Limited Wegener's Granulomatosis Research Articles

Wegener's Granulomatosis: Strawberry Gums of the Oral Cavity

Wegener's Granulomatosis is a rare and potentially life-threatening vasculitic disease of unknown origin. The diagnosis of Wegener's Granulomatosis is made on the basis of clinical presentation, positivity for serum c-ANCA and histologic examination of the lesions. This report details a case of limited Wegener's Granulomatosis with hyperplastic gingivitis lesions presenting as an initial manifestation of Wegener's Granulomatosis. The resemblance of the affected gums to over-ripe strawberries is pathognomonic of this condition and is thus highlighted. Because of the rarity of Wegener's Granulomatosis presenting initially in the oral cavity, clinicians should be alerted to the characteristic appearance of “strawberry gingivitis”.

Cardiac Involvement in Granulomatosis With Polyangiitis (Wegener Granulomatosis)

A 38-year-old man was admitted to the emergency department with a 12-hour onset of shortness of breath and position-related chest pain. In the last 3 months, he had had 2 similar but less intense episodes, each of which lasted for approximately 1 week. Both episodes were associated with subfebrility, flulike symptoms (dry cough, nasal obstruction), and increased fatigue and resolved with nonsteroidal antiinflammatory medication. The patient was diagnosed 2 years earlier with limited Wegener granulomatosis with upper and lower respiratory tract involvement (positive for antineutrophil cytoplasmic antibodies [anti-proteinase 3], a positive sinus ethmoidalis biopsy, and no renal involvement) and was undergoing immunosuppressive therapy (azathioprine and methylprednisolone). On admission, the patient was stable and afebrile and tolerated decubitus. The clinical examination was unremarkable, with no respiratory or cardiac pathological findings on auscultation. The chest radiograph showed a normal cardiac silhouette, clear lungs, and a small left pleural effusion. The ECG performed on admission showed sinus rhythm, normal PR interval, and diffuse flattened T waves. Initial blood tests showed mild anemia (hemoglobin 12.2 mg/dL) and marked inflammation, with an elevated erythrocyte sedimentation rate (86 mm/h) and C-reactive …

A 44‐year‐old woman with cutaneous bullae and extensive skin necrosis

Five weeks before presentation, the patient had experi-enced a cough and mild dyspnea. She was diagnosed withpneumonia, completed a 7-day course of ciprofloxacin anddoxycycline, and her respiratory symptoms resolved.Three weeks before presentation, the patient had reportedfevers, chills, diaphoresis, arthralgias, and myalgias, anddeveloped a small erythematous lesion on her left breast.The lesion subsequently enlarged and became hemor-rhagic. She simultaneously developed additional lesionson her chest, abdomen, arms, and back. The largest ofthese, located on her left arm, was 15 cm in diameter. Allof the lesions were exquisitely tender. She was hospital-ized at an outside hospital for 5 days and given a diagnosisof cocaine-induced vasculitis. Upon discharge, she wasinstructed to abstain from cocaine and was prescribedtrimethoprim/sulfamethoxazole for a possible cellulitis in-volving the skin lesions.Ten days after discharge from the outside hospital, thepatient presented to our institution for pain managementand further evaluation. The rash had progressed since herearlier discharge. She had continued to use cocaine, her-oin, and prescription opioids. She had last smoked co-caine 3 days before presentation and last used intranasalheroin 1 week before admission. She reported acquiringcocaine from a new supplier 2 weeks before her rash hadbegun.

Successful remission induction with a combination therapy of rituximab, cyclophosphamide, and steroids in a patient with refractory optic neuritis in Wegener’s granulomatosis

A 56-year old patient with a history of limited Wegener's granulomatosis presented with signs and symptoms of optic neuritis. Radiologic signs of orbital inflammation were absent. Testing of visual acuity and visual field allowed close monitoring of disease activity. Thus, rapid improvement of visual function was achieved with high-dose steroids. Despite maintenance therapy with cyclophosphamide recurrence occurred repeatedly as soon as tapering of steroids was begun. After remission induction with rituximab, the patient retained good visual function under maintenance therapy with azathioprine and low-dose corticosteroids. Optic neuritis in Wegener's granulomatosis without signs of orbital involvement is rare and most likely caused by occlusive vasculitis of the vasa nervorum. In this patient with optic neuritis refractory to conventional therapy, rituximab in combination with cyclophosphamide and corticosteroids was well tolerated and successfully used for remission induction, followed by maintenance therapy with azathioprine and low dose corticosteroids.

Mucocutaneous Leishmaniasis Masquerading as Wegener Granulomatosis

A 43-year-old Brazilian female presented in 2001 with nasal stuffiness and sinusitis. A biopsy was consistent with limited Wegener's granulomatosis although antineutrophil cytoplasmic antibodies were negative. Her nasal inflammation progressed despite trials of prednisone, methotrexate, and azathioprine. A septal perforation developed and a repeat biopsy showed granulomatous inflammation. In 2006 the patient was referred to Division of Rheumatology, University of California, Los Angeles. The nose was grossly erythematous and a magnetic resonance imaging revealed nasal destruction and sinusitis. Palatine biopsies showed chronic inflammation. Cyclophosphamide at 150 mg/d resulted in markedly improved mucocutaneous lesions. The patient developed a leg and arm rash in 2007. A skin biopsy was positive for Leishmania braziliensis. The cyclophosphamide was discontinued and amphotericin B was initiated with transient benefit. Remission was achieved with pentavalent antimony. Despite multiple nasopharyngeal biopsies, for a 6-year span, mucocutaneous leishmaniasis masqueraded as Wegener's granulomatosis. Cyclophosphamide not only resulted in clinical improvement, due to reduced inflammatory response, but also allowed widespread cutaneous dissemination.

Pulmonary Localization Revealing Wegener's Granulomatosis

Wegener's granulomatosis (WG) is the most frequent antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. It affects mainly the upper airways, lungs, and kidneys. Two forms are identified: systemic and limited. We describe three cases of limited WG diagnosed during a 7-year period. Our aim is to report three localized forms of WG and to put emphasis on the necessity of differentiating localized from systemic forms because of their different prognoses and manner of management. Our study contained two men and one woman with a mean age of 43 years. All our patients were symptomatic and presented with nonspecific respiratory signs. The cANCA were positive in all patients. The imaging findings consisted of cavitary masses. The diagnosis was based on surgical lung biopsy in all cases. All patients were put on cyclophosphamide and prednisolone. Only one patient presented with renal complications after a 2-year follow-up period. The two other patients did not present complications after, respectively, 1 month and 1 year of follow-up. These case reports put emphasis on a rare form of WG, the limited form. The low number of patients, due to the rarity of this disease, does not allow us to delineate the characteristics and the differences between this form and the systemic form, but we highlight the necessity of future investigations in order to explore the pathogenesis, therapeutic, and prognosis differences between these two subsets.

Antimullerian hormone, but not follicle stimulating hormone, reflects decline in ovarian reserve associated with oral cyclophosphamide use in Wegener's granulomatosis for the WGET research group

OBJECTIVE: Our aim was to determine the effect of cyclophosphamide (CYC) on ovarian reserve markers antimullerian hormone (AMH) and follicle stimulating hormone (FSH) in patients with Wegener's Granulomatosis (WG). DESIGN: Case control study of stored plasma. MATERIALS AND METHODS: Patients in Wegener's Granulomatosis Etanercept Trial (WGET) received cyclophosphamide (CYC) for severe WG and methotrexate for limited WG. The first and last available plasma samples from the 42 women who were under age 50 at WGET enrollment were analyzed in duplicate for FSH (ELISA Calbiotech) and AMH (ELISA DSLabs). Linear regression analysis and Wilcoxon rank sum tests were performed. RESULTS: While the change in AMH during the study was closely correlated with the dose of CYC received (0.8 ng/mL decline per 10 gms CYC, p=0.009), change in FSH did not correlate with CYC dose. Furthermore, AMH, but not FSH, exhibited a significant decline in patients receiving CYC compared to no CYC.Table 1Change in AMH & FSH from baseline to end of study.CYC in WGETNo CYC in WGETParameterNMean ± std.NMean ± std.p-valueRaw Change of AMH (last visit - baseline)∗Baseline AMH<0.5 ng/mL excluded.11-2.15 ± 1.9460.36 ± 0.890.011Raw Change of FSH (last visit - baseline)†Oral contraceptive pill & GnRH agonist users excluded.227.40 ± 18.4410-1.73 ± 14.080.347∗ Baseline AMH<0.5 ng/mL excluded.† Oral contraceptive pill & GnRH agonist users excluded. Open table in a new tab CONCLUSIONS: In this study, AMH was more closely linked to CYC ovarian damage than FSH. FSH testing was not obtained in the early follicular phase, so was influenced by endogenous estradiol and progesterone during the ovarian cycle of ovulating women. In the oncofertility setting, where patients are not routinely seen in the early follicular phase and may be taking oral contraceptives, AMH is a superior marker of ovarian function.

Clinical implications of serum thrombomodulin in PR3-ANCA-associated vasculitis

BackgroundVascular injury is the main mechanism in pathophysiology of PR3-ANCA-associated vasculitis. Soluble serum thrombomodulin (sTM) is a membrane-bound receptor for thrombin expressed by vascular endothelial cells.ObjectiveThe aim of study was to determine the blood levels of sTM in patients with PR3-ANCA-associated vasculitis.Material and methodsTwenty five patients with Wegener's granulomatosis (WG), 13 with generalized WG and 12 with limited WG, with histologically proven disease, and 15 healthy subjects as a control were investigated. An ELISA for detection of sTM and PR3-ANCA was performed. The disease activity was evaluated according to BVAS and DEI indexes.ResultsSignificant increases in sTM were found in both active generalized and limited active WG compared with control values: 108 ± 12, 56 ± 2, and 12 ± 4 ng/ml, respectively. Elevated ANCA titer correlated with disease activity, but more weakly than sTM levels did. Elevated sTM concentration is a result of vascular endothelial injury in the course of PR3-ANCA associated vasculitis.ConclusionsSoluble serum thrombomodulin is a promising, both diagnostic and therapeutic, marker of endothelial cell injury in relation to disease activity and progression in autoimmune disorders, reflecting the degree of endothelial cell damage.

Exophthalmos as a Presenting Manifestation of Limited Wegener's Granulomatosis in a Patient with Prior Graves' Disease

Wegener's granulomatosis is a granulomatous necrotizing vasculitis characterized by its predilection to affect the upper and lower respiratory tracts and kidneys. Ocular manifestations such as keratitis, conjunctivitis, scleritis, episcleritis, nasolacrimal duct obstruction, uveitis, retroorbital pseudotumor with proptosis retinal vessel occlusion, and optic neuritis have all been described. We present a case of limited Wegener's granulomatosis presenting with proptosis. A 57 year old woman with a history of Graves' disease 20 yrs ago, presented to her ophthalmologist with new onset of exophthalmos of right eye. An MRI of the brain and orbits revealed a soft tissue mass behind the right orbit. The biopsy of the mass revealed transmural inflammation with fibrinoid necrosis consistent with necrotizing vasculitis. She was diagnosed with Wegener's granulomatosis limited to the eye and was treated with oral cyclophosphamide and prednisone followed by weekly methotrexate with good response. Though ocular manifestations of Wegener's granulomatosis are well described, a review of the literature revealed that exophthalmos as the lone presenting manifestation is quite rare. Only two cases have been reported in the English literature since 1977. This case illustrates the importance of considering a diagnosis of limited Wegener's granulomatosis presenting with proptosis of the orbit.

Wegener's granulomatosis in which rheumatoid factor was useful for evaluating the disease status: a case report

IntroductionAccurate evaluation of disease status is very important in treatment of Wegener's granulomatosisCase presentationA 73-year-old Japanese man presented with chronic sinusitis and otitis media. He was admitted to our hospital because of bilateral lung nodules. Cytoplasmic antineutrophil cytoplasmic antibody was negative but his rheumatoid factor was high. He was diagnosed with limited Wegener's granulomatosis and received remission induction therapy. His serum rheumatoid factor level correlated with the Wegener's granulomatosis state when he experienced a severe infection and recurrence due to Wegener's granulomatosis.ConclusionWe describe a case of Wegener's granulomatosis in which rheumatoid factor was helpful for evaluating the therapeutic effect.

Limited Wegener's granulomatosis presenting as lung nodules in a patient with rheumatoid arthritis: a case report

BackgroundRheumatoid arthritis has varied pleuroparenchymal manifestations. Wegener's granulomatosis can develop in an established case of rheumatoid arthritis and this association although previously reported is very rare.Case presentationA 60-year-old lady had been diagnosed with rheumatoid arthritis on the basis of her clinical symptoms and serological tests which were positive RA factor and anti-CCP antibodies. Her rheumatoid arthritis activity had been mild and well controlled with hydroxychloroquine and low dose prednisone. She presented with a productive cough and right-sided pleuritic chest pain. CT scan of the chest showed three lung nodules with increased uptake on PET CT scan, raising concerns for an inflammatory or malignant process. The differential diagnosis included rheumatoid nodules, infections or malignancy. A CT-guided needle biopsy of the largest nodule was undertaken which showed vasculitis typical of Wegener's granulomatosis. Stains and cultures of the biopsy specimen were negative for bacteria, fungi and acid fast bacilli. A panel of serological tests for vasculitis were checked and showed elevated titers of cANCA and anti-proteinase 3 antibodies. Urine analysis and CT scan of paranasal sinuses was normal. Since the upper respiratory tract and the kidneys were spared a diagnosis of limited Wegener's granulomatosis affecting only the lungs was made. Due to the toxicity of cyclophosphamide, her relatively mild disease sparing the kidneys and the underlying rheumatoid arthritis, weekly methotrexate was started and low dose prednisone was continued. She had marked symptomatic improvement and complete resolution of the nodules was documented on subsequent imaging.ConclusionWegener's granulomatosis developing in a patient with rheumatoid arthritis is very rare but should be considered as it warrants a different and possibly more aggressive treatment approach.

A Hole in the Roof

A 20-year-old male noticed that some of the potato chips he was eating were coming out of his nose. Two years before, he had been diagnosed with limited Wegener's granulomatosis on the basis of a typical clinical picture; specifically, he developed a saddle nose and had a positive test for cytoplasmic-staining antineutrophil cytoplasmic antibodies, or c-ANCAs (Figure 1). At that time, his renal function and chest radiographs were normal. Since then, his disease had been managed with methotrexate, 7.5 mg/week, and folic acid supplementation.

Successful treatment of new onset Wegener’s granulomatosis with IVIG (intravenous immunoglobulin) during pregnancy: a case report

We describe a patient with limited Wegener's granulomatosis (WG) presenting during pregnancy with aggressive cutaneous involvement. She was treated with a combination of high-dose corticosteroids and intravenous immunoglobulin (IVIG) during her third trimester. The patient had otherwise uneventful pregnancy and a satisfactory outcome for both herself and her newborn. In the English literature, prior to this report, there have been de novo cases of WG in pregnant women that were diagnosed and treated during pregnancy and three cases of WG treated successfully with IVIG during pregnancy.

Rituximab for Remission Induction in a Patient with Relapsing Necrotizing Scleritis Associated with Limited Wegener's Granulomatosis

Purpose: The authors report a case of necrotizing scleritis associated with Wegener's granulomatosis (WG), which was treated with rituximab for relapsing disease. Method: Observational case report. Results: A 32-year-old male patient presented with necrotizing scleritis in his left eye. The patient was diagnosed as having limited WG. Cyclophosphamide was begun. Under maintenance treatment with azathioprine two relapses of scleritis occurred. Since a high cumulative dose of cyclophosphamide (22.5 g) was utilized initially, two intravenous infusions of rituximab 1 g was given. Complete resolution of scleritis occurred. Conclusions: Rituximab may be effective to induce remission in patients with scleritis due to WG.

Granulomatose de Wegener: relatos de casos

Wegener granulomatosis (WG) is characterized by a classic triad of granulomatous inflammation of the respiratory tract, necrotizing vasculitis and nephritis. The absence of renal disease defines a subset of " limited WG" . Approximately 50% of WG patients develop ophthalmic disease. The histopatological study and +c-ANCA were essential to make a definite diagnosis in these cases.

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.

Nasal Wegener’s and skin sarcoid—a rare combination of two granulomatous diseases

Wegener's granulomatosis (WG) and sarcoidosis are two distinct granulomatous diseases characterized by multisystem involvement. We report a patient who initially presented with symptoms of limited WG predominantly affecting the nose, followed by a facial rash, which was histologically proven to be due to sarcoidosis. The sequential development of these two diseases in one patient is very rare, and to our knowledge, only one such case has been reported in the last 50 years (Am J Kidney Dis 28:893-898, 1996).

Active Endobronchial Wegener??s Granulomatosis

A 47-year-old woman presented with symptoms of cough, streaky hemoptysis, and shortness of breath of 4 weeks' duration. A high-resolution computed tomography of the chest revealed cavitating nodular lesions. Her antinuclear cytoplasmic antibody (cANCA) was positive. She underwent flexible bronchoscopy, which revealed inflammation and ulceration of the arytenoids and epiglottis of the larynx (Fig. 1), along with multiple ulcerative lesions of the tracheal and the left main bronchus (“ulcerative tracheobronchitis”) (Fig. 2). Her transbronchial biopsy revealed necrotizing granulomatosis with capillaritis. This pathologic finding along with the radiologic and laboratory reports led to the diagnosis of Wegener's granulomatosis (WG).FIGURE 1: Inflammation and ulceration of the arytenoids and the epiglottis.FIGURE 2: Ulcerative tracheobronchitis of the posterior wall of the trachea and left main bronchus.DISCUSSION Wegener's granulomatosis is a multisystemic, idiopathic, necrotizing granulomatous vasculitis, which mostly involves the upper and lower airways, lungs, and the kidneys.1-5 Other sites that are also involved are the joints, eyes, skin, and the nervous system.1 When the condition is restricted to the respiratory system without the involvement of the renal system, it is referred as limited Wegener's granulomatosis, which is seen in 25% of patients.1,5,7,8 The lung is the most frequent and sometimes only organ involved. Approximately 85% of patients develop lung development during the course of the disease, and 45% have lung involvement at presentation.6,10,11 Endobronchial involvement occurs in up to 55% of the patients.4,13 Hoffman et al in his series of 158 patients found 73% of the patients had presented with upper airway involvement and 92% during the course of the disease. Lower airway involvement present in 48% of the patients at the time of presentation and 85% during the course of the disease.1 The endobronchial involvement could be in the form of active inflammation and ulceration of the mucosa called “ulcerating tracheobronchitis.” This is the most common presentation seen in up to 55% of the patients along with inflammatory pseudotumor and cobblestoning of the mucosa. The late complications include subglottic stenosis, tracheal stenosis, bronchial stenosis, and tracheobronchomalacia.1,2,4,7,13-16,22,24 There has been anecdotal case reports of main bronchus necrosis with WG.23 The etiology and pathogenesis of endobronchial involvement in WG remains uncertain. Feinberg had proposed the initial fibrinoid degeneration of collagen at sites where there is extensive deposition such as tracheal and nasal cartilage, eliciting a granulomatous immune response.19,20 This could cause inflammation and edema leading to stenosis and later organizing to form fibrotic stenosis or give rise to tracheobronchomalacia. The lower airway symptoms of WG consist of cough being the most common, followed by dyspnea, hemoptysis, chest pain, and stridor.13 The upper airway symptoms consist of sinusitis, rhinitis, otitis, and epistaxis; occasionally, one can get perforation of the nasal septum and saddle nose deformity resulting from destruction of the nasal cartilage.9 The radiologic features vary depending on activity of WG from masses, cavitating nodules, parenchymal bands, septal thickenings, parenchymal opacification, centrilobular nodules, interstitial thickening, and diffuse alveolar shadows in alveolar hemorrhage.1,9 Cavitating nodules and masses are predominantly seen in active diseases. Antineutrophil cytoplasm antibodies (cANCA) have been found positive in 70% to 90% of patients with active WG and has a sensitivity of up to 92%.21,25-27 The renal system involvement presents with abnormal laboratory findings like proteinuria, active urine sediment with microscopic hematuria to red cell casts, and in case of severe glomerulonephritis, it can be seen with decline of renal function as seen by an increase of serum creatinine and decreased creatinine clearance.5 The American College of Rheumatology has published the criteria for diagnosis of WG from other vasculitis condition, which include nasal or oral inflammation, abnormal chest radiograph, urinary sediment, and granulomatous inflammation on biopsy. Any 2 or more criteria present are suggestive of WG and has a sensitivity of 88.2% and specificity of 92%. The most common indications for flexible bronchoscopy include hemoptysis followed by dyspnea on exertion, stridor or wheezing, persistent cough, hoarseness of voice, biopsy of masses, and nodules or new infiltrates on immunosuppressive therapy to rule out opportunistic infections.3,9,13,18 Therapeutic procedures such as removal of pseudotumors by biopsy forceps, laser and electrocautery along with balloon dilatation in tracheal and bronchial stenosis can also be carried out with flexible bronchoscope.12,13,14,17,22 Untreated WG has a poor outcome with a median survival time of 5 months. Glucocorticosteroids have been the mainstay of treatment of WG, and the combination of cyclophosphamide and glucocorticosteroids has been the best in the treatment of WG.9 Recognition of the early spectrum of the endobronchial involvement is essential to ensure early therapy that may prevent irreversible organ damage. Active ulcerating tracheobronchitis in a case of WG which, if not diagnosed or treated, can progress into late endobronchial complication of subglottic stenosis, tracheal, or bronchial stenosis.13,24

Damage caused by Wegener's granulomatosis and its treatment: Prospective data from the Wegener's Granulomatosis Etanercept Trial (WGET)

To analyze damage occurring in patients with Wegener's granulomatosis (WG) enrolled in the WG Etanercept Trial (WGET) and to correlate that damage with disease activity, adverse events, and quality of life. The Vasculitis Damage Index (VDI) was applied to all 180 patients at trial entry and every 6 months throughout the trial. Items of damage were analyzed by presumed etiology (i.e., secondary to WG, to therapy, or both) and time of occurrence. Spearman's rank correlation coefficients were calculated between VDI scores and the Birmingham Vasculitis Activity Score for WG (BVAS/WG), frequency of flares, number of adverse events, and the patients' quality-of-life assessments. The mean VDI score was 1.3 at the study enrollment and 1.8 at the end of the trial. This increase was due to damage that occurred despite (or because of) therapy, including visual impairment, hearing loss, nasal blockade, pulmonary fibrosis, hypertension, renal insufficiency, peripheral neuropathy, gonadal failure, and diabetes mellitus. Only 11% of the enrolled patients had not sustained a single VDI item after 1 year of enrollment. When adjusted for baseline VDI, the baseline BVAS/WG correlated moderately well with the VDI score at 1 year (r = 0.20, P = 0.015). Increases in adjusted VDI scores also correlated with the number of adverse events, particularly among patients with limited WG (P = 0.06). Damage from both active disease and its treatment remain important problems for patients with WG. Despite the dramatic improvements in patient survival achieved over the last several decades, only a few patients with WG emerge from a period of active disease without sustaining some damage from the disease itself, its treatment, or both. An important measure of future therapeutic approaches will be their ability to reduce the damage accrued over time.

ANCA: Serology in Wegener′s granulomatosis

Wegener's granulomatosis (WG) is being increasingly diagnosed in India, which exists in two forms, the 'limited Wegener's granulomatosis' (LWG) having upper respiratory tract (URT) and lower respiratory tract (LRT) involvement and the 'classical Wegener's granulomatosis' (CWG), with the triad of URT, LRT involvement along with kidney involvement. Cytoplasmic ANCA (C-ANCA) or anti-Proteinase3 (anti-PR3), which is highly diagnostic for WG, rarely perinuclear ANCA (P-ANCA) may exist. To detect anti-neutrophil cytoplasmic antibodies (ANCA) and correlate it with serological, hematological parameters, and the Birmingham Vasculitis Activity Score (BVAS). Twenty-three clinically and histopathologically proven WG (16 CWG, 7 LWG) were studied. C-ANCA and P-ANCA patterns were identified by immunofluorescence and specificities were confirmed by 'alpha granule' enzyme linked immunosorbent assay (ELISA), anti-PR3, anti-MPO (myeloperoxidase) and anti-Lactoferrin (anti-LF) by ELISA. LRT involvement was seen in 91.3%, URT in 78.3%, and renal manifestations in 69.6% cases. The BVAS in CWG was significantly higher than BVAS in the LWG. Decreased hemoglobin, increased WBC counts, ESR, CRP and Creatinine were seen in CWG as compared to LWG. The C-ANCA was present in 65.2% patients and P-ANCA in 13% cases. Anti-PR3 was seen in 69.6% patients and anti-LF in 17.4% cases. Severity of disease and ANCA was higher in CWG than in LWG. Vasculitis syndromes are known to overlap and many go undetected; therefore ANCA testing, along with the clinical and histopathological observations may be helpful in early detection and management of WG cases.