An unusual presentation of malignant Invasive Mucinous Adenocarcinoma of Lung mimicking Pulmonary Renal syndrome

Background: A 54-year- old male presented to our centre with a chronic non-productive cough and breathlessness. Recent history of COVID treated and resolved few months back. He had a history of brain surgery performed five years back but details not known. Physical examination revealed no oedema and bilateral coarse creps with bronchiolar breathing. Laboratory findings indicated neutrophilic leucocytosis, elevated inflammatory markers, with elevated troponin I and D dimers. Urine analysis suggested microscopic haematuria with sediments. While 24 hour quantification revealed sub nephrotic proteinuria. As auto immune workup and vasculitis profile was negative and patient has not improved in spite of standard of therapy hence we went ahead with CT-Chest indicating ground-glass opacities in bilateral lung parenchyma and prominent interlobular/intralobular septal thickening. Then Bronchoscopy done which revealed the blood-stained secretions in the main stem bronchi and diffuse alveolar haemorrhage in bilateral bronchial segments indicating an inflammatory study, while tuberculosis diagnostic panel and infective bio fire panel in BAL was negative. Meanwhile, his repeat BAL culture suggested Carbapenem resistant Acinetobacter baumannii complex infection. As the patient did not respond to the standard of care for vasculitis. Probability considered was a small vessel vasculitis (namely Granulomatous polyangiitis) was considered due to lung manifestation involving upper respiratory tract with epistaxis, neutrophilic leucocytosis, elevated acute reactive protein, and renal manifestation including microscopic haematuria and proteinuria. However he responded poorly to conventional standard of treatment including pulse steroids and IVIG. Hence after MDT discussion we proceeded with lung biopsy which showed linear cores of lung tissue infiltrated by a malignant neoplasm and acinar pattern suggesting Invasive mucinous adenocarcinoma. Hence we went ahead with the biopsy diagnosis for the treatment plan. As he was to be started on chemotherapy, but he suddenly collapsed and went into hypotension, bradycardia, and cardiac arrest. In spite of high supports and post 4 cycles of CPR, was unable to revive and sadly succumbed to his illness. Discussion(s): In this rare case, the original diagnosis pointed to the pulmonary-renal syndrome, an autoimmune disease characterized by diffuse pulmonary haemorrhage and glomerulonephritis. However, negative autoimmune antibodies and vasculitis profile along with lung biopsy results indicated an unusual case of malignant lung adenocarcinoma presented with pulmonary renal syndrome. Conclusion(s): In cases suggesting pulmonary-renal syndromes, if autoimmune work up is negative and response is suboptimal relook the diagnosis.

Isolated Anti-GBM Disease and COVID-19 Vaccination

Introduction: IgA nephropathy is the most reported glomerulonephritis post-COVID vaccination. Other reported cases include atypical anti-GBM nephritis, among others. Treatment consists of immunosuppressants and plasmapheresis with renal replacement therapy. Renal outcomes have varied. A case is presented of isolated anti-GBM nephritis in a patient whose renal injury occurred weeks after receiving a booster dose of COVID vaccine. Case Description: A 59-year-old male with recent history of ureteral stones with stent placement, travel history in the last 6 months and use of doxycycline for suspected Lyme's disease in the last 3 months presented to the emergency department for decreased urine output, fevers, and arthralgias. He also received a Pfizer COVID vaccine booster 6 weeks ago. His symptoms had worsened in the last 2 weeks. On initial evaluation, he was noted to have stage 3 acute kidney injury (AKI) with creatinine 5.3 mg/dL. Although he had findings of nephrolithiasis, no ureteral obstruction or hydronephrosis were noted on imaging. He received extensive infectious work up which was all negative. Hemodialysis was initiated on day 7 for metabolic derangements and volume overload. After infectious work up was negative, renal biopsy was perfromed revealing linear IgG deposits. Serum anti-GBM antibodies were positive. Despite receiving plasmapheresis, cyclophosphamide and prednisone, the patient continued to require dialysis and was discharged on home hemodialysis. Discussion(s): The development of AKI with systemic symptoms occurred about 6 weeks following his COVID vaccine, longer than previously reported cases. The patient also has a history of nephrolithiasis. At this time, direct association of this patient's anti-GBM disease with the COVID vaccine is unclear however remains a clinical consideration. The presentation of anti-GBM disease is unique as disease is limited to renal involvement. (Figure Presented).

COVID-19 Vaccine as a Potential Triggering Factor for Anti-Glomerular Basement Membrane (GBM) Disease: A Case Report and Literature Review.

Coronavirus 2019 (COVID-19) is considered one of the most significant medical pandemics of this century, with high morbidity and mortality associated with the pandemic. The virus was recognized initially as a cause of pneumonia, but subsequent studies showed significant association with gastrointestinal, neurological, and autoimmune diseases. By 2020, several vaccines became available for use, significantly reducing the infection rate. A good safety profile supported most of the studies related to vaccines. However, this area is still under study, and some reports linked the COVID-19 vaccine to the development of thrombocytopenia, thrombosis, Guillain-Barre syndrome, autoimmune diseases, and myocarditis. These side effects need to be reported to VAERS (Vaccine Adverse Event Reporting System). The exact etiology of anti-glomerular basement (Anti-GBM) disease remains unknown, but the disease is thought to be triggered by environmental factors in genetically predisposed individuals. It is considered one of the serious diseases that could lead to permanent kidney impairment if not treated early and adequately. That's why a great effort is being made by health care practitioners to figure out and avoid the risk and triggering factors. Few previously published papers linked the COVID-19 vaccine and the development of anti-GBM disease, which raised concerns about digging more into this area. Herein, we are reporting a case of a patient who developed rapidly progressive glomerulonephritis (RPGN) due to anti-glomerular basement membrane (GBM) antibody disease two days after receiving the second dose of the COVID-19 vaccine.

COVID-19 PNEUMONIA: A CAUSE OF CAVITARY LUNG LESIONS

SESSION TITLE: Pathologies of the Post-COVID-19 World SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Cavitary lung lesions are a relatively common finding on imaging with a vast differential diagnosis. CASE PRESENTATION: A 36-year-old female with history of gestational diabetes, preeclampsia and obesity presented to an outside hospital for evaluation of acute onset shortness of breath and chest pain. The patient tested positive for COVID19 two weeks prior. She initially developed some mild symptoms including headache, nasal congestion, generalized body aches, cough with mild sputum production and shortness of breath. Despite improvement in these symptoms, she suddenly developed chest discomfort that radiated to the back and was worse with inspiration and cough. The patient had been fully vaccinated against COVID-19 and had received a booster dose as well. Upon evaluation in the emergency room, she was afebrile, respiratory rate was 20, blood pressure was 144/90 mmHg, heart rate was 88 and her oxygen saturation was 99% on room air. Her physical exam was unremarkable. Basic laboratory work up including CBC and chemistry was normal. EKG and troponins were normal as well. A chest x-ay was performed which showed bilateral nodular densities with possible cavitation. A CT chest was later performed and showed multiple bilateral subsolid pulmonary nodules, some with apparent central cavitation. The patient was admitted for further work up at our institution. The patient's subsequent evaluation was largely unrevealing. An autoimmune panel testing for SLE, rheumatoid arthritis, ANCA vasculitis, and Goodpasture's syndrome revealed only a weakly positive ANA with negative anti-DNA and anti-Smith antibody. HIV and QuantiFERON tests were negative. Blood cultures were negative as well. Unfortunately, the patient was not able to expectorate and therefore no sputum cultures were obtained. Due to the patient's clinical improvement and absence of hypoxemia, diagnostic bronchoscopy was deferred, and the patient was subsequently recommended to undergo short interval chest imaging. Repeat chest computed tomography scan one month later showed complete resolution of the previously seen cavitary pulmonary nodules. At the time of outpatient clinical follow up, the patient remained asymptomatic from a respiratory perspective. DISCUSSION: Cavitary pulmonary nodules on chest imaging is an atypical presentation of COVID19 pneumonia that has been rarely described in the literature. In our patient, the temporal correlation between her pulmonary nodules and COVID19 infection as well as her negative work up for other common infectious and inflammatory causes known to cause cavitary lung lesions, makes COVID19 the most plausible cause for her findings. The pathophysiology of these findings remains unclear but may be explained by endothelialitis and small vessel vasculitis may be implicated in the formation of these lesions. [1] CONCLUSIONS: We presented a rare case of cavitary pulmonary nodules due to COVID19 pneumonia. Reference #1: Ackermann M, Verleden SE, Kuehnel M, Haverich A, Welte T, Laenger F, Vanstapel A, Werlein C, Stark H, Tzankov A, Li WW, Li VW, Mentzer SJ, Jonigk D. Pulmonary Vascular Endothelialitis, Thrombosis, and Angiogenesis in Covid-19. N Engl J Med. 2020 Jul 9;383(2):120-128. DISCLOSURES: No relevant relationships by Karim Anis No relevant relationships by Carolyn Garcia

POS-126 PROLIFERATIVE GLOMERULONEPHRITIS WITH MONOCLONAL IgG DEPOSITS;A NOVEL PATHOLOGIC PRESENTATION

Introduction: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a rare form of PGN that mimics immune-complex (IC) GN by light microscopy (LM), but shows monoclonal IgG deposits by immunofluorescence (IF). PGNMID often presents with membranoproliferative (MPGN) pattern or endocapillary hypercellularity. Focal crescents are not uncommon in PGNMID, but diffuse crescentic involvement is very rare. Methods: 78-year-old man with a history of hypertension and multiple cardiovascular comorbidities presented with weakness, dizziness, and anorexia, and was found to have severe hypertension and acute kidney injury with serum creatinine of 12 mg/dl (baseline 1 mg/dl). He was a chronic smoker and alcoholic. He reported productive cough with scanty whitish sputum, but denied hemoptysis. Urine analysis showed marked proteinuria, hematuria, and leukocyturia. Renal ultrasound revealed bilateral decrease corticomedullary differentiation without obstruction. Hemodialysis was initiated. Imaging showed bilateral upper lobe pneumonia with concerns for alveolar hemorrhages. Serology for complements, ANA, dsDNA, ANCA, Hepatitis B and C, Covid19 was negative. Kappa/lambda free light chain ratio was normal. SPEP, UPEP and immunofixation were negative for paraproteinemia. Renal biopsy showed diffuse crescentic and endocapillary PGN with MPGN features, and linear monoclonal IgG3-kappa immune deposits. Given the lack of clinical evidence of cryoglobulinemia and presence of immune-type electron dense deposits without organized substructures by EM, the findings were most consistent with PGNMID. However, the unusual biopsy presentation raised concerns for possible concurrent anti-GBM disease. Subsequently, Solu-Medrol was started followed by prednisone 1 mg/kg. He received 2 sessions of plasmapheresis before anti-GBM serology returned negative. Bone marrow biopsy revealed monoclonal B-cell lymphocytosis with CLL phenotype. Unfortunately, the patient developed Covid19 infection, and passed away before receiving further treatment. [Formula presented] Results: PGNMID is a rare form of renal involvement by monoclonal immunoglobulin deposition that mimics ICGN on renal biopsy. Nephrotic range proteinuria, hematuria and renal insufficiency are usual presentation. Cases of PGNMID classically show IgG3k, in a granular glomerular capillary wall, mesangial, and occasionally subepithelial distribution. By EM, these deposits appear granular typical of ICGN which lack organized substructure. The predominant LM patterns are MPGN and endocapillary hypercellularity. Less frequently focal crescents may be present, but diffuse crescentic involvement is especially rare (~5%). In our case, the diffuse cellular crescents and semilinear to linear GBM staining was unusual. Together with the clinical presentation, the findings prompted concerns for a concomitant Goodpasture syndrome, but anti-GBM antibody returned negative. The pathogenesis is still unclear, but some authors suggest infection as a possible trigger for crescentic transformation in PGNMID. The presence of crescents seem to confer a poorer prognosis and associated with progression to ESRD. Conclusions: Our case is a unique presentation of PGNMID in a patient who presented with clinical and pathologic features concerning for Goodpasture syndrome. PGNMID can rarely present with diffuse crescents and IF findings similar to anti-GBM nephritis in a patient with RPGN. No conflict of interest

POS-131 A STUDY OF ETIOLGIES AND SHORTTERM OUTCOME OF RAPIDLY PROGRESSIVE GLOMERULONEPHRITIS IN A TERTIARY CARE HOSPITAL

Introduction: Rapidly progressive Glomerulonephritis (RPGN) is a syndrome which is caused by glomerulonephritis which results in rapid decline in renal function over a short period of time. Its histological hallmark is extensive crescent formation. It is a heterogeneous disease with various aetiologies leading to glomerular injury. The renal outcome will be dependent on the etiology and mode of treatment and timely initiation of treatment. Methods: The number of renal biopsies performed at our tertiary hospital over a period of 6 months of the study period were determined. The biopsies with crescentic glomerulonephritis were further scrutinized. The demographical data which includes, age, gender, and the baseline estimated glomerular filtration rate (eGFR) as determined by the Modified Diet in Renal Disease (MDRD) formula was collected. The serological test results of ANA, p-ANCA, c-ANCA, ASOT, anti-GBM antibody, C3 and C4 level were also be recorded. The underlying disease process of each of the RPGN cases were recorded into anti-GBM disease, Immune complex mediated, pauci-immune vasculitis, idiopathic or double antibody disease. Treatment of each patient and shorterm renal outcome were recorded. Results: Out of 112 biopsies done over a period of 6 months,16 were crescentric glomerulonephritis. The average age was 30.2 years, there were 5 male and 11 female patients. The majority of them (81.25%) were immune complex mediated and the remainder were ANCA mediated. The underlying cause of the 13 immune complex mediated crescentic glomerulonephritis was lupus nephritis in 8 (61.5%), post infection glomerulonephritis in 3 (23.07%), and antiGBM in 2 (15.38). Pauciimmune glomerulonephritis is 3 out of 16(18.75%) patients. Every patient was treated with immunosuppression. Plasma exchange was done in 2 antiGBM and 2 pauciimmune glomerulonephritis patients. out of 16,10 (62.5%) patients required dialyisis at the time of presentation. 4 out of 10 patients initiated on dialysis became dialysis independent with in 20 days. Renal recovery was not there in both antiGBM patients. one patient of pauciimmune glomerulonephritis succumbed due to Covid- 19 Iinfection. One patient with antiGBM GN had pulmonary alveolar haemorrhage which was responded to plasma exchange. One patient with IRGN was having chronic changes of diabetes on renal biopsy. [Formula presented] Conclusions: The most common cause of RPGN was immune complex mediated GN, mainly SLE lupus nephritis. In our study RPGN was more prevalent in females than males. With early and appropriate treatment renal recovery can be substantially good. Patients with antiGBM GN have poor renal outcomes. No conflict of interest

Anti-Glomerular Basement Membrane Disease as a Potential Complication of COVID-19: A Case Report and Review of Literature.

Since the COVID-19 outbreak has started, many reports showed that COVID-19 does not affect only the respiratory system but can alter multiple organs including kidneys. Anti-glomerular basement membrane disease (anti-GBM) is a systemic disease affecting mainly kidneys and lungs. It can sometimes be triggered by a respiratory infection such as influenza however the mechanism is not clear yet. We describe a novel case of Anti-GBM disease possibly complicating COVID-19. We report a case of a 63-year-old man who was admitted to our hospital for fever and myalgia and was found to have COVID-19. During hospitalization, he developed kidney injury along with pulmonary hemorrhage and was found to have anti-GBM antibodies. Our patient was treated as a case of Anti-GBM disease potentially triggered by COVID-19. Hence, the anti-GBM disease could be a potential complication of COVID-19.