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INTRODUCTION: Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is an autoimmune disease marked by necrotizing granulomatous inflammation and vasculitis affecting small-sized vessels. It commonly impacts the renal and respiratory systems. MATERIALS AND METHODS: This retrospective case series sampling conducted in a tertiary care hospital between May 2023 and April 2024 examined six newly diagnosed GPA patients who were proteinase 3 cytoplasmic-antinuclear cytoplasmic antibody (PR3 c-ANCA) positive and had concurrent respiratory infections. None of them had any prior immunosuppressive conditions. The age range was 18-47 years with a mean of 35.0 (standard deviation: 11.83). All the patients had pneumonia (N=6, 100%). Out of all, five had bacterial pneumonia (N=5, 83.3%) and one had tuberculous pneumonia (N=1, 16.7%). A high level of PR3 c-ANCA (>150 RU/mL) was noted in four patients (N=4, 66.7%). Common symptoms included dry cough (N=5, 83.3%), loss of weight and appetite (N=2, 33.3%), and fever (N=2, 33.3%). Three patients had otitis media and/or nasal polyposis (N=3, 50%). Two patients (N=2, 33.3%) with life-threatening organ dysfunction were given concurrent antibiotics and steroids; the antibiotics were later modified based on culture and sensitivity results. One of these patients received antituberculosis therapy as Mycobacterium tuberculosis (MTB) was detected after 27 days of incubation in mycobacterial growth indicator tube broth. The remaining four patients (N=4, 66.7%) received antibiotics initially for 5-7 days until clinical resolution of pneumonia. Ultimately, they all showed clinical and radiological resolution (N=6, 100%) within 3-6 months of treatment. RESULTS: The patients exhibited constitutional symptoms such as fever and weight loss; lower airway disease symptoms including dry cough and hemoptysis; nasal and ear disease symptoms like epistaxis, ear pain, and ear discharge; and a renal disease symptom, hematuria. Computed tomography of the thorax revealed bilateral consolidations, most of which were cavitating. Bronchoalveolar lavage cultures grew Escherichia coli, Burkholderia cepacia, Pseudomonas aeruginosa, Klebsiella pneumoniae, and MTB, whereas pus swab cultures from otitis media grew Pseudomonas aeruginosa, Staphylococcus aureus, and coagulase-negative staphylococci. DISCUSSION: This study highlights the therapeutic challenges of GPA complicated by concurrent infections. Patients exhibited typical GPA signs, confirmed by PR3 c-ANCA levels. Concurrent infections require cautious antibiotic treatment before starting immunosuppressive therapy, except in life-threatening organ dysfunction. A unique case presented with both tuberculosis and GPA. Tailored treatment regimens combining antibiotics and immunosuppressives, including corticosteroids, methotrexate, and rituximab, resulted in clinical and radiological improvement in all the patients within 3-6 months. The addition of co-trimoxazole reduced the incidence of non-severe GPA relapses. CONCLUSION: Tailored treatment plans addressing both infectious and autoimmune aspects are essential for optimal care in GPA complicated by concurrent infections. This study highlights the need for a multidisciplinary approach involving pulmonologist, rheumatologist, microbiologist, and pathologist in the diagnosis and treatment of GPA, emphasizing the importance of individualized treatment plans tailored to the specific clinical scenario.
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BACKGROUND: A Chronic Obstructive Pulmonary Disease (COPD) phenotype is a single or group of disease characteristics that describe differences between individuals based on clinically important factors such as symptoms, exacerbations, morbidity, and treatment responses. Many studies estimated the prevalence of various phenotypes, but very few studies looked into their quality of life. We aimed to estimate the prevalence of different COPD phenotypes and their disease-specific Health-Related Quality of Life (HRQoL). MATERIALS AND METHODS: The prospective study, with a sample size of 136, was conducted between May 2021 and December 2022 in a tertiary teaching institute. Based on their clinical features, COPD patients were classified into 4 different clinical phenotypes, and their disease-specific quality of life was assessed using St. George Respiratory Questionnaire-COPD(SGRQ-c) and COPD Assessment Test (CAT) questionnaires. RESULTS: Among 136 COPD patients, the frequency of Non-Exacerbator (NE), Exacerbator Emphysema (EEM), Exacerbator Chronic Bronchitis (ECB), and Asthma COPD overlap (ACO) phenotypes was 79(58.1 %), 16(11.8 %), 31(22.8 %), and 10(7.4 %) respectively. Based on the SGRQ-c score, the ECB and EEM phenotypes had a significantly poorer Quality of life (QoL) when compared with NE(P<0.0001), ACO(P=0.011), phenotypes. Similarly, ECB and EEM phenotypes had significantly poorer QoL when compared to NE(P<0.0001), and ACO(P=0.015), based on the CAT score. ECB and EEM patients also had the worst scores in all individual CAT items and SGRQ-c components. CONCLUSION: NE was the most common followed by ECB phenotype. ECB and EEM phenotypes recorded the poorest quality of life without any significant differences among them. Further research is needed in the future to determine whether phenotype-specific therapies can produce better clinical outcomes.