- Primary membranous nephropathy presenting with crescentic glomerulonephritis 25 years after initial presentation: A case report . [Journal Article]
- CNClin Nephrol 2018 Jun 22
- Membranous nephropathy (MN) is a common cause of nephrotic syndrome. Rarely, it can present with rapidly-progressive renal failure and hematuria. While this may be due to lupus nephritis, superimpose...
Membranous nephropathy (MN) is a common cause of nephrotic syndrome. Rarely, it can present with rapidly-progressive renal failure and hematuria. While this may be due to lupus nephritis, superimposed anti-glomerular basement membrane (GBM) disease, or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, there have been rare reports of anti-GBM - and ANCA-negative crescentic glomerulonephritis presenting in primary membranous nephropathy (pMN). We present the case of a patient with long-standing pMN who developed acute deterioration of renal function and was found to have a flare of MN along with crescentic glomerulonephritis (GN) despite a negative serum ANCA, anti-GBM, and antinuclear antibody (ANA) work-up. He was started on dialysis and immunosuppressive therapy, and eventually recovered enough renal function to become dialysis-independent. A brief review of available literature suggests that crescentic GN presenting with pMN is a rare but established entity. Much more rarely has it been reported to occur in patients with previously-diagnosed pMN. In these contexts, crescentic GN may be occurring as the most severe manifestation of pMN rather than as a separate entity. Immunosuppressive therapy is often given, however, prognosis is guarded as half of patients will have worsening renal function and a quarter will develop end-stage renal disease. .
- Stevens-Johnsons syndrome or drug-induced lupus - a clinical dilemma: A case report and review of the literature. [Journal Article]
- BRBiomed Rep 2018; 9(1):37-41
- Tumor necrosis factor inhibitors are the first biological agents used in the treatment of rheumatoid arthritis (RA) to have yielded satisfactory results in terms of clinical improvement and radiologi...
Tumor necrosis factor inhibitors are the first biological agents used in the treatment of rheumatoid arthritis (RA) to have yielded satisfactory results in terms of clinical improvement and radiologic progression, but they are also associated with the possibility of occurrence of a number of autoimmune systemic events [drug-induced lupus (DIL), vasculitis, sarcoidosis] and localized adverse events [uveitis, psoriasis, interstitial lung disease, erythema multiforme including the major form Stevens-Johnson syndrome (SJS)]. During treatment with TNF inhibitors, many patients develop positivity for antinuclear, antihistone and anti-double stranded DNA antibodies, though only a minority of patients will develop clinical manifestations and approximately less than 1% will fulfill the classification criteria for systemic lupus erythematosus. Mucocutaneous manifestations are the most frequent manifestations of DIL following treatment with TNF inhibitors, and can be severe and occasionally difficult to differentiate from erythema multiforme/SJS. Stopping the causative drug (the TNF inhibitor) and general supportive measures are usually sufficient in mild forms, but in moderate to severe forms, systemic glucocorticoids and sometimes immunosuppressive drugs are required. The present report presents the case of a patient with rheumatoid arthritis who developed severe recurrent cutaneous reactions and positive autoantibodies during TNF inhibitor treatment, with difficulties in differential diagnosis and treatment. A review of the literature is also presented.
- Ocular manifestations of emerging arboviruses: Dengue fever, Chikungunya, Zika virus, West Nile virus, and yellow fever. [Review]
- JFJ Fr Ophtalmol 2018 Jun 18
- Arboviruses are viral diseases transmitted by mosquitoes and tick bites. They are a major cause of morbidity and sometimes mortality. Their expansion is constant and due in part to climate change and...
Arboviruses are viral diseases transmitted by mosquitoes and tick bites. They are a major cause of morbidity and sometimes mortality. Their expansion is constant and due in part to climate change and globalization. Mostly found in tropical regions, arboviruses are sometimes the source of epidemics in Europe. Recently, the Chikungunya virus and the Zika virus were responsible for very large epidemics impacting populations that had never been in contact with those viruses. There are currently no effective antiviral treatments or vaccines. Ocular manifestations due to those infections are thus more frequent and increasingly better described. They are sometimes, as with Zika, complicated by a congenital ocular syndrome. The goal of this review is to describe the ophthalmological manifestations of Dengue fever, Chikungunya virus, Zika virus, West Nile virus, and yellow fever.
- Consequences of cathepsin C inactivation on membrane exposure of proteinase 3, the target antigen in autoimmune vasculitis. [Journal Article]
- JBJ Biol Chem 2018 Jun 20
- Membrane-bound proteinase 3 (PR3m) is the main target antigen of anti-neutrophil cytoplasmic autoantibodies (ANCA) in granulomatosis with polyangiitis (GPA). Binding of ANCA to PR3m triggers neutroph...
Membrane-bound proteinase 3 (PR3m) is the main target antigen of anti-neutrophil cytoplasmic autoantibodies (ANCA) in granulomatosis with polyangiitis (GPA). Binding of ANCA to PR3m triggers neutrophil activation with the secretion of enzymatically active proteases, thereby contributing to vascular damage. PR3 and related proteases are activated from proforms by the lysosomal cysteine protease cathepsin C (CatC) during neutrophil maturation. We hypothesized that pharmacological inhibition of CatC provides an effective measure to reduce PR3m and has therefore implications as a novel therapeutic approach in GPA. We first studied neutrophilic PR3 from 24 patients with Papillon-Lefèvre syndrome (PLS), a genetic form of CatC deficiency. PLS neutrophil lysates showed a largely reduced, but still detectable (0.5-4%) PR3 activity when compared to healthy control cells. Despite extremely low levels of cellular PR3, the amount of constitutive PR3m expressed on the surface of quiescent neutrophils, and the typical bimodal membrane distribution pattern, was similar to what was observed in healthy neutrophils. However, following cell activation, there was no significant increase in the total amount of PR3m on PLS neutrophils, whereas the total amount of PR3m on healthy neutrophils was significantly increased. We then explored the effect of pharmacological CatC inhibition on PR3 expression in normal neutrophils using a potent cell permeable CatC inhibitor and a CD34+ hematopoietic stem cell model. Human CD34+ hematopoietic stem cells were treated with the inhibitor during neutrophil differentiation over 10 days. We observed strong reductions in PR3m, cellular PR3 protein and proteolytic PR3 activity whereas neutrophil differentiation was not compromised.
- Cogan's syndrome with pyoderma gangrenosum: management of two uncommon disorders with aggressive presentation in a patient. [Journal Article]
- BCBMJ Case Rep 2018 Jun 19; 2018
- Pyoderma gangrenosum (PG) coexisting with Cogan's syndrome (CS) is uncommon, although cutaneous manifestations are known to develop in CS. A middle-aged white female patient had chronic relapsing PG ...
Pyoderma gangrenosum (PG) coexisting with Cogan's syndrome (CS) is uncommon, although cutaneous manifestations are known to develop in CS. A middle-aged white female patient had chronic relapsing PG requiring ciclosporin and prednisolone. Despite receiving optimal doses of ciclosporin and prednisolone, she developed acute vestibulo-auditory symptoms as a result of CS. Ciclosporin was switched to methotrexate and prednisolone was increased. However, she continued to develop acute scleritis, requiring methylprednisolone pulses, and still had further flares of PG. Her methotrexate was held off when she developed severe pneumonia and she then received a trial of intravenous immunoglobulins (IVIG) for her recurrent leg ulcers. Unfortunately, she failed to respond to IVIG. Her ulcers eventually responded to six doses of monthly intravenous cyclophosphamide induction. Although CS is not an antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, we used pulse cyclophosphamide, based on the experience of cyclophosphamide efficacy in severe ANCA-associated vasculitis (AAV). Following induction, both diseases currently remain under control with azathioprine as maintenance treatment.
- Autoantibodies to Cytosolic 5'-Nucleotidase 1A in Primary Sjögren's Syndrome and Systemic Lupus Erythematosus. [Journal Article]
- FIFront Immunol 2018; 9:1200
- CONCLUSIONS: Our results confirm the relatively frequent occurrence of anti-cN-1A in pSS and SLE patients and the variation in anti-cN-1A reactivity between independent groups of these patients. The explanation for this variation remains elusive. The correlation between anti-cN-1A reactivity and polyautoimmunity should be evaluated in future studies. We conclude that anti-cN-1A should be classified as a myositis-associated-, not as a myositis-specific-autoantibody based on its frequent presence in SLE and pSS.
- Long-term outcome of coronary artery dilatation in Kawasaki disease. [Journal Article]
- APAnn Pediatr Cardiol 2018 May-Aug; 11(2):125-129
- CONCLUSIONS: The majority (77.4%) of small- and medium-sized dilatations regress within 2 years, but giant aneurysms tend to persist. The outcome of coronary dilatation is determined by the diameter and not by the location. Regression rate is faster in smaller dilatations. Left main coronary artery is the most frequent location for dilatation.
- Antinuclear antibodies in scrub typhus: Transient occurrence during acute illness. [Journal Article]
- JVJ Vector Borne Dis 2018 Jan-Mar; 55(1):52-57
- CONCLUSIONS: The disappearance of ANA during the convalescent phase suggests that ANA is expressed during the acute phase of scrub typhus infection. Its association with organ dysfunction warrants further study of the mechanisms and impact of autoantibody formation in scrub typhus.
- Rhupus syndrome and Chiari's network. [Journal Article]
- JFJ Family Med Prim Care 2018 Jan-Feb; 7(1):249-251
- A 69-year-old female patient was admitted to our clinic with photosensitivity, symmetric erosive polyarthritis, and cutaneous vasculitis of lower extremities. Rhupus syndrome was diagnosed, and Chiar...
A 69-year-old female patient was admitted to our clinic with photosensitivity, symmetric erosive polyarthritis, and cutaneous vasculitis of lower extremities. Rhupus syndrome was diagnosed, and Chiari's network in the right atrium and interatrial septum patent foramen ovale was achieved on transthoracic and transesophageal echocardiography. If it is thought that increased prevalence of antiphospholipid antibodies in patients with rhupus, this congenital remnant is important for the thrombosis risk, cardiac event, and stroke. The association of both diseases may lead to more serious events and cause worse prognosis. Here, our aim is to present a 69-year-old female patient with rhupus syndrome presenting with cutaneous vasculitis and Chiari's network in the right atrium.
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- Five-decade-delayed closed flexor tendon rupture due to Galeazzi dislocation fracture associated with Behçet syndrome: A case report. [Journal Article]
- IJInt J Surg Case Rep 2018 May 29; 48:87-91
- CONCLUSIONS: Reportedly, metalloproteases weaken tendon structure by acting as a collagenase in patients with Behçet syndrome. Also, vasculitis next to a tendon and steroid intake are considered to impede the tendon repair process. Hence, even minor trauma may lead to complete tendon rupture. Although an injury seems slight, we should take into account the possible history of bone and joint trauma.