentially opening up new therapeutic avenues.     

Benign gastro-bronchial fistula – an uncommon complication of esophagectomy: case report

Background  

Gastro-bronchial fistula (GBF) is a rare and devastating complication following esophagectomy. Making the correct diagnosis is difficult and there is no agreement on the treatment for this rare condition.     

Clinical manifestations of granulomatosis with polyangiitis (Wegener’s granulomatosis) in the upper respiratory tract seen by otolaryngologists in Japan

We studied 91 patients with granulomatosis with polyangiitis (GPA; Wegener’s granulomatosis) who were dealt with by otolaryngologists in Japan. The upper respiratory tract (URT) alone was involved in 56%. Regarding findings of PR3-ANCA, histology and initial diagnosis, of the 40 patients in whom sites other than the URT were involved, 64 and 73 % were positive for PR3-ANCA and histology, respectively. On the other hand, of 51 patients in whom only the URT was involved, only 49 and 31 % were positive for PR3-ANCA and histology, respectively. With regard to diagnosis, definitive and probable diagnoses were made in 78 and 20 %, respectively, of patients involving sites other than the URT. On the other hand, definitive and probable diagnoses were made in only 22 and 37 %, respectively, of patients involving the URT alone; the Japanese diagnostic criteria were inapplicable to 41 %. Of the 21 patients to whom the diagnostic criteria were inapplicable, 13 (62 %) developed additional symptoms and signs during the observation period, and they then fulfilled the Japanese diagnostic criteria. Among these patients, 8 were MPO-ANCA-positive and 3 patients had 2 or more sub-lesions in the URT. If the diagnostic criteria included cases with MPO-ANCA (±) and 2 lesions in the URT, the diagnostic rates increased from 59 to 86 % even though they were of limited form. Thus, about 60 % of the patients with URT symptoms alone did not satisfy the Japanese diagnostic criteria at the initial visit.     

Late recovery of renal function by rituximab in a patient with Wegener’s granulomatosis

Background

Rituximab has proven effective in the treatment of complicated granulomatosis with polyangiitis (Wegener’s, GPA). Two controlled trials in adults demonstrated beneficial effects of rituximab compared to cyclophosphamide in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis to induce remission and to treat relapses. Pediatric experience with rituximab in GPA is limited; the impact on renal function is unknown.

Case-diagnosis/treatment

We report a female adolescent with GPA and necrotizing glomerulonephritis that lead to end-stage renal disease (ESRD). After 22 months of peritoneal dialysis, she still experienced relapses and major treatment-associated adverse effects. After a single dose of rituximab, she rapidly achieved clinical remission and, unexpectedly, steadily recovered glomerular filtration rate, plateauing at 25 ml/min/1.73 m². Peritoneal dialysis could be discontinued for 16 months.

Conclusions

This case documents a potent beneficial effect of rituximab on renal manifestation of GPA even in long-established ESRD.     

Migration of an AXIOS stent complex into the colon – A case report

IntroductionPancreatic fluid collections are a common complication of acute pancreatitis. They are classified as acute peripancreatic fluid collections and pancreatic pseudocysts. There has been an increase in the use of endoscopic ultrasound-guided drainage stents for management of these collections. As a result, complications such as stent migration are becoming more prevalent.Presentation of caseA 47-year-old male presented to the emergency department with upper abdominal pain, nausea and vomiting, and intermittent fevers. The patient had a known history of a pancreatic pseudocyst. He had undergone an endoscopic cyst-gastrostomy and placement of an AXIOS and Compass stents for drainage prior to the current presentation. The patient was investigated with a computed tomography (CT) scan that demonstrated acute pancreatitis, and migration of his AXIOS/Compass stent complex into the transverse colon. The patient was managed conservatively, and ultimately passed the stent through his bowel motions without issue. Follow up abdominal x-ray confirmed the passage of the stent.DiscussionStent migration is a recognised complication of stent placement but is infrequently described for lumen-apposing metal stents like the AXIOS stent. The AXIOS stent has a dumbbell configuration designed to reduce the rate of migration compared to the original double-pigtail plastic stents. Despite this, stent migration still occurs, as in this case.ConclusionComplications of AXIOS stents can also include migration of the stent despite their specific design to prevent this. Conservative management is feasible rather than endoscopic retrieval and can be considered if there are no complicating features.     

An infected urachal cyst presenting as an acute abdomen – A case report

INTRODUCTIONAn infected urachal cyst is one of a spectrum of presentations of urachal pathology, all of which are rare in adulthood.PRESENTATION OF CASEWe report the case of a 45-year-old obese Russian lady who presented with a 2-week history of suprapubic pain radiating to the right iliac fossa. Although previously fit and well, she had a history of 17 miscarriages. Both USS and CT suggested a complicated inflammatory mass in the lower abdomen. Ultimately the diagnosis was made by laparotomy, which revealed an abscess of an urachal cyst. The infected cyst and bladder dome were excised. The patient made a good recovery with an uneventful follow up.DISCUSSIONUrachal cysts are the commonest type of urachal anomaly. Infection is the usual mode of presentation amongst adult cases otherwise the condition usually remains asymptomatic. An infected urachal cyst is an important diagnosis to make as complications include sepsis, fistula formation, and rupture leading to peritonitis. Treatment is by complete excision, however, techniques have been debated.CONCLUSIONThis is a rare but important diagnosis however we recommend that in patients with atypical histories, it should be included in the differential diagnosis.     

Calcaneal osteomyelitis caused by Acremonium sp in an immunocompetent adult – A case report

Fungal osteomyelitis of calcaneus is rare in immunocompetent adults. A case of fungal osteomyelitis of calcaneus in a 19 year old immunocompetent female without any history of trauma caused by Acremonium sp is presented. The patient was treated with surgical debridement, autogenous iliac crest bone grafting and antifungal chemotherapy for 6 months. At latest follow up after 2 years, patient is asymptomatic and the osteolytic lesion is healed with no signs of recurrence. This case report highlights a rare presentation of chronic painful calcaneal osteomyelitis with an osteolytic lesion.