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Arteropathies, Microcirculation and Vasculitis
Published in Mary N. Sheppard, Practical Cardiovascular Pathology, 2022
Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disorder that affects multiple organs. Cardiovascular disease is common, with accelerated atherosclerosis, intravascular thrombosis associated with antiphospholipid syndrome, and hypertensive cardiomegaly. Vasculitis with superimposed thrombosis may occur to the heart and brain with infarction. Mesenteric ischaemia may be caused by vasculitis, thrombosis and accelerated atherosclerosis and results in lethal intestinal infarction. Isolated capillaritis can also occur (Fig. 11.8).
Pulmonary vasculitis and alveolar haemorrhage syndromes
Published in Muhunthan Thillai, David R Moller, Keith C Meyer, Clinical Handbook of Interstitial Lung Disease, 2017
Rebecca C Keith, Stephen K Frankel
Similar to the pathologic findings of capillaritis, bland pulmonary haemorrhage is characterized by RBC-filled airspaces, the presence of haemosiderin-laden macrophages, oedema of the alveolar septal wall, and reactive type II hyperplasia (12). Unlike capillaritis, there is no evidence of inflammatory cell infiltration, necrosis or damage to the vessel wall (12,13). Partially treated or resolving capillaritis may have a similar appearance to bland haemorrhage, confounding the diagnosis (13).
Diagnosis of lung rejection and infection: A pathologist’s perspective
Published in Wickii T. Vigneswaran, Edward R. Garrity, John A. Odell, LUNG Transplantation, 2016
Vijayalakshmi Ananthanarayanan, Aliya N. Husain
The ISHLT came to the conclusion that if a reason to suspect AMR on the basis of clinical, morphologic, or serologic grounds exists, immunohistochemistry studies for byproducts of complement activation (C3d and C4d) should be performed. Only strong diffuse endothelial staining should be considered positive (Figure 32.5B). That being said, it is important to remember that there are caveats to interpreting C4d immunostaining in isolation without an appropriate clinical context. C4d immunostaining can also be seen with other causes of complement activation such as infection or DAD (Figure 32.6A).14,15 At our institution we routinely perform C4d immunostaining on all heart and lung allograft biopsy specimens. Unlike the situation in the heart biopsies, C4d immunostaining of samples from lung transplant recipients (performed prospectively on all biopsy specimens from 100 consecutive patients) was not positive in any case. Furthermore, none of the specimens showed capillaritis or capillary injury. However, almost all the C4d stains demonstrated background staining, especially in the elastic fibers (Figure 32.6B).
Diagnosing retinal vasculitis and its implications for treatment
Published in Expert Review of Ophthalmology, 2019
Nesrine Abroug, Sourour Zina, Molka Khairallah, Imen Ksiaa, Melek Kechida, Hager Ben Amor, Sana Khochtali, Moncef Khairallah
Retinal vasculitis (RV), also called retinal perivasculitis, is a sight-threatening inflammatory condition involving retinal blood vessels. It is characterized by focal or diffuse perivascular cuffing or sheating on clinical examination and by retinal vascular staining and leakage on fluorescein angiography (FA). RV most commonly affects retinal veins (retinal periphlebitis) than arteries (retinal periarteritis). Retinal capillary involvement (capillaritis), detectable only by FA, may accompany periphlebitis or periarteritis, or occur as isolated finding. RV may be classified as primary (idiopathic) or secondary, caused by local or systemic inflammatory or infectious disorders. In any patient with RV, a targeted and tailored diagnostic approach is recommended, emphasizing relevant clinical clues from the history, physical examination, and ocular assessment, and using a limited number of oriented laboratory investigations. The exclusion of underlying neoplastic condition and then discrimination between infectious and noninfectious etiologies are essential for appropriate management and favorable outcome. Clinician also should be aware of sight-threatening complications of RV that may include macular edema, macular ischemia, other macular complications, and new vessels leading to vitreous hemorrhage, tractional retinal detachment, or neovascular glaucoma [1,2].
Refractory isolated pulmonary capillaritis rescued by rituximab
Published in Modern Rheumatology Case Reports, 2018
Stuart Clarence Wiber, Shahin Jamal, Kun Huang
Over the subsequent year, despite complete exposure avoidance, he had several episodes of worsening dyspnoea which required increase in prednisone dose. An open lung biopsy in 2015 revealed healed pulmonary capillaritis (Figure 1). Specifically, some vessels showed thickened media and intima with surrounding haemosiderin-laden macrophages and abundant neutrophils (Figure 1). Staining for immune deposits was negative. There were no signs of hypersensitivity pneumonitis on pathology. He never had renal involvement. In the absence of clinical signs or serologies to suggest systemic vasculitis, he was diagnosed with isolated pulmonary capillaritis (IPC). He was started on oral cyclophosphamide 125 mg daily in October of 2015 for a year with total exposure of 30 g. His disease remained in remission. In November 2016, he was switched to azathioprine 150 mg daily for maintenance therapy.
Sarcoid-like Uveitis with or without Tubulointerstitial Nephritis during COVID-19
Published in Ocular Immunology and Inflammation, 2023
Hilal Eser-Ozturk, Tugba Izci Duran, Ozlem Aydog, Yuksel Sullu
A 14-year-old female patient was admitted to the outpatient clinic with complaints of redness and blurred vision in the left eye. She applied with complaints of weakness, chest pain, and flank pain two weeks after her contact with her SARS-CoV-2 positive father. A kidney biopsy was performed after sCr was detected as 1.8 mg/dL. Interstitial fibrosis and tubular atrophy < ~ 5% in kidney biopsy, Hyaline casts including shed epithelium in tubules, edema between tubules, lymphoplasmocytic inflammation, and tubulitis were observed. It was noted that neutrophils and eosinophils were heavily involved in inflammation. Lymphocytic capillaritis was observed. A diagnosis of acute TIN was made. She was using 1 mg/kg/day oral methylprednisolone due to TIN. On ophthalmic examination, visual acuities were 20/20, OD, and 20/30, OS. +2 cells were seen in the anterior chamber of the left eye on slit-lamp biomicroscopy. Fundus examination of the left eye revealed hyperemia and swelling of the optic disc. Right eye examination was within normal limits. Topical prednisolone acetate and cycloplegic treatment were started. Systemic CS treatment was discontinued after renal functions regressed to normal. After systemic CS was discontinued, the patient presented again with the complaint of blurred vision in the right eye. Visual acuities were 20/125, OD, and 20/20, OS. She had 3+ cells, granulomatous KPs, and Koeppe nodules in the right eye and +1 cells in the left eye. Fundus examination showed bilateral optic nerve swelling. FA revealed staining of the optic disc and peripheral retinal leakage in both eyes. In ICGA, hypo fluorescent dots were seen that were present in the middle phase and disappeared in the late phase. In the examinations, ACE and lysozyme levels were normal, PPD anergic, QFT, viral and autoimmune markers were negative, and thorax CT was normal. Oral methylprednisolone 1 mg/kg/day and MTX 20 mg/week were started to the patient. In the control examination performed three months later, kidney functions were normal, and the patient’s visual acuity was 20/20 in both eyes. However, 2+ cells and granulomatous KPs were seen in the slit lamp examination of both eyes. Topical CS treatment was restarted, and ADA treatment was planned.