scholarly journals Top Differential Diagnosis Should Be Microscopic Polyangiitis in ANCA-Positive Patient with Diffuse Pulmonary Hemorrhage and Hemosiderosis

2014 ◽  
Vol 2014 ◽  
pp. 1-5
Author(s):  
Nicholas D. Ward ◽  
Diane E. Cosner ◽  
Colleen A. Lamb ◽  
Wei Li ◽  
Jacqueline K. Macknis ◽  
...  

A rat model of antineutrophil cytoplasmic antibody (ANCA) associated vasculitides reveals crescentic glomerulonephritis as seen in human renal biopsies and diffuse lung hemorrhage that is not well documented in human lung biopsies. A 64-year-old male, with shortness of breath and mild elevation of serum creatinine, was found to have a positive serum test for ANCA, but negative antiglomerular basement membrane antibody. A renal biopsy showed pauci-immune type of crescentic glomerulonephritis and focal arteritis. The prior lung wedge biopsy was retrospectively reviewed to show diffuse hemorrhage and hemosiderosis with focal giant cells. In addition, small arteries revealed subtle neutrophil aggregation, and margination along vascular endothelium, but no definitive vasculitis. The pathology of ANCA associated vasculitides results from activated neutrophils by ANCA and subsequent activation of the alternative complement cascade with endothelial injury, neutrophil aggregation and margination. Our findings, after the correlation between lung biopsy and renal biopsy, imply that the top differential diagnosis in the lung biopsy should be microscopic polyangiitis when diffuse pulmonary hemorrhage and hemosiderosis are present in this ANCA-positive patient.

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Judy Tan ◽  
Fuad Spath ◽  
Rakesh Malhotra ◽  
Zaher Hamadeh ◽  
Anjali Acharya

We describe a case of a patient who developed microscopic polyangiitis (MPA) in the setting of exposure to silicone after breast implantation. A 57-year-old Hispanic woman was admitted to our hospital with complaints of fever, cough, and hemoptysis. She had undergone silicone breast implantation two years prior to presentation. She was diagnosed as having microscopic polyangiitis (MPA) based on acute progressive renal failure, hematuria, pulmonary hemorrhage, and positivity for myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA). A renal biopsy performed showed focal segmental necrotizing and crescentic glomerulonephritis. The patient received high dose steroids, cyclophosphamide, and plasmapheresis with remarkable clinical response. This case report raises the possibility of the development of MPA after silicone exposure from breast implantation.


2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Yasuyuki Nakada ◽  
Nobuo Tsuboi ◽  
Yasuto Takahashi ◽  
Hiraku Yoshida ◽  
Yoriko Hara ◽  
...  

We report a case in which antineutrophil cytoplasmic antibody- (ANCA-) associated glomerulonephritis and membranous glomerulopathy (MGN) were detected concurrently. The patient showed rapidly progressive renal deterioration. A renal biopsy showed crescentic glomerulonephritis, together with marked thickening and spike and bubbling formations in the glomerular basement membranes. Indirect immunofluorescence examination of the patient’s neutrophils showed a perinuclear pattern. Enzyme-linked immunosorbent assays revealed that the ANCA in this case did not target myeloperoxidase (MPO) or proteinase 3 (PR3) but bactericidal-/permeability-increasing protein, elastase, and lysosome. The relationship between these two etiologically distinct entities, MPO-/PR3-negative ANCA-associated glomerulonephritis and MGN, remains unclear.


MedAlliance ◽  
2020 ◽  
Vol 8 (3) ◽  
pp. 66-72

Disseminated lung diseases is a group of pathologies characterized by damage to the small airways, alveo-li, interstitium of the lungs united by a common symp-tom, dissemination in the lung tissue detected by X-ray. Pathologies included in the DLD group are numerous, and differential diagnosis between them is difficult, due to the similarity of symptoms and the absence of patho-gnomonic signs. Transbronchial cryobiopsy of the lungs is a relatively new technique, currently used in diagnos-tics, along with other methods of morphological verifi-cation in disseminated lung diseases. However, there are only sporadic publications on its use in Russia. The article describes the first experience of using transbronchial cry-obiopsy in patients with disseminated lung diseases after negative transbronchial biopsy. Objective. To obtain the first cli nical experience with the TBLC method. To assess the role and efficacy of TBLC in the diagnosis of DLDs. Ma-terials and methods. Patients with oncology in medical history or with previous tuberculosis were not included into the study; a total of 15 patients were included. The process was verified in 11 (73.33%) patients. Of these, sarcoidosis of the lungs was detected in 8 (72.73%) cases, pulmonary tuberculosis in 2 (18.18%), idiopathic intersti-tial pneumonia in 1 (9.09%). Complications were record-ed in 2 patients (13.33%): apical pneumothorax, which did not require pleural cavity drainage; and pulmonary hemorrhage requiring prolonged mechanical ventilation.Conclusions. Transbronchial cryobiopsy in patients with disseminated lung disease after unsuccessful transbron-chial lung biopsy appears to be a highly informative and relatively safe procedure. Cryobiopsy through a rigid bronchoscope tube appears to be safer. TBLC from the apex of the lungs, as well as in smokers, seems to be a more difficult task to verify, but this requires further studies


2021 ◽  
Vol 49 (2) ◽  
pp. 030006052199331
Author(s):  
Chun-Yang Zhang ◽  
Ran Miao ◽  
Wei Li ◽  
Hao-Yong Ning ◽  
Xiang-En Meng ◽  
...  

The relationship between antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and lung cancer remains unclear. A 66-year-old man presented with pulmonary nodules. Histological examination of a specimen from computed tomography-guided percutaneous transthoracic biopsy revealed adenocarcinoma. The patient was treated using cryoablation and systemic chemotherapy. Sixteen months later, the patient presented with fever, nasal inflammation, recurrent lung lesions, elevated serum creatinine levels, and high levels of ANCA. Histological examination of a specimen from ultrasound-guided percutaneous renal biopsy revealed pauci-immune necrotizing crescentic glomerulonephritis. The patient responded to treatment, but granulomatosis with polyangiitis recurred and he later died. This case highlights the possibility of sequential AAV with lung cancer. Although this is relatively rare, further research is needed to better understand the association or pathophysiological link between lung cancer and AAV.


2021 ◽  
Vol 14 (9) ◽  
pp. e243715
Author(s):  
Yoshiaki Zaizen ◽  
Masaki Tominaga ◽  
Shuji Nagata ◽  
Tomoaki Hoshino

A 55-year-old man with mental retardation and calcaneal tendon thickening was referred for a suspected genetic disease. His serum cholestanol was elevated and genetic analysis of his blood cells for CYP27A1 revealed a homozygous missense mutation. We diagnosed him with cerebrotendinous xanthomatosis (CTX). Chest radiography revealed diffuse micronodular and reticular opacities. Histological findings obtained from the transbronchial lung biopsy revealed foamy macrophages and multinucleate giant cells with marked lipid crystal clefts. Although there are few reports of pulmonary lesions in CTX, we concluded from the radiological and histopathological findings that the pulmonary lesions were indeed caused by the CTX. The patient was treated with chenodeoxycholic acid. His neurological findings and calcaneal tendon thickening were unchanged; however, his serum cholestanol and radiological abnormalities of the chest decreased.


2016 ◽  
Vol 6 (12) ◽  
pp. 1048-1050
Author(s):  
S Karki ◽  
D Karki

Tuberculosis of the oral cavity which is an uncommon occurrence can be primary or secondary. In the absence of active pulmonary tuberculosis, isolated tonsillar tuberculosis is rare. Herein, we report two cases of bilateral tonsillar tuberculosis who presented as recurrent sore throat for which tonsillectomy was done. No active primary pulmonary lesion was found in these cases. Histopathological examination revealed caseating epithelioid granulomas with Langhans giant cells. Ziehl Neelson stain for acid fast bacilli was positive in one case. Tonsillar tuberculosis, though a rare entity, should be considered in the clinical differential diagnosis of tonsillar lesions. Histopathological examination with Ziehl Neelson stain should be performed for definite diagnosis.


2018 ◽  
Vol 39 (04) ◽  
pp. 459-464 ◽  
Author(s):  
Alexandre Karras

AbstractMicroscopic polyangiitis (MPA) is one of the main clinical presentations of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Although the disease is defined by clinical and pathological criteria, the anti-myeloperoxidase (MPO) specificity of ANCAs is observed in almost 80% of MPA patients. The direct pathogenic role of anti-MPO antibodies has been proven in animal models, in which the disease was transmitted by transfer of anti-MPO antibodies or anti-MPO–specific splenocytes. The most frequently affected organs in this disease are the kidneys and the lungs. Necrotizing and crescentic glomerulonephritis can be revealed by rapidly progressive renal failure, but kidney injury can be more slowly progressive and lead to end-stage renal disease without major extrarenal manifestations. The most frequent pulmonary manifestation is diffuse alveolar hemorrhage, but some patients may present with chronic interstitial fibrosis leading to respiratory failure. General signs such as fever and weight loss, muscular and articular symptoms, peripheral neuropathy, and cutaneous involvement may also reveal the disease. Although the relapse rate is quite low after induction of remission, 5-year mortality is 25%, with even higher mortality rates in older patients (> 65 years old), or those with significant kidney dysfunction. Iatrogenic causes (particularly infections) are an important cause of deaths in these vulnerable patients. Future studies are warranted to determine the optimal maintenance immunosuppressive regimen to minimize side effects of immunosuppression.


2016 ◽  
Vol 49 (2) ◽  
pp. 112-116 ◽  
Author(s):  
Pedro Paulo Teixeira e Silva Torres ◽  
Marise Amaral Rebouças Moreira ◽  
Daniela Graner Schuwartz Tannus Silva ◽  
Roberta Rodrigues Monteiro da Gama ◽  
Denis Masashi Sugita ◽  
...  

Abstract Hypersensitivity pneumonitis is a diffuse interstitial and granulomatous lung disease caused by the inhalation of any one of a number of antigens. The objective of this study was to illustrate the spectrum of abnormalities in high-resolution computed tomography and histopathological findings related to hypersensitivity pneumonitis. We retrospectively evaluated patients who had been diagnosed with hypersensitivity pneumonitis (on the basis of clinical-radiological or clinical-radiological-pathological correlations) and had undergone lung biopsy. Hypersensitivity pneumonitis is clinically divided into acute, subacute, and chronic forms; high-resolution computed tomography findings correlate with the time of exposure; and the two occasionally overlap. In the subacute form, centrilobular micronodules, ground-glass opacities, and air trapping are characteristic high-resolution computed tomography findings, whereas histopathology shows lymphocytic inflammatory infiltrates, bronchiolitis, variable degrees of organizing pneumonia, and giant cells. In the chronic form, high-resolution computed tomography shows traction bronchiectasis, honeycombing, and lung fibrosis, the last also being seen in the biopsy sample. A definitive diagnosis of hypersensitivity pneumonitis can be made only through a multidisciplinary approach, by correlating clinical findings, exposure history, high-resolution computed tomography findings, and lung biopsy findings.


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