Delayed presentation of atypical HUS after COVID-19 with diffuse alveolar haemorrhage is uncommon and can be life threatening.

ANCA-associated Vasculitides (AAV) are characterized by small vessel necrotizing inflammation and can present with multisystem organ involvement, including organ/life threatening manifestations of rapidly progressive glomerulonephritis and diffuse alveolar haemorrhage, where immediate and aggressive intervention is needed to prevent further organ damage. Although, the rationale of plasma exchange (PLEX) in AAV is strong, through removing the pathogenic ANCAs; target either myeloperoxidase (MPO) or proteinase 3 (PR3), and other inflammatory molecules, especially in the initiation when the immunosuppressive treatment is no sufficient to prevent the organ damage, overall impact on patient outcomes is not well-established, while the risk of infections seems to be higher in the PLEX-treated patients. A comprehensive overview of the challenges and uncertainties surrounding the use of PLEX in the management of AAV will be reviewed, providing the current practice recommendations guiding treatment decisions.

UNASSIGNED: Diffuse alveolar haemorrhage (DAH) is considered a rare condition in children. There is no consensus on the management of DAH syndromes in Africa or other low- and middle-income countries. In this brief report, the clinical characteristics, management and outcomes of children treated for DAH in the Chris Hani Baragwanath Academic Hospital paediatric pulmonology unit in Johannesburg, South Africa are described. Fifteen children were included in this case series, of whom 11 (73.3%) presented with severe microcytic anaemia. Of the 11 children who had bronchoalveolar lavage, 9 (81.8%; 60.0% of the total) had haemosiderin-laden macrophages on microscopy. Only 5 children had a lung biopsy, of whom 3 (60.0%) had capillaritis. All the children were started on oral prednisone at presentation, and 11 (73.3%) received additional complementary treatment. Nine children (60.0%) had normal haemoglobin levels 1 year after initiation of treatment. Our series supports previous reports that DAH is uncommon in children. A large proportion of our patients responded well to treatment despite some resource limitations.
UNASSIGNED: The study provides additional data on children presenting with diffuse alveolar haemorrhage in a South African tertiary hospital.
UNASSIGNED: There is a need for South African pulmonologists to come together and conduct a national audit of these patients in different hospitals to determine the incidence in our country, as well as to inform a management plan in the presence or absence of specialised tests.

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease with enhanced NETosis and impaired degradation of neutrophil extracellular traps (NETs). Galectin-3 is a β-galactoside binding protein and is associated with neutrophil functions as well as involved in mediating autoimmune disorders. In this study, we plan to examine the associations of galectin-3 with the pathogenesis of SLE and NETosis. Galectin-3 expression levels were determined in peripheral blood mononuclear cells (PBMCs) of SLE patients for the association with lupus nephritis (LN) or correlation of SLE disease activity index 2000 (SLEDAI-2K). NETosis was observed in human normal and SLE and murine galectin-3 knockout (Gal-3 KO) neutrophils. Gal-3 KO and wild-type (WT) mice induced by pristane were used to evaluate disease signs, including diffuse alveolar haemorrhage (DAH), LN, proteinuria, anti-ribonucleoprotein (RNP) antibody, citrullinated histone 3 (CitH3) levels, and NETosis. Galectin-3 levels are higher in PBMCs of SLE patients compared with normal donors and positively correlated with LN or SLEDAI-2K. Gal-3 KO mice have higher percent survival and lower DAH, LN proteinuria, and anti-RNP antibody levels than WT mice induced by pristane. NETosis and citH3 levels are reduced in Gal-3 KO neutrophils. Furthermore, galectin-3 resides in NETs while human neutrophils undergo NETosis. Galectin-3-associated immune complex deposition can be observed in NETs from spontaneously NETotic cells of SLE patients. In this study, we provide clinical relevance of galectin-3 to the lupus phenotypes and the underlying mechanisms of galectin-3-mediated NETosis for developing novel therapeutic strategies targeting galectin-3 for SLE.

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Pulmonary-renal syndrome (PRS) is defined as a combination of diffuse alveolar haemorrhage and glomerulonephritis. An 18-year-old woman visited our hospital with a 2-day history of fever, dyspnoea, and leg edema. Laboratory investigations revealed an elevated inflammatory reaction, increased serum creatinine levels, and normocytic anaemia. Additionally, the anti-streptolysin-O titre was positive, and complement component-3 levels were decreased. Urinalysis revealed proteinuria and hematuria. Bronchoalveolar lavage aliquots were progressively more hemorrhagic. These findings supported a diagnosis of PRS secondary to streptococcal infection. The patient was treated with high-dose methylprednisolone and antibiotics. After 4 days of treatment, her respiratory symptoms and serum creatinine levels improved. Steroid tapering was performed over 15 days. The findings in this case indicate that streptococcal infection is a potential cause of PRS, and that short-term steroid therapy is an effective treatment.

Haemophagocytic syndrome, diffuse alveolar haemorrhage, catastrophic antiphospholipid syndrome and various types of thrombotic microangiopathies are rare conditions with significant morbidity and mortality. A common feature is late diagnosis, which can affect the success of treatment. The aim of this review article is to summarize the basic diagnostic and therapeutic steps of the present subpopulation of critically ill patients.

BACKGROUND: The medical and social interest in the SARS-CoV-2 infection is currently high. This infection can, in severe cases, be accompanied by a series of complications, such as thromboembolic disease or pulmonary parenchymal haemorrhage.
METHODS: The paper presents two rare cases of massive intrathoracic haemorrhage caused by pulmonary parenchymal haemorrhage and exacerbated by full anticoagulant treatment of thromboembolic disease.
RESULTS: In both cases, the haemorrhage originated in the left lower lobe and was life threatening, requiring urgent anatomical lung resection - left lower lobectomy.
CONCLUSIONS: The combinaion of anticoagulant therapy and thromboembolic events related to COVID-19 can cause, in rare cases, massive pulmonary haemorrhage. This rare complication proved lethal in one out of two of the cases described in this paper. An imminent and adequate reaction is necessary when the first signs of haemorrhage appear.

Idiopathic pulmonary hemosiderosis (IPH) is a rare cause of diffuse alveolar hemorrhage (DAH). Glucocorticosteroids (CS) represent the first line therapy for IPH. Although most patients respond to CS, steroid refractoriness is seen in an appreciable minority of patients. This paper reviews and evaluates the efficacy and safety profile of liposomal dexamethasone 21-palmitate (liposteroid) for the treatment of IPH. Medline, Embase and Web of Science biomedical databases were searched between 1980 and 2020 to identify papers describing patients with IPH, who were treated with liposteroid. A total of five articles were identified. Four in the form of case reports and one as a case series. A total of 12 pediatric patients (5 boys, 7 girls) were identified, with a median age of 2.3 years (range 0.5-8.6). Liposteroid therapy in intravenous doses ranging 0.06-0.1 mg/kg body weight appeared to be effective for both remission induction therapy, and maintenance therapy. There was no mortality among patients treated with liposteroid, either in the acute phase or during follow-up. The majority of patients for whom long-term follow-up data were available, were cured or in disease remission. No acute adverse events were reported, and long-term side effects were minimal and tolerable. Liposteroid represents a potential alternative or supplement to conventional CS therapy, as it appears to be more efficacious and associated with fewer side effects. Larger prospective, controlled trials are necessary to be able to define more precisely the therapeutic role of liposteroid in IPH.

Diffuse alveolar haemorrhage (DAH) after a generalized tonic-clonic seizure is a rarely described illness likely involving physical disruption of alveolar-capillary interface similar to the mechanism of neurogenic pulmonary oedema. Based on our review of the English literature, only 11 cases have been reported to date. Recognition of this sparsely reported entity is important for optimal management, including avoidance of medications that have been implicated in causing DAH. Current experience with two additional patients with post-ictal DAH extends the reported experience to 13 and summarizes what is, to our knowledge, the entire experience of such patients reported in the English literature. This case report highlights the key aspects of clinical presentation, radiological and pathological findings, clinical course and management implications with the goal of enhancing awareness of this condition by respiratory clinicians.