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Eye and/or orbit involvement occurs in Granulomatosis with polyangiitis (GPA) patients frequently. The aim of our study was to describe the clinical manifestations, therapy and outcome of ocular involvement in our GPA patients. A retrospective study was conducted including patients with GPA who followed up in Rheumatology clinics during 1990-2016 at King Khalid University Hospital, Riyadh. Information relating to demographics, ocular manifestations, laboratory findings, therapy and outcome of GPA patients were noted. Ocular involvement was detected in 9 (39.1%) of the 23 GPA cases identified. The mean age of ocular GPA patients was 51.8 (range 27 - 62) years, the mean age at onset of disease was 39.6 (range 11 - 57) years and the mean duration of disease was 9.0 (range 2 - 19) years. Concomitant ear, nose, throat and sino-nasal manifestations occurred with ocular symptoms in 77.8% GPA patients. The most frequent manifestations were, eye pain (66.7%), scleritis/episcleritis (55.6%), eye redness and itching (55.6% each). Antineutrophil cytoplasmic antibodies (ANCA) were positive in 88.9% patients, 55.6% had c-ANCA and 33.3% had p-ANCA. Infections were observed in 22.2% of patients, which included pneumonia in one patient and esophageal candidiasis and bacterial meningitis in another. All patients received oral prednisolone, 44.4% received intravenous cyclophosphamide, 22.2% refractory cases received rituximab doses and the disease outcome was good. Comparison of ocular GPA with non-ocular GPA patients showed that 77.8% of ocular GPA patients had concomitant sino-nasal symptoms compared to 42.9% in non-ocular GPA patients and 22.2% of the ocular GPA patients had renal involvement compared to 64.3% in non-ocular GPA patients (p = 0.049). We found that the frequency of ocular manifestations in our GPA patients was similar to reports elsewhere, and the most frequent symptom was eye pain and scleritis/episcleritis.

Objectives: Granulomatosis with polyangiitis (GPA) affects respiratory system very often. In this report, we document the pulmonary manifestations and computed tomography (CT) findings in GPA patients. Subjects and Methods: We conducted retrospective study of GPA patients followed up at King Khalid University hospital, King Saud University, Riyadh, during the period 1990–2014. Various disease manifestations, X-ray and CT findings, therapy, and outcome in GPA patients with pulmonary involvement were retrieved. Results: Among 23 cases of GPA, 14 were males and 9 were females. Mean age at onset of disease was 42.1 ± 14.5 (range: 11–63) years. Respiratory tract was the most frequently involved system (82.6%); upper airways were affected in 56.5% and lungs in 65.2% patients. Among 19 patients with respiratory system involvement antineutrophil cytoplasmic antibody (ANCA) was positive in 18 (94.7%) patients; c-ANCA was positive in 15 (78.9%) and p-ANCA was positive in 3 (15.8%) patients. Chest CT findings in 15 patients showed that lung nodules were the most frequent pathologies, seen in 12 (80.0%) patients, and were cavitated in 8 (53.3%) patients. Airspace opacification was seen in 73.3%, air bronchogram in 20.0%, and ground-glass opacities in 33.3%. CT results at follow-up showed resolution of lung nodules in 9 (60.0%) patients. Lung biopsies showed chronic granulomatous inflammation with vasculitis and necrosis. Intravenous cyclophosphamide was received by 63.2% patients and 10.5% required rituximab therapy along with other drugs. Conclusion: Respiratory system involvement in our GPA patients was similar to previous reports but with variation in certain parameters. Disease outcome was good, and lung CT at follow-up showed resolution of nodules with treatment.

Objective: To explore clinical and laboratory features, therapy and outcome of antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV) patients from our tertiary care center. Methods: This study of AAV patients seen in Rheumatology clinics at King Khalid University hospital (KKUH), King Saud University, Riyadh during the period 1990-2014 was carried out retrospectively. Demographic, clinical, haematological and immunological parameters along with therapy, complications and outcome were retrieved from patients' medical charts. Different characteristics were compared between the three groups of AAV; GPA (Granulomatosis with polyangiitis), MPA (Microscopic polyangiitis) and EGPA (Eosinophilic granulomatosis with polyangiitis). Results: We identified 34 AAV patients (21 males: 13 females; 31 Saudis: 3 non-Saudis) comprising of 23 GPA, 2 MPA and 9 EGPA cases. The mean age of onset was 42.1±17.6 years (range 11-75) and mean duration of disease was 8.7± 5.1 years (range 1-20). The most frequently affected system was pulmonary in all AAV (73.5%), GPA (65.2%) and EGPA (100%) while it was renal in MPA (100%) patients. Ophthalmological and upper airways involvement was higher in GPA. Neurological involvement was higher in EGPA (p

Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronchial asthma and tissue eosinophilia. Familial EGPA is extremely rare. Only two case reports have been published so far. We present a Saudi family with 3 cases of EGPA and almost three-fourths of family members affected by asthma. We explored genetic basis of EGPA in this family and found that genes were mutated in four affected siblings suggesting genetic involvement in susceptibility to EGPA.

Granulomatosis with polyangiitis (GPA) frequently affects ear, nose and throat (ENT) at disease onset. Our aim was to report on our experience with the ENT manifestations in GPA patients, therapy and outcome. A retrospective study of GPA patients was performed who followed up in Rheumatology clinics at King Khalid University hospital, Riyadh during the period 1990-2016. Demographics, different ENT manifestations, laboratory and diagnostic features, therapy and outcome of GPA patients were retrieved from their medical charts. ENT involvement was observed in 15 (65.2%) of the 23 GPA cases identified. Otologic symptoms were noted in 3 (13.0%), nose and sinus symptoms in 13 (56.5%) and throat symptoms in 3 (13.0%) GPA patients. Epistaxis (39.1%) was the most prevalent nose and sinus symptom followed by sinusitis (30.4%), otitis media and hearing loss were the frequent otologic symptoms, oral ulcers and hoarseness of voice constituted frequent throat symptoms in GPA patients. Of the 15 ENT-GPA patients, 9 were males and 6 were females (male: female; 1.5: 1). Their mean age at disease onset was 33.8 ± 18.3 (range 11-57) years and mean duration of disease was 10.1 ± 5.9 (range 1-20) years. ANCA was positive in 93.3% ENT-GPA patients, 73.3% had c-ANCA and 20.0% had p-ANCA. Infections were noted in 33.3% ENT-GPA patients that included pneumonia, septicemia, esophageal candidiasis, bacterial meningitis and herpes zoster. All patients received oral prednisolone, 60.0% received intravenous cyclophosphamide, 20.0% refractory cases received rituximab doses, and the disease outcome was good. Comparison of ENT- GPA with non- ENT GPA cohort showed that 26.7% ENT-GPA patients had renal involvement compared to 87.5% non ENT-GPA patients (p = 0.009). Our study showed that the frequency of ENT symptoms in our GPA patients was less compared to other studies, and the disease outcome was good. Renal involvement was significantly less frequent in ENT-GPA cohort compared to non ENT-GPA cohort.