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.

Silicone Sphere Implant Extrusion From Orbital Granulomatosis With Polyangiitis: A Rare Complication in the Anophthalmic Socket.

Orbital implant extrusion is a known complication following evisceration and enucleation. In this case report, we present a 45-year-old woman who presented with a left silicone implant exposure and infection 2 years following evisceration with saddle nose on examination. CT of the maxillofacial bones without contrast showed bilateral soft tissue infiltration around the superior recti muscles, as well as a nasal septum perforation from extensive sinus disease. Left orbitotomy revealed a small fibrotic mass near the orbital roof. Biopsy and serology results were consistent with granulomatosis with polyangiitis.

From strawberry gingivitis to palatal perforation: The clinicopathological spectrum of oral mucosal lesions in granulomatosis with polyangiitis.

BACKGROUND: Reports of oral manifestations of granulomatosis with polyangiitis (GPA) usually refer to single-case reports; "strawberry gingivitis" has been increasingly reported. OBJECTIVE: To study the clinicopathological findings of four patients in which the diagnosis of GPA was suspected from the observation of their oral lesions and compare these to existent data. METHODS: Retrospective study of a case series. RESULTS: One patient presented typical "strawberry gingivitis" with localized disease and negative ANCA results. Two patients presented rapidly growing oral ulcers associated with systemic compromise and high ANCA levels. One patient presented with a chronic granulomatous lesion that leaded to palatal perforation. CONCLUSION: Oral manifestations of GPA may vary from rapidly evolving lesions in acutely ill patients to chronic and locally destructive lesions in slowing developing disease. These differences are also evident in the histopathological findings.

Gingival hyperplasia as first sign of recurrence of granulomatosis with polyangiitis (Wegener's granulomatosis): case report and review of the literature.

BACKGROUND: Granulomatosis with polyangiitis (GPA), formerly referred to as Wegener's granulomatosis, is a rare systemic disease of unknown etiology which can affect all areas of the body, including the oral cavity. The typical oral manifestations occur as nonspecific erosive/ulcerative lesions of the oral cavity or appear with hyperplastic gingivitis, a so called "strawberry gingivitis". CASE PRESENTATION: We report here about an extremely rare case with hyperplastic gingivitis as the first sign of recurrence of GPA in the absence of oral manifestations in the primary disease. A 72 year-old female was referred to our Department of Cranio-Maxillofacial Surgery with hyperplastic gingivitis. The patient was diagnosed with GPA already eight years before. We referred the patient to our Clinic of Internal Medicine where she was successfully treated with rituximab. At the follow-up visit, the patient showed complete remission of the hyperplastic gingiva. CONCLUSION: The often overlooked oral manifestation may be interpreted as the first evidence of resurgent GPA in general and therefore could be pathognostic for the disease. This case affirms the need of health professionals to be acquainted with orofacial manifestations of rare diseases such as GPA. As a consequence, dentists will be able to assist in diagnosing GPA more easily leading to a better prognosis for patients suffering from this disease.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting as diffuse myositis.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis is a complex multisystemic syndrome with heterogeneous presentation. Most often, there is a clinical history of asthma or other atopic conditions, and current presentation generally includes signs of cutaneous or pulmonary involvement. Very few reports described myalgia or weakness as the chief complaint. Of these, only a few included muscle biopsy evaluation and none showed convincing evidence of primary myositis. We believe this report is the first to demonstrate true myositis in the setting of early eosinophilic granulomatosis with polyangiitis. CASE PRESENTATION: This report describes a 74 year old Caucasian man, with no known allergies, presenting severe myalgia, muscle weakness, jaw claudication, and fever. Blood work showed marked eosinophilia and high creatine kinase levels. Biceps brachialis muscle biopsy revealed eosinophilic necrotizing vasculitis and true myositis with myophagocytosis of non-necrotic fibers and strong sarcolemmal MHC-1 overexpression by immunohistochemistry. This patient was successfully treated with prednisone and azathioprine. CONCLUSION: Our finding of true myositis in a case of eosinophilic granulomatosis with polyangiitis suggests that primary auto-immunity against muscle fibers, distinct from the secondary effects of vasculitis, can occur in this entity and may represent an overlap syndrome. Early recognition of eosinophilic granulomatosis with polyangiitis in patients presenting with myositis may provide an opportunity to treat the vasculitis before onset of severe multisystemic disease. We recommend the use of muscle biopsy with immunohistochemistry for MHC-1 to confirm the diagnosis of myositis in the setting of eosinophilic granulomatosis with polyangiitis.

Oral granulomatosis with polyangiitis a systematic review.

OBJECTIVE: Granulomatosis with polyangiitis is an unusual multisystemic inflammatory disease, with vasculitis of small- and medium-sized vessels, with a predilection for upper lower airways and kidneys. The etiology remains unknown although it may originate from different stimuli, in genetically susceptible patients. MATERIALS AND METHODS: A detailed database search was performed. The variables were demographics, localization, histopathological findings, antineutrophil cytoplasmic autoantibody, cytoplasmic (c-ANCA) tests, treatment, and follow-up. RESULTS: Fifty-two cases were identified; the mean age was 49.6 years, with a range from 6 to 87 years. It was most frequently seen in females (57.7%). The most common race was white (59.6%). The most frequent location was in the maxillary gingiva (28.8%), followed by both the upper and lower gingiva (19.2%). The most common clinical presentation was "strawberry gingivitis" (61.5%). The main symptom was pain, in 50%. Regarding the c-ANCA test, it was positive in 71.2% of cases. The most common therapy was prednisone and cyclophosphamide, utilized in 51.9%. The average follow-up was 23.6 months, and 88.5% of patients were still alive at follow-up. CONCLUSION: The diagnosis initially was difficult to establish, an early diagnosis and treatment are mandatory. If untreated the disease can be associated with morbidity and mortality. For the oral clinician, this disease needs to be addressed in the differential diagnosis of oral lesions.

Granulomatosis with polyangiitis mistaken as a temporomandibular joint disorder: A case report.

INTRODUCTION: Granulomatosis with polyangiitis (GPA), is a rare systemic disease that if left untreated, it may lead to death within 6-12 months. This case report describes a 15-year-old female with a 14-month history of epiphora, nasal breathing difficulties, headaches, and jaw pain. The patient reported having various medical procedures attempted to address her symptoms. The classical presentation of strawberry gingivitis led to the diagnosis of GPA. Based on a multi-language search, this is the first reported case, that GPA is mistaken as Temporomandibular Joint Disorders (TMD). CASE PRESENTATION: A 15-year-old female with nearly 14 months of numerous complaints, including headaches and jaw pain, was referred for an orofacial pain (OFP) consultation. The patient had completed a dental examination and was prescribed chlorhexidine for gingivitis control. The OFP examination was not consistent with signs and symptoms of TMD. However, the gingival appearance of strawberry gingivitis was suggestive of GPA. A rheumatologist confirmed the diagnosis of GPA based on the clinical interview, serology testing, and dental findings. A course of rituximab and corticosteroids, and regular dental cleanings were recommended. A 4-month follow-up visit demonstrated complete resolution of her jaw pain and headaches. Gingival tissue appeared normal. CONCLUSION: This case emphasizes the need for familiarity with systemic diseases that can present oral manifestations. It also stresses the importance for dental professionals to be knowledgeable of differential diagnoses for TMD, headaches, and autoimmune disorders. Why is this case new information? This is believed to be the first published case mistaking GPA as TMD. This is a case in which a multidisciplinary approach and management were keys for a successful treatment outcome. What are the keys to successful management of this case? Obtain a thorough clinical history. Know the oral manifestation of systemic diseases. Know differential diagnosis of TMD and headaches. What are the primary limitations to success in this case? Having no access to the medical records during the OFP consultation. Having no awareness of differential diagnosis for gingivitis: strawberry, plaque-induced.

Oral manifestations of anti-neutrophil cytoplasmic antibody-associated vasculitis: an update and narrative review of the literature.

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a multisystem disorder of small blood vessels subdivided into granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Oral manifestations (OMs) have been reported to include mucosal ulceration, gingival enlargement, alveolar bone necrosis, tooth loss, oro-antral communication, palatal perforation, parotitis, and candidal infection mainly in GPA. They may appear during the course of the disease, as a disease flare-up, or as the presenting sign. These OMs are often nonspecific and can mimic an array of conditions, therefore formulating a differential diagnosis can be challenging. This review updates the OMs of GPA, and, for the first, time includes OMs of other AAVs. It provides recommendations for the overall assessment and the diagnosis and management of all AAV OMs with considerations for treatment coordination. The role of oral health care providers in multidisciplinary care is highlighted.

Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis.

Wegener's granulomatosis (WG) is a systemic vasculitis of medium-sized and small blood vessels. Aortic involvement in WG is very uncommon. We present a 43-year-old patient with an aortitis with aneurysm formation as a manifestation of WG. The patient was operated on and an aortoiliac Dacron inlay graft was inserted. Postoperatively, he recovered uneventfully. Abdominal pain occurring during a WG flare may result from vasculitis of large abdominal arteries with or without aneurysmatic changes for which surgical treatment and immunosuppressive agents are indicated to prevent a possible rupture.

Strawberry gingivitis as the first presenting sign of Wegener's granulomatosis: report of a case.

Wegener's granulomatosis is a rare multi-system disease characterized by the classic triad of necrotizing granulomas affecting the upper and lower respiratory tracts, disseminated vasculitis and glomerulonephritis. Oral lesions as a presenting feature are only encountered in 2% of these cases. Hyperplastic gingival lesions or strawberry gingivitis, is a characteristic sign of Wegener's granulomatosis. The latter consists of reddish-purple exophytic gingival swellings with petechial haemorrhages thus resembling strawberries. Recognition of this feature is of utmost importance for timely diagnosis and definitive management of this potentially fatal disease. A case of strawberry gingivitis as the first presenting sign of Wegener's granulomatosis affecting a 50-year-old Malay male is reported here. The differential diagnosis of red lesions that may present in the gingiva is discussed.

Gingival Swelling as the Initial Manifestation of Granulomatosis with Polyangiitis.

We describe a case of granulomatosis with polyangiitis (GPA; formerly named Wegener granulomatosis) that presented initially as florid areas of gingival swelling. The patient also had upper respiratory symptoms that included sinus congestion and cough of recent onset. Clinical-pathologic correlation aided the interpretation of non-specific biopsy findings and immediate referral to an appropriate medical specialist. Treatment was rendered at an early stage of disease with a good response to date. Review of the literature indicates that gingival swelling, often with the characteristic appearance of "strawberry gingivitis" may represent the initial sign of disease in 2% of patients with GPA. Biopsy of gingival lesions often shows a non-specific histologic appearance that should be interpreted in the context of the clinical appearance and pertinent medical history. The clinical investigations that lead to definitive diagnosis and treatment are presented to facilitate the management of this uncommon but potentially fatal condition.

Vasculitis Presenting as Autonomic Failure.

INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a vasculitic process that can cause neurological dysfunction in addition to characteristic sinus and pulmonary manifestations. This case report highlights the spectrum of nervous system manifestations and includes rarely reported autonomic and pituitary involvement. CASE REPORT: A 62-year-old woman presented with orthostatic intolerance, tachycardia, dry mouth, and temperature sensitivity; subsequent autonomic reflex study demonstrated widespread postganglionic sympathetic sudomotor, cardiovagal, and cardiovascular adrenergic impairment reflective of autonomic neuropathy and overall autonomic failure. Additional progressive symptoms included dysarthria, dysphagia, bilateral hearing loss, voice hoarseness, and right-sided facial numbness with multiple cranial neuropathies identified on neurological examination. The diagnosis of central diabetes insipidus was also confirmed. Pachymeningitis was present on brain magnetic resonance imaging. Pathologic review of the dural biopsy specimen revealed necrotizing granulomatous vasculitis consistent with GPA. She was treated with intravenous methylprednisolone and rituximab. Over the next 2 months, she had near-complete resolution of her symptoms with normalization on repeat autonomic testing. CONCLUSIONS: This is a unique GPA case presenting with autonomic failure and pituitary dysfunction with conclusive objective findings of autonomic dysfunction. Autonomic dysfunction and other disease manifestations were responsive to immunosuppressive therapy.

Granulomatosis with polyangiitis presenting as pancreatic disease.

Granulomatosis with polyangiitis (GPA) is a rare necrotising small vessel vasculitis typically associated with oronasal, pulmonary and renal manifestations. Pancreatic disease is an exceedingly rare initial presentation and is associated with delayed diagnosis and rapid progression. We discuss a 66-year-old woman presenting with epigastric pain, elevated lipase and radiographic evidence of focal pancreatitis. She had no relevant medical history and no lithiasis seen on imaging. Pertinent findings include strawberry gingivitis, positive proteinase-antineutrophil cytoplasm antibody (98% specificity) and focal nodular parenchymal lung lesions on CT chest-all of which are consistent with a diagnosis of GPA. She was promptly started on high-dose steroids which resulted in significant clinical and biochemical improvement. Cyclophosphamide was added once biopsy confirmed the absence of malignancy. In order to optimise the clinical outcomes of GPA, physicians must keep a wide differential and high index of suspicion in the setting of unexplained pancreatitis with systemic features.

Wegener's granulomatosis: description of a case with oral manifestation.

Wegener's granulomatosis is a multisystemic granulomatous vasculitis that predominantly affects the airways and the kidneys, but may affect any organ. Otorhinolaryngological manifestations may be oral ulcers, gingival swelling or septal perforations that can cause saddle nose deformities, rhinitis, sinusitis and hearing loss. The oral ulcers usually occur when the disease is advanced. Renal involvement is characterized by focal and segmental glomerulosclerosis, and determines the evolution. The diagnosis is made by clinical symptoms and signs, the presence of c-ANCA and a positive biopsy. The anatomic pathology is characterized by vasculitis, granulomatous inflammation with multinuclear giant cells and necrosis. The prognosis has improved as a result of treatment with immunosuppressants associated with corticosteroids. We report a case of a 53-year-old patient with Wegener's granulomatosis with oral manifestation, which began as chronic mastitis.

Granulomatosis with polyangiitis complicated with refractory optic neuritis and maxillary osteomyelitis.

We report a case of a 61-year-old man with granulomatosis with polyangiitis (GPA) complicated with refractory optic neuritis and maxillary osteomyelitis. He had been treated with prednisolone (PSL) as cryptogenic organizing pneumonia in the respiratory department for 2 years. Afterward, he complained tenderness of paranasal sinuses and rapidly progressive visual loss of the left eye. Although both MPO-ANCA and PR3-ANCA were negative, he was diagnosed as GPA based on the American College of Rheumatology 1990 criteria. Ophthalmologic and oral examination revealed left optic neuritis and destructive maxillary bone. Magnetic resonance imaging (MRI) showed the optic neuritis and inflammation around the optic nerve. This finding suggested that the direct spread of inflammation from paranasal sinuses caused the optic neuritis. In a short time, increasing a dose of PSL and administration of intravenous cyclophosphamide were initiated. Antibiotics were also administered to treat sinusitis. Although his visual acuity of the left eye deteriorated to no light perception temporarily, it finally improved after treatment and findings of MRI were also improved. In contrast, destruction of maxilla bone had been progressing. This is a rare case of GPA complicated with optic neuritis due to sinusitis and maxillary osteomyelitis.

Granulomatosis With Polyangiitis Presenting With Strawberry Gums.

This case report describes an ulcerated violaceous nodule on the right nasal ala as well as 3 small ulcers on the neck, back, and buttocks.