- Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda. [Review]
- CEClin Exp Rheumatol 2018 Feb 20
- Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to classification and diagnostic termin...
Polyarteritis nodosa (PAN) is a rare form of primary systemic vasculitis with heterogeneous presentations, treatments and disease course. Historical approaches to classification and diagnostic terminology are reviewed. Since differentiation of PAN from microscopic polyangiitis (MPA) and other ANCA vasculitides by the Chapel Hill conference statements, and with hepatitis associated PAN defined as a secondary vasculitis, the phenotyping and subclassification of PAN has received little attention. Monogenic disorders similar to PAN have been described (familial Mediterranean fever, Adenosine Deaminase-2 deficiency), and cutaneous PAN and single organ vasculitis, discussed. The overlapping phenotypes between PAN and other primary vasculitic syndromes and subphenotypes within PAN are explored. This work will underpin development of newer treatment regimens and future genetic and related aetiologic studies.
- Acute respiratory distress syndrome in a man with Epstein-Barr virus infection-induced hypocomplementemic urticarial vasculitis. [Letter]
- JFJ Formos Med Assoc 2018 Feb 16
- Immune-mediated Neuropathies Our Experience over 3 Years. [Journal Article]
- JNJ Neurosci Rural Pract 2018 Jan-Mar; 9(1):30-35
- CONCLUSIONS: Immune-mediated neuropathies are treatable and hence should be diagnosed early for good quality outcome.
- Update on antiphospholipid antibody syndrome. [Journal Article]
- RARev Assoc Med Bras (1992) 2017; 63(11):994-999
- Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related...
Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antiphospholipid antibodies (aPL) associated with thrombosis and/or pregnancy morbidity. Most APS events are directly related to thrombotic events, which may affect small, medium or large vessels. Other clinical features like thrombocytopenia, nephropathy, cardiac valve disease, cognitive dysfunction and skin ulcers (called non-criteria manifestations) add significant morbidity to this syndrome and represent clinical situations that are challenging. APS was initially described in patients with systemic lupus erythematosus (SLE) but it can occur in patients without any other autoimmune disease. Despite the autoimmune nature of this syndrome, APS treatment is still based on anticoagulation and antiplatelet therapy.
- Propylthiouracil-induced ANCA-negative cutaneous small vessel vasculitis. [Journal Article]
- JCJ Community Hosp Intern Med Perspect 2018; 8(1):35-37
- Propylthiouracil (PTU) is a commonly used medication for the treatment of hyperthyroidism. PTU is known to cause different adverse reactions including autoimmune syndromes. PTU-induced autoimmune syn...
Propylthiouracil (PTU) is a commonly used medication for the treatment of hyperthyroidism. PTU is known to cause different adverse reactions including autoimmune syndromes. PTU-induced autoimmune syndromes can be classified into drug-induced lupus or drug-induced vasculitis. Differential diagnoses could be very challenging. PTU-induced vasculitis is more common than PTU-induced lupus, and has a higher risk of morbidity and mortality. Usually it is limited to the skin in a form of cutaneous leukocytoclastic vasculitis, but may also affect organs including kidneys and lungs. Discontinuation of PTU should be a first step in the treatment and could lead to complete resolution of symptoms. Typically, lesions resolve spontaneously within 2-4 weeks, but chronic or recurrent disease may occur in up to 10% of patients. In cases without improvement after drug discontinuation, cases refractory to glucocorticosteroids, with necrotizing skin lesions or extracutaneous organ involvement referral to rheumatologist for more aggressive immunosuppressive treatment is indicated. Optimal duration of immunosuppressive therapy is unknown, but it is reasonable to gradually taper mediations and monitor clinical response. Frequent monitoring for side effects is mandatory for patients on PTU therapy. Treatment should be stopped immediately, if patient develops any of autoimmune syndromes. An accurate and prompt diagnosis is essential, because it determines further management. We report a rare case of antineutrophil cytoplasm antibody-negative cutaneous small vessel vasculitis as a result of longstanding exposure to PTU.
- Comment on the article entitled "Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: A new overlap syndrome" (Autoimmunity Reviews 16 (2017) 1036-1043). [Letter]
- ARAutoimmun Rev 2018 Feb 08
- Anti-tubulin-α-1C autoantibody in systemic lupus erythematosus: a novel indicator of disease activity and vasculitis manifestations. [Journal Article]
- CRClin Rheumatol 2018 Feb 09
- A variety of autoantibodies has been involved in the pathogenesis of systemic lupus erythematosus (SLE), some of which are well known and applied as disease biomarkers. This study aimed to determine ...
A variety of autoantibodies has been involved in the pathogenesis of systemic lupus erythematosus (SLE), some of which are well known and applied as disease biomarkers. This study aimed to determine the prevalence of a novel autoantibody, anti-tubulin-α-1C, in patients with SLE and investigate its clinical significance. Anti-tubulin-α-1C autoantibody levels were determined by enzyme-linked immunosorbent assay (ELISA) in 128 SLE patients, 38 primary Sjögren's syndrome (pSS) patients, and 106 healthy controls (HCs).White blood cell (WBC) count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), IgM, IgG, C3, C4, RF, ANA, dsDNA, Sm, AnuA, aCL, anti-SSA, and anti-SSB were measured by standard laboratory techniques. SLE Disease Activity Index (SLEDAI) was evaluated accordingly. Anti-tubulin-α-1C antibody levels were significantly increased in SLE patients. Elevated anti-tubulin-α-1C were correlated with higher levels of SLEDAI, increased titers of anti-Sm antibody, and decreased titers of anti-dsDNA antibody and significantly associated with cutaneous and mucosal vasculitis and milder renal involvement. Anti-tubulin-α-1C may become a novel biomarker indicative of active vasculitis in SLE and could be applied in future clinical practice.
- The use of cerebral imaging for investigating delirium aetiology. [Journal Article]
- EJEur J Intern Med 2018 Feb 06
- CONCLUSIONS: Use of CT brain was common in delirium patients, with an 11% rate of positive findings. Fewer patients had MRI brain scans, which added diagnostic information in some cases. Future studies are needed to define the significance of cerebral imaging in delirium management and establish guidelines for its use.
- [Etiological profile of secondary Raynaud's phenomenon in an internal medicine department. About 121 patients]. [Journal Article]
- JMJ Med Vasc 2018; 43(1):29-35
- CONCLUSIONS: In our study, the Raynaud's phenomenon was most frequently secondary to connective tissue diseases. This may be a selection bias because our department is a third-line unit where patients are often referred for systemic disease suspicion.
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- Pruritic arthropod bite-like papules in T-cell large granular lymphocytic leukaemia and chronic myelomonocytic leukaemia. [Journal Article]
- CEClin Exp Dermatol 2018 Feb 09
- T-cell large granular lymphocytic leukaemia (T-LGLL) is a clinically indolent mature T-cell neoplasm characterized by a monoclonal population of CD3+ CD8+ cytotoxic T cells, which usually presents as...
T-cell large granular lymphocytic leukaemia (T-LGLL) is a clinically indolent mature T-cell neoplasm characterized by a monoclonal population of CD3+ CD8+ cytotoxic T cells, which usually presents as neutropenia, anaemia and thrombocytopenia. Chronic myelomonocytic leukaemia (CMML) is a clonal haematopoietic disorder with features of both a myeloproliferative neoplasm and myelodysplastic syndrome (MDS). Patients with CMML exhibit a persistent peripheral blood monocytosis in addition to myelodysplastic features. Because of the rarity of T-LGLL, its cutaneous manifestations are poorly documented, but include vasculitis, vasculopathy, persistent ulcerations, generalized pruritus and disseminated granuloma annulare. Various types of skin lesions have been observed in patients with CMML and reportedly occur in approximately 10% of cases. We report the extraordinary case of a patient with MDS who developed T-LGLL, and subsequently the MDS progressed to CMML. The patient then developed diffuse arthropod bite-like papules and intractable pruritus.