Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A variety of anti-rheumatic drugs including biologics are currently used to treat rheumatoid arthritis (RA). These drugs, as well as RA itself, can cause kidney injury. RA may trigger mesangial proliferative glomeru- lonephritis (MesPGN), membranous nephropathy (N), thin basement membrane disease, and renal amyloi- dosis. As for anti-rheumatic drugs, non-steroidal anti-inflammatory drugs (NSAID) increase serum Cr lev- els due to a reduction of glomerular circulation, particularly in the presence of dehydration. Among disease- modifying anti-rheumatic drugs (DMARD), methotrexate as an anchor drug for RA rarely causes tubular ob- struction as a result of its crystallization, and bucillamine occasionally elicits IN. Calcineurin inhibitors induce vasoconstriction of the afferent arteries. Recently developed anti-rheumatic drugs, biologics, include biological inhibitors of TNF-a, IL6, and CD80/26. These can generally induce the remission of RA, while they have been reported to albeit uncom- monly trigger autoimmune renal disorders (AIRD). A recent meta-analysis identified a total of 29 cases with biologics-induced AIRD, 62% of who manifested AIRD within 12 months after treatment with biologics. AIRD cases were classified into 3 different groups: isolated autoimmune renal disorders (IARD, n =13), glo- merulonephritis with systemic vasculitis (GNSV, n= 12), and glomerulonephritis with lupus-like syndrome (GNLS, n=4). The IARD cases had 4 MesPGN, 4 MN, and 2 crescentic GN, while the GNSV cases had 8 crescentic GN and 3 purpura GN, and the GNLS cases had all MesPGN. To detect these renal disorders early in RA patients, urinalysis and serum Cr measurement should be peri- odically performed. New urinary biomarkers (L-FABP and Ngal) may be more sensitive for kidney injury. Notably, in RA patients receiving biologics, ANA, anti-dsDNA, and ANCA should also be tested at the base- line and regular intervals. [Review].
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PMID:[Biologics-Induced Kidney Injury -with Special Attention to Anti-Rheumatic Drugs -]. 3069 31

Systemic lupus erythematosus (SLE) is known to involve the gastrointestinal tract, but gallbladder involvement is rare. The authors report the case of a 26-year-old postpartum female who presented with acute right upper quadrant abdominal pain and was diagnosed with acute acalculous cholecystitis (AAC). In the presence of concomitant features of nephritis, pericardial effusion, anaemia and positive ANA titre, the diagnosis of SLE was confirmed during hospitalisation. Histopathological analysis of the gall bladder revealed evidence of vasculitis. Although rare, AAC can be the first presentation of patients diagnosed with SLE. Prompt diagnosis and management results in a better patient outcome.
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PMID:Acute Acalculous Cholecystitis as a Presenting Manifestation in Systemic Lupus Erythematosus. 3075 73

The various forms of Alexander disease (AD) have been linked to heterozygous point mutations in the coding region of the human Glial Fibrillary Acidic Protein (GFAP) gene. The aim of this study was to confirm and characterise an adult variant of AD based on the presence of Rosenthal fibres, which were identified at brain autopsy. We performed histological and immunohistochemical studies and mutation screening by cycle sequencing of exons 1, 4, 6, and 8. A heterozygous D128N GFAP mutation, previously described in three other cases of adult-onset AD (AOAD), was genetically confirmed. The mutation was seemingly sporadic. Symptoms of the female, 65-year-old patient started with occasionally asymmetric motor impairment and concluded, 23 months later, with a lack of spontaneous movement in all four limbs, reduced consciousness, an acute respiratory problem, and eventually lethal exitus. The most striking characteristics were a cerebellar syndrome with subsequent clinical signs due to brainstem and spinal cord involvement. The final diagnosis was based on a complete autopsy, detection of Rosenthal fibres, GFAP, vimentin, alpha B-crystallin, ubiquitin, hsp27, neurofilament, and synaptophysin, and the identification of the corresponding GFAP gene mutation. Blood analyses were positive for ANA and rheumatoid factor. In conclusion, this work describes sporadic, rapidly advancing AOAD in a female patient and links it with other published cases with the same mutation. Reflections are provided on the influence of vasculitis and ANA in AD as well as the presence of Rosenthal fibres in the neurohypophysis.
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PMID:Adult-onset Alexander disease with a heterozygous D128N GFAP mutation: a pathological study. 3094 95

Crescentic glomerulonephritis (GN) without immune reactants or deposits (referred to as pauci-immune) is typically characterized by the presence of anti-neutrophilic cytoplasmic antibodies (ANCA). While ANCA-negative patients might be expected to have a more benign course, they often have poor renal outcomes, especially without treatment with steroids and immune-modulating therapy. Pauci-immune crescentic GN can also co-exist with other autoimmune conditions, including rheumatoid arthritis (RA). Here, we describe an ANCA-negative patient with RA who developed dialysis-requiring acute kidney injury (AKI) with findings consistent with focal pauci-immune crescentic GN (i.e., no IgG or immune complex on kidney biopsy). Coexistent conditions included Klebsiella sepsis attributed to pneumonia, rhabdomyolysis, leukocytoclastic immune-mediated skin vasculitis, and positive ANA. He had spontaneous improvement in renal function without immunosuppressive therapy. This crescentic GN was not associated with poor renal outcome as AKI resolved with supportive care and treatment of his infection. The AKI was likely multifactorial with co-existing acute tubular necrosis in the setting of Kebsiella sepsis and rhabdomyolysis, and the crescentic GN was felt more likely to be related to the infection rather than having a primary role. This case highlights the importance of viewing crescentic GN in the context of the clinical picture, as it may not always lead to the need of aggressive immune suppression and is not a universally poor prognostic kidney finding. However, these cases do warrant close follow-up as our patient had recurrent RA disease manifestations over the next 2 years that eventually led to his death from severe pulmonary hypertension.
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PMID:Significance of Crescentic Glomeruli in Acute Kidney Injury with Rheumatoid Arthritis. 3119 27

ANCA (anti-neutrophil cytoplasmic antibody) vasculitides are systemic autoimmune diseases in which anti-neutrophilic cytoplasmic antibodies activate primed neutrophils, thereby generating an inflammatory cascade resulting in the damage of small sized blood vessels in various organs of the body, including the heart. Pleuropericardial involvement is underrecognized as a complication of ANCA vasculitis and is highlighted in this case report of a 51-year-old male who presented with an initial symptomatic presentation of pleuropericardial effusion progressing to pericardial tamponade in the setting of a later renal biopsy proven pauci-immune crescentic glomerulonephritis with high ANA titres along with positive cANCA (cytoplasmic ANCA) and PR3 (proteinase 3) antibodies. He was found to have acute renal failure which progressively got better with cyclophosphamide.
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PMID:Pericardial Tamponade: An Uncommon Clinical Presentation in cANCA Related Vasculitis and Glomerulonephritis in Association with Very High Titres of ANA. 3153 8


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