Lymphopenia in Wegener's granulomatosis. A new clinical activity index?

BACKGROUND: Significant lymphopenia is a prominent feature in systemic erythematous lupus, but has not been described in Wegener's granulomatosis (WG). We suggest from a retrospective analysis that lymphopenia may also be an index of WG disease activity. METHODS: Medical charts form 19 patients diagnosed with systemic active WG between 1990 and 2000 were reviewed retrospectively. All patients had crescentic glomerulonephritis and alveolar hemorrhage. Clinical and biological markers were reviewed at three different time points: diagnosis, time of relapse, and during remission. RESULTS: Average lymphocyte count was significantly lower at diagnosis and relapse than during remission times (p < 0.008 and p < 0.000002, respectively). During disease activity either at diagnosis and during relapses, ANCA titers were highly positive (> or =50 IU/ml) in 27.5% of patients (8/29). The corresponding lymphocyte counts were below normal (1,500/min(3)) in 28 of 29 patients (96.5%). In patients with either negative or weakly positive ANCA, mean lymphocyte count was 728.5, 744.2 and 2,551/ml at diagnosis, during relapse and remission times, respectively. There was a clear negative correlation between the lymphocyte count and disease activity. CONCLUSION: Lymphopenia appears to be a good marker of WG activity. This index might be useful in all patients including those with negative ANCA.     

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.     

Wegener granulomatosis – an atypical case

 An unusual sequence of the clinical manifestations of microvascular disease is described in a 15 year-old girl. She initially presented with acute renal failure caused by a crescentic glomerulonephritis associated with positive tests for MPO-ANCA. Eighteen months later she had pulmonary hemorrhage and respiratory failure. An open lung biopsy showed granulomas that were diagnostic for Wegener granulomatosis. We discuss the diagnostic dilemmas faced in attempts to distinguish infective causes of pulmonary granulomas, such as tuberculosis or fungi, from granulomas associated with vasculitis, in a patient previously treated with immunosuppressive therapy. Received: 4 August 1999 / Revised: 28 October 1999 / Accepted: 3 January 2000     

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.     

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 righttesticular 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 withprominent angiocentric granulomata in the walls of arteries,veins and foci of fibrinoid necrosis,surrounded bypalisading 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 patientshowed an excellent response after local complete excision.He remains free of disease 18 months after orchiectomy.(Asian J Androl 2006 Nov,8:737-739)     

Rare presentation of Wegener’s granulomatosis in the pituitary gland: Case report and literature review

IntroductionWegener’s granulomatosis (WG) is a systemic vasculitis that can affect a variety of organs including ear, nose and throat, lungs and kidneys. However WG is unusual in the pituitary and rare in the central nervous system.Presentation of caseA 56-year-old male with likely WG presented with polyuria and polydipsia despite six months of conservative medical management. MRI scanning revealed an enlarging heterogeneously enhancing pituitary gland. Following endoscopic transsphenoidal pituitary biopsy and debulking, final tissue pathology was diagnostic for WG in the pituitary gland.DiscussionDiagnosis remains difficult but most patients present with central diabetes insipidus (CDI) as well as varying degrees of hypopituitarism on a background of disease activity in other organs. Clinical judgment needs to balance the need for invasive surgical tissue diagnosis with increasing immunosuppressive therapy.ConclusionIt is important to consider this rare complication of WG to ensure timely diagnosis and management.     

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.     

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.     

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.     

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.     

Traumatic lacunar infarction in basal ganglion in children

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What about sentinel node practice in early-staged cutaneous melanoma in France in 2003?

AIM: The aim of this study was to establish the status of sentinel lymph node (SLN) biopsy procedure in cutaneous melanoma in France in 2002. MATERIAL AND METHODS: This study was based upon the statistics of the main French melanoma centers. A short questionnary was sent to Head Physician by email. The authors asked for the global attitude as far as SLN was concerned, number of cutaneous melanoma diagnosed during year 2002 and of SLN procedures performed, critters of inclusion and postoperative management in each case. Abstension could be argued in a free item. Answers were sent back by email. RESULTS: The authors collected 22 answers coming from overall territory; 64% performed SLN procedure (14 centers), 36% applied "wait and watch" policy. Staffs performing SLN diagnosed a mean of 101 (8-400) melanoma and biopsied a mean of 21 (0-53) sentinel nodes. The others diagnosed a mean of 151 (15-250) melanoma. Patients were enrolled for Breslow thickness upper to 1.5 mm in 71%, to 1 mm in 29%. Ulceration was a critter of inclusion in 93% (21 staffs), 100% enrolled patients whose tumor presented signs of regression. SLN was performed for primary sites located overall body in 71%, only in limbs and trunk in 29%. Positive node lead to regional lymph node clearance, then observation or interferon protocol. Negative node lead to "wait and watch policy" in 14%, different interferon protocols according to Breslow thickness in 86%. CONCLUSION: SLN procedure is not homogenous in France. France is divided as far as SLN is concerned. If 64% are performing SLN, more than 50% of the new melanoma are not included in the trial.