Granulomatosis With Polyangiitis (Wegener's Granulomatosis) Nasal Reconstruction: Improved Outcomes With No Delay.

BACKGROUND: Granulomatosis with polyangiitis (Wegener's granulomatosis) causes progressive nasal collapse, nasal obstruction, and central face deformity. It is not known whether cartilaginous nasal reconstruction should be performed immediately or delayed until after disease "burnout." OBJECTIVES: The aims of this research regarding nasal collapse due to Wegener's granulomatosis were to (1) assess the functional and aesthetic outcomes following immediate vs delayed nasal reconstruction; and (2) measure the impact of psychosocial well-being (anxiety, depression, social isolation) in immediate vs delayed nasal reconstruction. METHODS: Wegener's patients with either immediate or delayed nasal surgery (n = 61) were compared. Functional and aesthetic severity were compared with the validated Standard Cosmesis and Health Nasal Outcome Survey (SCHNOS) score (t test). In addition, Patient-Reported Outcomes Measurement Information System (PROMIS) perioperative and 1-year follow-up surveys were analyzed. RESULTS: At initial consultation, SCHNOS score severity types were similar for both groups (immediate vs delayed): mild 15% vs 15%; moderate 59% vs 60%, and severe 26% vs 25%. Over a 30 ± 4 month period, delayed surgery patients' conditions deteriorated, with a shift from mild to more severe SCHNOS scores, from 25% severe at initial consultation to 85% before surgery. PROMIS scores at presentation were high compared to the general public; by the time of delayed surgery, patients had significantly worsened: anxiety from 28 to 73; depression from 18 to 62; and social isolation from 20 to 80. Although both immediate and delayed groups improved after surgery in functional and psychosocial scores, the immediate surgery group's improvement was superior. CONCLUSIONS: Data showed superior functional and aesthetic scores and superior psychosocial indicators with immediate cartilaginous nasal reconstruction, compared with waiting until disease burnout to undergo surgery.

Rhinoplasty for Sinonasal Deformities in Granulomatosis with Polyangiitis: A Systematic Review and Meta-Analysis.

BACKGROUND: Granulomatosis with polyangiitis (GPA) is a vasculitis that affects respiratory and kidney vessels. It primarily involves the nose and sinuses but can progress systemically. Granulomatosis with polyangiitis causes severe nasal deformities, impacting aesthetics and breathing. Literature focuses on rhinoplasty for saddle nose deformities in non-GPA patients, but its suitability for GPA patients remains uncertain. AIM: Our study aims to assess the effectiveness and safety of rhinoplasty in GPA patients by analyzing the existing literature. METHODS: Four databases were searched; 2 reviewers independently screened the retrieved references, then relevant data for our study were extracted. Categorical outcomes were analyzed using pooled proportions and 95% CI. Statistical heterogeneity was assessed using the I2 statistics χ 2 test with a P -value lower than 0.1 indicating heterogeneity. RESULTS: In our systematic review and meta-analysis, we incorporated 16 studies encompassing 136 patients who underwent nasal reconstruction as part of their treatment for GPA. The collective findings indicate a favorable success rate for rhinoplasty, with a pooled ratio of 89% (95% CI: 84%, 95%). The rate of revision surgeries was relatively low, with a pooled rate of 19% (95% CI: 12%, 26%). Furthermore, rhinoplasty demonstrated comparatively lower rates of recurrence (pooled rate: 22%, 95% CI: 3%, 42%) and complications (pooled rate: 12%, 95% CI: 7%, 18%). CONCLUSION: Rhinoplasty benefits GPA patients with saddle nose deformity and septal perforation with high success rates and low recurrence, revision, and complication outcomes. However, more research is needed for validation and refinement.

Facial Rejuvenation in a Patient With Granulomatosis With Polyangiitis-Treatment Protocol for Compromised Skin Flap.

Facial rejuvenation outcomes have not been well studied in patients with granulomatosis with polyangiitis (GP)-formerly Wegener granulomatosis. This report highlights a case of a 49-year-old female with a history of GP, presenting with facial aging and functional nasal concerns. The patient underwent facial rejuvenation and nasal reconstruction procedures, including primary open functional rhinoplasty, septal perforation repair with Alloderm, deep-plane rhytidectomy, platysmaplasty, nanofat grafting, and fractionated erbium laser to the face (sparing the nose and peri-incisional areas). Despite a routine operation, postoperative course was complicated by wound healing and vascular congestion issues related to her underlying autoimmune disease. This case highlights the risks associated with postsurgical healing in patients with GP undergoing esthetic surgery. To the best of our knowledge, this report is the first to discuss management considerations in a patient with GP undergoing facial rejuvenation surgery.

[Surgical Treatment for Pneumothorax with Granulomatosis with Polyangiitis].

A 42-year-old man had been treated for granulomatosis with polyangiitis for eight years. He was referred to our department with the diagnosis of right pneumothorax on chest radiograph. After chest drainage, the surgical treatment was performed because of continuing air leak from chest tube. Under thoracoscopic approach, the pleural adhesions were carefully dissected and the air leak site was sutured and enforced by a polyglycolic acid sheet with fibrin glue. No recurrence of pneumothorax was observed six months after surgery.

Face Transplantation for Granulomatosis With Polyangiitis (Wegener Granulomatosis): Technical Considerations, Immunological Aspects, and 3-Year Posttransplant Outcome.

BACKGROUND: We report new data for a rare face transplant performed 3 years ago. Granulomatosis with polyangiitis (GPA) (Wegener) is a severe autoimmune necrotizing vasculitis and parenchymal inflammatory disease that can affect any organ including those of the craniofacial region. Skin involvement manifests as malignant pyoderma. This account (1) highlights the technical details of face transplantation for this unique indication, (2) reports the 3-year posttransplant outcome, and (3) describes relevant immunological aspects. METHODS: A Le Fort III near-total face and near-total scalp transplant was performed after extensive trauma and subsequent bone and soft tissue infection in a patient with GPA. Incisions were planned along facial aesthetic subunits. The vascular pedicle comprised the facial and superficial temporal arteries bilaterally. The functioning left eye was preserved and fitted into the donor tissues. RESULTS: The procedure took 21 hours, and transfusion was limited to 4 units of packed red cells. Early medical and surgical complications were successfully treated. At 3 years, acceptable aesthetic outcome was achieved with adequate color match and scalp hair growth. The patient has recovered light touch, temperature, and 2-point discrimination and has evidence of symmetric cheek elevation albeit with limited eyelid and frontalis function. GPA relapse did not occur. Four acute rejections were fully reversed. CONCLUSIONS: This case represents a new underlying disease (trauma + GPA) leading to face transplantation and a unique clinical scenario where allografting was indicated for potentially life-threatening and sight-preserving reasons and not for mere functional and aesthetic concerns. Despite complexity, 3-year clinical outcome is encouraging, and the patient is no longer at risk for dural exposure, meningitis, and related morbidity.

Reconstruction of Wegener Granulomatosis Nose Deformity Using Fascia Lata Graft.

Wegener granulomatosis (WG) is a necrotizing vasculitis that involves the upper respiratory tract, the lungs, and the kidneys. It also causes deformities of the nose in practically all patients, altering the cartilaginous and bony structure as well as the inner lining of the nose.The surgical repair of these nasal distortions is a challenge mainly due to the impairment of wound healing and graft take that is caused both by the disease itself and by the prolonged immunosuppressant treatment.Many different reconstruction plans have been developed, using costal or calvarial bone grafts, cartilage grafts and local skin and mucosa flaps. Results have been diverse.The authors present the case of a 56-year-old patient who suffered from WG and consulted for nasal sequelae. She initially underwent several failed attempts at reconstruction; finally, the authors performed a nasal repair using a double strip of fascia lata as a dorsal graft, with no complications and a good aesthetic result.Fascia lata graft is a new and good option for nasal reconstruction in patients with WG because it is easily obtainable, it can be set in place through closed rhinoplasty and it can achieve good vascularization with low risk of resorption or disease reactivation.

[Varicella gastritis under immunosuppression : Case report of a woman after lung transplantation due to granulomatosis with polyangiitis]. / Varizella-Gastritis unter Immunsuppression : Kasuistik einer Patientin nach Lungentransplantation bei Granulomatose mit Polyangiitis.

A 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. The gastroscopy showed diffuse ulcerative gastric lesions. Tests for varicella zoster virus and Epstein-Barr virus via polymerase chain reactions (PCR) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis. Intravenous antiviral therapy with acyclovir was administered resulting in a normalization of all clinical symptoms, especially of abdominal pain and inflammation parameters.

Case of EGPA and Eosinophilic Chronic Rhinosinusitis Concomitant with IgG4 Related Disease.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic vasculitis in patients with bronchial asthma and eosinophilic rhinosinusitis. Serum IgG4 levels are markedly elevated in patients with active EGPA, a disease which has been closely associated with IgG4-related disease (IgG4RD). A 68-year-old male with a history of asthma and eosinophilic rhinosinusitis developed vasculitis and orbital symptoms. The results of a laboratory examination showed eosinophilia (4,067/µl; 39%), while image evaluations revealed hypertrophy of the rectus muscles, trigeminal nerve, lachrymal gland, and bilateral submandibular glands. Biopsy of the paranasal sinus showed the prominent infiltration of eosinophils and IgG4-positive plasma cells. The patient was diagnosed with EGPA concomitant with IgG4RD and treated with systemic steroids. Although concomitant cases of EGPA with IgG4RD are extremely rare, clinical manifestations associated with both diseases are sometimes mixed. Therefore, systemic scrutiny may be required for cases of EGPA with high serum IgG4 levels and pathognomonic symptoms or findings of IgG4RD

A rare association of chronic lymphocytic leukemia with c-ANCA-positive Wegener's granulomatosis: a case report.

BACKGROUND: Wegener's granulomatosis is a systemic vasculitis of the small- and medium-sized vessels, produced by the action of ANCA, which involves the respiratory tract, kidneys, and eyes, with a potential for lethal evolution in the first year after diagnosis. Its association with chronic lymphocytic leukemia is rarely described in the literature, and it may be difficult to diagnose and to treat this association. CASE PRESENTATION: We present the case of a 73-year-old Caucasian patient, a rare case in which Wegener's granulomatosis is associated in a patient with chronic lymphocytic leukemia, who is admitted in the Infectious Disease Department for fever, diplopia, headache, purulent and hemorrhagic nasal secretions, intense asthenia, and weight loss. The patient had associated eyelid edema; scleritis; chemosis; subconjunctival hemorrhage at the left eye; swelling of the left region of the eyehole, of the base of the nasal pyramid, and of the left zygomatic region; anterior nasal bleeding; pustulous non-itching lesions at the cervical region and posterior thorax; enlarged bilateral axillary lymph nodes; hepatomegaly; and moderate splenomegaly. During the surgical treatment of the pansinusitis, a biopsy from the tissue is taken; the biopsy fragments of the nasal mucosa pleads for Wegener's granulomatosis. The c-ANCA were positive. The patient's evolution was favorable under treatment with meropenem, teicoplanin, fluconazole, transfusions of platelet concentrates, and methylprednisolone. CONCLUSIONS: The real dimension of the association between chronic lymphocytic leukemia and Wegener's granulomatosis is not known; it may be useful to evaluate the vasculitis by testing ANCA routinely in patients with chronic lymphocytic leukemia and by histopathological examinations of the lesions.

Granulomatosis with Polyangiitis (Wegener's Granulomatosis) Complicated with Ruptured Posteromedial Papillary Muscle in the Absence of Coronary Angiographic Findings.

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, associated with antineutrophil cytoplasmic autoantibody-associated systemic vasculitis, and it can affect many organ systems via the inflammation of small-to-medium-sized vessels. Cardiac involvements in GPA are relatively rare. We report a 75-year-old woman who was diagnosed with GPA and rapid progressive glomerulonephritis that resulted in a partial posteromedial papillary muscle rupture, but with no coronary angiographic findings. The surgical and pathological findings with regard to the ruptured papillary muscle revealed necrotic muscle and acute ischemic change. The mechanism of papillary muscle rupture in GPA is coronary vasculitis leading to myocardial infarction. The ischemic change is not always detected on coronary angiography, so assessment using an echocardiogram is important.

A case of marginal zone B cell lymphoma mimicking IgG4-related dacryoadenitis and sialoadenitis.

BACKGROUND: IgG4-related dacryoadenitis and sialoadenitis (IgG4-DS), so-called Mikulicz's disease, is characterized by elevated serum IgG4 and infiltration of IgG4-positive plasma cells in glandular tissues. Recently, several studies reported both malignant lymphoma developed on the background of IgG4-associated conditions and IgG4-producing malignant lymphoma (non-IgG4-related disease). CASE PRESENTATION: We report on the case of a 70-year-old man who was strongly suspected IgG4-DS because of high serum IgG4 concentration (215 mg/dl) and bilateral swelling of parotid and submandibular glands. Biopsies of cervical lymph node and a portion of submandibular gland were performed. These histopathological findings subsequently confirmed a diagnosis of marginal zone B cell lymphoma. CONCLUSION: Differential diagnosis of IgG4-DS is necessary from other disorders, including Sjögren's syndrome, sarcoidosis, Castleman's disease, Wegener's granulomatosis, lymphoma, and cancer. We suggest that biopsy of swollen lesions is important for a definitive diagnosis of IgG4-DS and discuss the mechanism of development in this case.

Orbital presentation of a nasal midline destructive lesion in a young boy.

Midline Destructive Lesions (MDL) are well known to cause nasal problems. There is a long differential diagnosis of such lesions. However, in the pediatric population, the 2 main diseases to be aware of are Non-Hodgkin's T-cell lymphoma and granulomatosis with polyangiitis (previously known as Wegener's granulomatosis). The authors present the report of a 15-year-old boy who presented with epiphora, chemosis, and limitation of left abduction. CT scan of his orbits suggested a destructive lesion of the ethmoid sinuses. His laboratory investigations revealed a positive ANCA. The patient underwent endoscopic sinus surgery, and this was characteristic for granulomatosis with polyangiitis. He was treated with systemic steroids and then maintained on cyclophosphamide, which controlled his disease activity. This case highlights the need for ophthalmologists to have a high index of suspicion for MDL and concomitant orbital disease.

Acute laryngeal dyspnea as first presentation of granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is a multi-organ disease which mostly affects lungs, kidney, and head and neck region. We report a rare case of acute laryngeal dyspnea and rapidly progressive pulmonary changes as first manifestations of disease. A 53 year-old woman presented with symptoms of two-week dyspnea, which aggravated rapidly in the preceding hours. Laryngological examination revealed subglottic infiltrations and vocal fold oedema which required urgent tracheotomy. During few days she developed gingival ulcerations and pulmonary infiltration with negative serum c-ANCA titers. The histopathological examination of subglottic and gingival biopsies and the clinical picture established the diagnosis of GPA. She was treated with prednisone and cyclophosphamide with recovery; however, during over 3 years of follow-up, pulmonary symptoms relapsed and subglottic stenosis persisted. The difficulties in diagnosis and treatment in this unusual presentation of GPA are outlined with conclusion that in patients with subglottic infiltration, which develops rapidly, even when this is a sole presentation of the disease, and when c-ANCA are negative, GPA should always be considered.

[A diagnosis of Wegener's granulomatosis in a 14-year-old patient].

The paper describes a case of Wegener's granulomatosis with upper airway and lung involvements in a 14-year-old patient. Cavernous pulmonary tuberculosis was presumptively diagnosed during planned examination. The diagnosis of Wegener's granulomatosis was established by examining the materials obtained during marginal resection of the lung. The clinical and laboratory indices and histological findings of the biopsy specimens taken during open lung biopsy were considered.

Nasal Reconstruction in Granulomatosis with Polyangiitis: A Two Decade Review.

Background: Granulomatosis with polyangiitis (GPA) leads to progressive destruction of the nasal tissues resulting varying degrees of saddle deformity and nasal obstruction. Reconstructive techniques are numerous, but there are no large series reporting their results. Objective: This study sought to measure complications and outcomes after rhinoplasty for GPA. Methods: We conducted a retrospective review of 42 patients with GPA who underwent nasal reconstruction of saddle nose deformity between 2005 and 2019 using primarily costal cartilage and soft tissue grafts. Results: Thirty-six patients met the criteria for inclusion. All were followed for a minimum of 12 months. Six patients required revision surgery due to infection or GPA flare ups. Five patients had complications. All patients were given a questionnaire at 12 months to rate their degree of satisfaction with their appearance and breathing. Conclusion: The findings of this study suggest that the use of strong cartilage grafts and the timing of surgery result in improvement in breathing and appearance after rhinoplasty in patients with GPA. Clinical Trial Registration number: REB # 21-125.

Remission of Granulomatosis with Polyangiitis Only After Resection of a Pulmonary Nodule.

Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous lesions and is classified as ANCA-associated vasculitis (AAV). We herein report a case of GPA that was remitted by resection of a pulmonary lesion without immunosuppressive therapy. We detected activated neutrophils and neutrophil extracellular traps (NET) formation in resected lung tissue by immunofluorescence. Activated neutrophils and NETs might be involved in the pathophysiology of AAV and induce the vicious cycle of ANCAs and NETs. In cases of GPA with no other severe lesions, the reevaluation of the disease activity after diagnostic resection is crucial for considering the need for immunosuppressive therapy.

Outcomes of renal transplantation in recipients with Wegener's granulomatosis.

Wegener's granulomatosis (WG) is the leading cause of rapidly progressive glomerulonephritis-induced end-stage renal disease (ESRD). In this study, we compared transplant outcomes between recipients with ESRD caused by WG to recipients with ESRD secondary to other causes. Using OPTN/UNOS data from 1996 to 2007, 919 recipients with WG were identified. Post-transplant outcomes included rates of delayed graft function, acute rejection within one-yr post-transplant, overall and death-censored graft survival, and patient survival and were compared between recipients with ESRD secondary to WG versus ESRD from other causes. Recipients with ESRD because of WG had superior unadjusted and adjusted rates of graft loss, patient death, and functional graft loss (adjusted hazard ratio [HR] 0.711, 0.631, and 0.625 respectively, p < 0.001). When we compared the WG cohort to a non-WG, non-diabetic population, the HR for graft loss was still significant, but patient death and death-censored graft loss were not. Subgroup analysis of recipients aged over 60 confirmed that WG recipients had better unadjusted outcomes. This study supports the notion that renal transplantation is an effective treatment option for patients with ESRD secondary to WG. They fare similarly, if not better, than other patients.