Pulmonary vasculitis and alveolar haemorrhage syndromes
Muhunthan Thillai, David R Moller, Keith C Meyer in Clinical Handbook of Interstitial Lung Disease, 2017
Alveolar haemorrhage syndromes are uniformly characterized by a disruption of the alveolar–capillary membrane and diffuse bleeding into the alveolar space. While haemoptysis often prompts an investigation of the possibility of DAH, this diagnosis should also be considered in any patient who presents with dyspnoea, hypoxaemia and a chest radiograph that demonstrates diffuse pulmonary infiltrates. Anaemia or haemoptysis are common but may not always be identified at the time of presentation. Diagnosis is usually made at the time of bronchoscopy when serial lung lavage reveals a progressive increase in blood content of sequentially retrieved bronchoalveolar lavage (BAL) aliquots, which verifies the presence of blood in the alveolar space. DAH is not a specific diagnosis but instead is a manifestation of an underlying disease process for which there is a broad differential diagnosis. Pulmonary histopathology can be used to classify the alveolar haemorrhage syndromes into one of three subgroups based on the histopathologic findings that are (a) pulmonary capillaritis, (b) bland pulmonary haemorrhage, or (c) diffuse alveolar damage (DAD) with haemorrhage (Table 20.1).
Procoagulant Activity and Lung Disease
Gary A. Levy, Edward H. Cole in Procoagulant Activity in Health and Disease, 2019
In spite of the multiple mechanisms protecting the lung from diffuse bleeding, such bleeding is well described in a variety of lung diseases in the absence of a systemic coagulopathy.118 The major entities associated with diffuse pulmonary hemorrhage are listed in Table 2. Accumulating evidence indicates that the common denominator for most of the disorders associated with diffuse hemorrhage is a diffuse capillaritis.119 Thus, in contrast to the diffuse alveolar injury (acute or chronic) leading to an enhanced procoagulant milieu, limited but persistent fibrin deposition, and pulmonary fibrosis, most of the conditions listed in Table 2 are associated with diffuse small vessel inflammation resulting in microvascular leakage that over-whelms the defenses of the otherwise normal alveolus. Moreover, the initiation of capillaritis for the most part appears to be due to antibody (and presumably complement) deposition along basement membranes rather than direct injury or cytokine stimulation of endothelial or epithelial cells. Although little critical information is available, it is suggestive that differences in the extent of hemorrhage between ARDS and the diffuse alveolar hemorrhage syndromes (as well as the course of the fibrin deposits) may reflect differences in the relative importance of proinflammatory cellular and humoral mechanisms operative in these two syndromes, respectively.
Vasculitis
Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley in Diagnostic Pulmonary Pathology, 2008
Histologic features of the collagen vascular disease-associated pulmonary disease include alveolar capillaritis, septal necrosis, alveolar hemorrhage, and hemosiderin-filled macrophages (Figs. 7 and 8). Occasionally, hemorrhage without capillaritis may be seen. The pneumonitis in SLE predominantly involves the alveolar septa, however, occasionally it may be secondary to vasculitis (Fig. 9) (35). In some patients, necrotizing arteritis with infiltration of the arterial wall by mononuclear cells may be present. In a small percentage of patients with SLE (14%), pulmonary hypertension secondary to pulmonary arteriopathy with plexiform lesions may be seen (39, 40). These patients also reportedly have a higher prevalence of Raynaud’s phenomenon and antiphospholipid antibodies (41).
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.