Noninfectious Pulmonary Manifestations of Renal Disease In Children
Lourdes R. Laraya-Cuasay, Walter T. Hughes in Interstitial Lung Diseases in Children, 2019
Henoch-Schonlein purpura (HSP) or anaphylactoid purpura is a leukoclastic vasculitis of the small blood vessels of the skin, kidney, gastrointestinal tract, lungs, liver, heart, and spleen. There is a nonthrombocytopenic purpuric rash on the lower extremities and buttocks with joint swelling, diarrhea, hematochezia associated with colicky abdominal pain, and nephritis. It occurs 1 to 3 weeks after an upper respiratory infection. Rarely, pulmonary hemorrhage, perihilar patchy infiltrates, reticulonodular changes and pneumonic processes may be present.24 Rapidly progressive glomerulonephritis may follow. If chronic renal failure results, signs of uremic pneumonitis appear. IgA deposits in dermal capillaries or renal biopsy support the diagnosis of HSP.
Nephrology
Fazal-I-Akbar Danish in Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
Anuria:1 Shock → ATN → anuria.2 Rapidly progressive glomerulonephritis.3 Total renal artery/vein occlusion.4 Total urinary tract obstruction.
A unique presentation of a rare disease: biopsy proven systemic lupus erythematosus and microscopic polyangitis: an overlap syndrome
Published in Journal of Community Hospital Internal Medicine Perspectives, 2019
Diana Meyler, Abilio Arrascaeta-Llanes, Ravi Gupta, Sanjay Godhwani
Systemic lupus erythematosus and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis overlap syndrome is a rare disease originally described in 2008. With only a few reported cases in the literature, this case report provides further insight into this rare presentation with renal and pulmonary involvement, and considerably high mortality if not promptly recognized in a timely fashion. A recent literature review by Jarott and collegues found only 8 cases of SLE/ANCA overlap syndrome with renal involvement [7]. Most of the patients in the review were of female sex, and this case report is unique due to its manifestation in a male patient, which, anecdotally, indicates poor prognosis. These diseases are typically seen in young to middle aged females, and given the rarity of this case, biopsy findings were confirmed by two pathologists from separate institutions. Up to 70% of patients diagnosed with overlap syndrome have no previous history of SLE or vasculitis [5]. The most common symptoms at presentation are arthritis, serositis, cytopenias and pulmonary hemorrhage [6]. Rapidly progressive glomerulonephritis, as exhibited in our patient, is an almost universal feature.
Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease
Published in Expert Review of Clinical Immunology, 2020
Alvise Berti, Sara Boukhlal, Matthieu Groh, Divi Cornec
Constitutional symptoms are present in half of the cases at relapse. Mononeuritis multiplex is a cause of severe relapse and occurs in as many as 36% of the patients [11]. Renal manifestations are less frequent than the neurological ones and occur in an average 9 years after diagnosis [9]. In a study, the most common presentations were rapidly progressive glomerulonephritis and chronic kidney disease. Symptoms were hematuria, proteinuria, and renal dysfunction in all patients and dialysis was initially required in half of the patients. Most renal biopsies showed pauci-immune segmental necrotizing glomerulonephritis. In the same cohort, only 14% of patients returned to normal renal function during the follow-up. Gastrointestinal involvement, which is a severity criterion, is rarely reported during follow-up in EGPA patients [11]. Heart involvement is a main cause of death of patients with EGPA and is responsible for a third of deaths observed during the follow-up of different cohorts, by myocardial infarction, cardiac insufficiency, or arrhythmia [3]. Cardiac events are related to specific heart involvement of EGPA and cardiovascular complications and occur in approximately 8–20% of EGPA patients [3,15]. They affect the prognosis with a 5-year survival rate of 91% for patients without versus 78% in patients with cardiac involvement [3].
Relationship between autoantibodies to erythropoietin receptor and renal outcome in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis
Published in Biomarkers, 2020
Trang Thi Thu Tran, Akinori Hara, Kiyoki Kitagawa, Shinji Kitajima, Tadashi Toyama, Yasunori Iwata, Norihiko Sakai, Miho Shimizu, Shuichi Kaneko, Kengo Furuichi, Takashi Wada
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a heterogeneous and multisystem disorder of unknown aetiology characterized by inflammation and necrosis of small and medium-sized blood vessels (Ntatsaki et al.2014). The major clinicopathologic categories of AAV are microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA) (Ntatsaki et al.2014). During the disease course, renal involvement, characteristically rapidly progressive glomerulonephritis, is a major feature (Guillevin and Terrier 2014). Along with impaired renal function, the major abnormal laboratory findings are typically non-specific inflammatory markers, including elevated C-reactive protein (CRP), elevated white blood cell count and low haemoglobin levels (Guillevin and Terrier 2014).
Related Knowledge Centers
- Acute Kidney Injury
- Goodpasture Syndrome
- Granulomatosis With Polyangiitis
- Hematuria
- Syndrome
- Kidney
- Glomerular Filtration Rate
- Lupus
- Idiopathic Disease
- Scar