Challenges in diagnosis of limited granulomatosis with polyangiitis.

Granulomatosis with polyangiitis (GPA) is an orphan disease with multifaceted clinical presentations and delayed diagnosis. Given the risks of delayed diagnosis and treatment, improving clinicians' awareness of atypical course of this disease is critically important. The aim of this report is to analyze a case of delayed diagnosis of GPA in view of similar publications. We analyzed articles retrieved from Scopus and MEDLINE/PubMed. The following keywords were used: "granulomatosis with polyangiitis", "Wegener granulomatosis", and "diagnostic errors". All case studies that fulfilled the Chapel Hill Consensus Conference and the American College of Rheumatology GPA criteria were retrieved. We report a 71-year-old female patient with a facial defect in the nasal region, nasal congestion, and serosanguineous discharge. Her final diagnosis of GPA was reached after a series of incorrect diagnoses in the past 40 years. A deforming facial lesion developed during this period of uncertainty and absence of appropriate treatment. This patient presented with atypical features of laboratory and instrumental examinations. Anti-neutrophil cytoplasmic antibodies (ANCA) were negative, while rheumatoid factor (RF; 46.3 IU/mL) and anti-citrullinated protein antibody (ACPA; 25.6 IU/mL) were elevated. The histological analysis of the nasal mucous membrane specimen did not indicate definite signs of vasculitis. However, it revealed a granuloma with aggregation of macrophages and massive infiltration of lymphocytes, ruling out previous diagnosis of carcinoma. We analyzed delayed diagnosis of GPA in our patient in the context of 12 previously reported similar cases of limited form of GPA. We emphasize the importance of histological examination for differential diagnosis of GPA.

Prostate involvement in granulomatosis with polyangiitis.

To present a case of prostate involvement (PI) in granulomatosis polyangiitis (GPA) and analyse related published reports. We employed the following keywords for retrieving reports indexed by MEDLINE/PubMed and/or Scopus: "granulomatosis with polyangiitis", "Wegener granulomatosis" and "prostate involvement". Additional searches were performed through Google Scholar and HINARI. All cases that fulfilled the American College of Rheumatology criteria for GPA, standards of Chapel Hill Consensus Conference, and did not match with exclusion criteria were analysed and summarised. A 35-year-old man presented with complaints of stuffy nose, difficulty breathing through the nose, swelling and pain in the left half of the nose, low-grade fever, and discomfort. The nasal mucosal biopsy did not reveal any specific changes. During the inpatient treatment, he developed eye redness, tearing, dysuria, and decreased urinary stream. Prostate-specific antigen (PSA) was elevated (2.81 µg/L; normal values ≤ 1.4 µg/L for males below 40 years). Prostate biopsy findings were consistent with diagnosis of GPA, which was confirmed by detecting elevated anti-PR3 antibodies (4.1 IU; normal values < 1.0 IU). We analysed our case in view of the clinical course of 45 published cases of PI in GPA. PI in GPA is a rare clinical manifestation of the vasculitis. Patients with atypical clinical symptoms of GPA are at risk of delayed diagnosis. The awareness of variable clinical presentations of GPA, particularly specific affection of the prostate gland, is crucial for timely diagnosis.