scholarly journals Renal involvement in granulomatosis with polyangiitis

2020 ◽  
Vol 8 (6) ◽  
pp. 158-159
Author(s):  
Aya Fraj ◽  
Olfa Berriche ◽  
Sondes Arfa ◽  
Jihen Chelli ◽  
Wafa Bel Abed ◽  
...  
Keyword(s):  
Renal Failure ◽  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Terminal Renal Failure ◽  
Upper Respiratory

Granulomatosis with polyangiitis (GPA) is defined by the presence of small vessel vasculitis that affects the upper respiratory tract, lungs and kidneys. Renal involvement is frequent. The disease varies from asymptomatic to fulminant rapidly progressive glomerulonephritis forms, leading to terminal renal failure in days.

scholarly journals A Clinical Approach of Renal Involvement in Granulomatosis with Polyangiitis

2020 ◽  
Vol 17 (2) ◽  
pp. 29-36
Author(s):  
Marilena Stoian ◽  
Lucia Indrei ◽  
Victor Stoica
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Hospital Admissions ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Granulomatous Inflammation ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
High Dose ◽  
Clinical Approach ◽  
Upper Respiratory Tract

AbstractGranulomatosis with polyangiitis (GPA, Wegener’s granulomatosis) is one of the antineutrophil cytoplasmic antibody (ANCA) - associated small vessel vasculitis, involving various organs such as nasal septum, sinuses, upper respiratory tract, lungs, and kidneys. GPA is pathologically characterized by necrotizing granulomatous inflammation. ANCA-associated small vessel vasculitis represent a major challenge in hospital admissions; therefore, early and accurate diagnosis with aggressive treatment is essential to improve the disease outcome.Renal involvement of granulomatosis with polyangiitis is characterized morphologically by extensive crescent formation (extracapillary proliferation in Bowman’s space), and clinically by crescentic or rapidly progressive glomerulonephritis that causes hematuria, erythrocyte casts, and proteinuria with progressive loss of renal function. The diagnosis of granulomatosis with polyangiitis is established most securely by biopsy specimens showing the triad of vasculitis, granulomata, and large areas of necrosis (known as geographic necrosis) admitted with acute and chronic inflammatory cells. Usually, renal involvement is severe and is the leading cause of mortality. The combination of high-dose corticosteroids and cyclophosphamide is the mainstay of treatment for vasculitis and disease resistance to this combination is rare.

scholarly journals Mycoplasmal upper respiratory infection presenting as leukocytoclastic vasculitis

2015 ◽  
Vol 7 (1) ◽  
Author(s):  
Mana Rao ◽  
Abhinav Agrawal ◽  
Manan Parikh ◽  
Rikka Banayat ◽  
Maria Joana Thomas ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Leukocytoclastic Vasculitis ◽  
Small Vessel ◽  
Stevens Johnson Syndrome ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Cutaneous Lesions ◽  
Upper Respiratory Infection ◽  
Mycoplasmal Infection ◽  
Upper Respiratory

Mycoplasma is a virulent organism that is known to primarily infect the respiratory tract; however, affection of the skin, nervous system, kidneys, heart and bloodstream has been observed in various forms, which include Stevens Johnson syndrome, erythema multiforme, toxic epidermal necrolysis, encephalitis, renal failure, conduction system abnormalities and hemolytic anemia. Small vessel vasculitis is a lesser-known complication of mycoplasma pneumonia infection. We report a case of mycoplasmal upper respiratory tract infection with striking cutaneous lesions as the presenting symptom. Mycoplasmal infection was confirmed by serology testing, skin biopsy was suggestive of leukocytoclastic vasculitis. This case brings forth an uncommon manifestation of mycoplasmal infection with extra-pulmonary affection, namely small vessel vasculitis.

Isolated deafness as a presenting symptom in granulomatosis with polyangiitis

2021 ◽  
Vol 14 (3) ◽  
pp. e241159
Author(s):  
Amy Kousha ◽  
Michael Reed ◽  
Sara Else
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Organ Damage ◽  
Sudden Onset ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Diagnostic Uncertainty ◽  
Tract Involvement ◽  
Upper Respiratory ◽  
Bilateral Deafness

Granulomatosis with polyangiitis (GPA) is a rare small vessel vasculitis commonly affecting the lungs, upper respiratory tract and kidneys. It is an idiopathic condition but likely due to an autoimmune process, resulting in granulomatous lesions and glomerulonephritis. Upper respiratory tract involvement is commonly seen in patients with GPA. Our case is that of an elderly lady (Mrs C) presenting with sudden onset bilateral deafness. She was later found to have extensive lower respiratory tract involvement although she was never particularly symptomatic of this. The presentation suggested a single organ disorder and led to some initial diagnostic uncertainty. Imaging and laboratory investigations eventually led to the diagnosis and she was successfully treated with corticosteroids and rituximab with good response and hearing improvement. This case highlights the importance of early diagnosis in a rapidly progressive disease which undetected can lead to catastrophic end organ damage and disability.

scholarly journals Patient with Antineutrophil Cytoplasmic Antibody Associated Small Vessel Vasculitis, Acute Renal Failure, and Coronavirus Disease-19 Pneumonia: A Diagnostic and Therapeutic Challenge

2020 ◽  
Vol 8 (T1) ◽  
pp. 542-547
Author(s):  
Biljana Gerasimovska-Kitanovska ◽  
Gjulshen Selim ◽  
Zvezdana Petronijevik ◽  
Blagica Pecanova ◽  
Gjorgi Stojchev ◽  
...  
Keyword(s):  
Renal Failure ◽  
Acute Renal Failure ◽  
Systemic Vasculitis ◽  
Renal Involvement ◽  
Rapidly Progressive Glomerulonephritis ◽  
Pulmonary Hemorrhage ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Necrotizing Glomerulonephritis ◽  
Pulmonary Renal Syndrome

BACKGROUND: Antineutrophil cytoplasmatic antibody (ANCA)-associated vasculitis (AAV) has a predilection for the kidney and more than three quarters of patients have renal involvement with rapidly progressive glomerulonephritis. Small-vessel systemic vasculitis may present as pulmonary-renal syndrome and is characterized by necrotizing glomerulonephritis and pulmonary hemorrhage. Diagnosis and therapy for AAV in coronavirus disease (COVID) COVID-19 pandemic require multi-disciplinary collaboration due to the affection of multiple systems and risks associated with immunosuppressive medications. CASE REPORT: A 69-year-old non-smoker, non-diabetic female presented in the outpatient unit at the department of pulmonology with dry cough, malaise, and sub-febrile temperature, lasting for 1 month. The patient had a high suspicion of severe pulmonary-renal syndrome, ANCA-AAV, and acute renal failure requiring hemodialysis. She was treated with corticosteroids, cyclophosphamide, and plasma exchange. The treatment led to temporary improvement. Infections with COVID-19, Enterococcus in the urine, and Acinetobacter in the tracheal aspirate further complicated the clinical picture and despite antibiotic treatment, use of tocilizumab and convalescent plasma, the outcome was lethal. CONCLUSION: It is important to establish the diagnosis and distinguish accurately between vasculitis and infection to provide adequate and timely therapy.

scholarly journals Upper respiratory tract manifestations in patients with ANCA-associated vasculitides and their association with the presence and type of ANCA

2021 ◽  
Vol 59 (5) ◽  
pp. 555-562
Author(s):  
I. G. Smirnova ◽  
N. M. Bulanov ◽  
P. I. Novikov ◽  
I. A. Osipova ◽  
S. V. Moiseev
Keyword(s):  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Upper Respiratory Tract ◽  
X Ray ◽  
Saddle Nose Deformity ◽  
Radiological Patterns ◽  
Upper Respiratory ◽  
Eosinophilic Granulomatosis

Aim of the work – to compare the frequency of upper respiratory tract (URT) involvement in patients with ANCAassociated vasculitides (AAV), to reveal its main clinical and radiological patterns and to estimate their association with the serological profile (ANCA presence and type).Material and methods. This retrospective study evaluated 369 patients with granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). The enrolled patients were diagnosed with AAV according to the ACR criteria, CHCC classification (2012) and EMA algorithm. Patients with URT manifestations underwent standard ENT assessment and X-ray/CT. Serum ANCA levels were measured by ELISA.Results. URT involvement was diagnosed in 280 (75.9%) patients with AAV. It was significantly more common amongthe patients with GPA (86.4%) and EGPA (85.5%) compared with the MPA group (29.2%) (p<0.001).URT manifestations mainly appeared as sinusitis (77.2% – GPA; 33.3% – MPA; 70.8% – EGPA) and rhinitis with crusting (87.8%, 72.2% and 16.9% respectively).Proteinase 3 ANCA positive patients had a significantly higher incidence of bone destructive URT lesions, including sinuses wall destruction (p<0.001) and saddle nose deformity (p=0.016), compared with myeloperoxidase-ANCA-positive patients. Similar results were obtained in the GPA group separately.Localized disease with isolated URT involvement was observed in 41.3% cases of ANCA negative GPA (p<0.001).Conclusion. The frequency and patterns of upper respiratory tract manifestations depend both on the nosologic form of AAV and type of ANCA. Localized forms of URT involvement can be observed in patients with GPA and are closely associated with absence of ANCA, which determines the need for especially high suspicion level.

scholarly journals 177. ALTERATIONS OF DIVERSITY AND COMPOSITION OF THE UPPER RESPIRATORY TRACT MICROBIAL COMMUNITY IN GRANULOMATOSIS WITH POLYANGIITIS

Rheumatology ◽  
2019 ◽  
Vol 58 (Supplement_2) ◽  
Author(s):  
Peter Lamprecht ◽  
Nicole Fischer ◽  
Jiabin Huang ◽  
Lia Burkhardt ◽  
Marc Lütgehetmann ◽  
...  
Keyword(s):  
Microbial Community ◽  
Respiratory Tract ◽  
Granulomatosis With Polyangiitis ◽  
Upper Respiratory Tract ◽  
Upper Respiratory

scholarly journals Henoch-Scholein Purpura (HSP): A Clinico-etiological Study

2016 ◽  
Vol 12 (1) ◽  
pp. 38-33
Author(s):  
SN Kareem ◽  
N Riyaz
Keyword(s):  
Age Of Onset ◽  
Renal Involvement ◽  
Gastrointestinal Symptoms ◽  
Occult Blood ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Joint Involvement ◽  
Upper Respiratory Tract ◽  
Palpable Purpura ◽  
American College Of Rheumatology

Background: Henoch–Schönlein (HSP) is a small vessel vasculitis affecting skin and internal organs.  Objectives: To determine the age group affected, etiological factors, clinical features, complications and association with other diseases. Methods: The study included 50 patients over a period of 3 years selected on the modified criteria by American College of Rheumatology. Clinical assessment was done and investigated for any focus of hidden infections especially Dental and ENT by respective departments. Associated conditions, complications and recurrence were also observed. Results: All patients had palpable purpura (pruritic in 58% and with hemorrhagic bullae in 6%) with upper respiratory tract prodrome (44%), joint involvement (94%) and gastrointestinal symptoms (90%). Precipitating factors were sore throat (74%), caries tooth (44%), drugs (30%) and food (16%) in varying combinations. On investigation 56% had raised ESR and 22% were with abnormal peripheral smears. All biopsies showed leucocytoclastic vasculitis. Occult blood in stool was detected in 10% and 2% showed Streptococci in throat swab culture. Renal involvement was 54% (RBC and albumin-42%, RBC alone-12%). One patient had intestinal perforation. Bleeding time, Clotting time, Platelet count, Cryoglobulin, ANA, Rheumatoid factor, HBs Ag, VDRL and X-ray chest were within normal limits. LE cell test was negative. Follow up study showed recurrence in 98%. Conclusion: HSP is a small vessel vasculitis in response to any unknown foreign or endogenous antigen. The age of onset varied from below 7 to 58 years, with most of them below 20. The most important clinical presentation was palpable purpura in lower extremities. GIT and joint involvement were more. Renal involvement found to be the most common complication. DOI: http://dx.doi.org/10.3126/njdvl.v12i1.10593 Nepal Journal of Dermatology, Venereology & Leprology Vol.12(1) 2014 pp.28-33 

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