Granulomatous Polyangiitis With Renal Involvement: A Case Report and Review of Literature

Abstract

Granulomatosis with polyangiitis (GPA), formerly named Wegner’s granulomatosis is an antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis of the small vessels. GPA can affect several organ systems even though predominantly affects respiratory and renal systems. Pathogenesis is initiated by activation of the immune system to produce ANCA, Cytoplasmic (C-ANCA) antibody, which thereby leads to widespread necrosis and granulomatous inflammation. Multisystem involvement with varied symptomatology makes GPA diagnosis more challenging. Early diagnosis and management are vital and can alter the prognosis of the disease. We present a literature review and a clinical scenario of a 26-year-old male with a history of chronic sinusitis, testicular carcinoma in remission, recent onset of worsening cough, epistaxis, hoarseness of voice, weight loss, and dark-colored urine. Workup revealed high titers of C-ANCA, C-reactive protein, procalcitonin, CT chest evidence of mass-like consolidation, and bronchoscopy findings of friable tissue that was not amenable for biopsy. Methylprednisolone and rituximab (RTX) were administered, which resulted in marked clinical improvement. Therefore, a keen eye for details is necessary to diagnose GPA early, which can improve disease outcomes dramatically.

Granulomatosis with polyangiitis with salivary glands involvement: Presenting a case and describing its clinical, pathophysiological, and therapeutic aspects

“…Upper respiratory involvement is predictable in more than 90% of patients, lower respiratory tract involvement (as pulmonary infiltrations, pleural effusion, and nodules) in about 15%-50% of patients, renal involvement in 10%-20% of patients, eye defects (commonly as scleritis) in about 50%, skin defects (as purpura, nodules, ulcers, and granulomas) in 50%-60%, nervous system (commonly as peripheral neuropathies) in 30%-40%, musculoskeletal system (as myalgia and/or arthralgia) in 70%, and even cardiovascular system (as valvular lesions or pericarditis) in less than 10% of patients. [17][18][19][20] To evaluate patients suspected of GPA, all clinical and paraclinical assessments should be considered including minute physical examination, tracking laboratory parameters including blood count, electrolytes, inflammatory markers, imaging, and tittering specific markers of PR3-ANCA and histological assessments if required. 21 Radiological assessment according to the involved organs should be also proposed.…”

“…In this regard, appearing generalized systemic symptoms, especially nonspecific symptoms, are expected in relation to GPA. Upper respiratory involvement is predictable in more than 90% of patients, lower respiratory tract involvement (as pulmonary infiltrations, pleural effusion, and nodules) in about 15%–50% of patients, renal involvement in 10%–20% of patients, eye defects (commonly as scleritis) in about 50%, skin defects (as purpura, nodules, ulcers, and granulomas) in 50%–60%, nervous system (commonly as peripheral neuropathies) in 30%–40%, musculoskeletal system (as myalgia and/or arthralgia) in 70%, and even cardiovascular system (as valvular lesions or pericarditis) in less than 10% of patients 17–20 …”

Renal Outcomes in Older Adults with Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis: A New Prediction Model

“…Patients with AAV were likely to attain renal involvement. The rate of renal involvement in GPA was 54% at diagnosis and increased to 70% over more than 5 years [20].…”

Atlantoaxial instability and cervical noninfectious spondylodiscitis in a patient with Wegener’s granulomatosis: A case report

“…Glomerulonephritis is the most common presentation of WG in kidneys. Reduced glomerular filtration rate (GFR), increased serum creatinine level >2 mg/dl, and end‐stage renal disease (ESRD) are the subsequent common problems in WG in lower frequencies 14,15 …”

“…Reduced glomerular filtration rate (GFR), increased serum creatinine level >2 mg/dl, and end-stage renal disease (ESRD) are the subsequent common problems in WG in lower frequencies. 14,15 Central and peripheral nervous system (CNS/PNS) manifestations of WG are not uncommon but are less noticed. Headaches, sensory problems, motor impairments, neuropathy, cranial nerve deficits, seizure, neuropsychiatric presentations, intraparenchymal lesions, encephalopathy, and hypophysial disturbances are among the most common CNS/PNS presentations of WG.…”