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Concept: Vascular-related cutaneous conditions


Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a severe condition encompassing two major syndromes: granulomatosis with polyangiitis (formerly known as Wegener’s granulomatosis) and microscopic polyangiitis. Its cause is unknown, and there is debate about whether it is a single disease entity and what role ANCA plays in its pathogenesis. We investigated its genetic basis.

Concepts: Vasculitis, Wegener's granulomatosis, Autoimmune diseases, Arthritis, Granuloma, Anti-neutrophil cytoplasmic antibody, Proteinase 3, Vascular-related cutaneous conditions


Vasculitis is a destructive inflammatory process affecting blood vessels. Pulmonary vasculitis may develop secondary to other conditions or constitute a primary idiopathic disorder. Thoracic involvement is most common in primary idiopathic large-vessel vasculitides (Takayasu arteritis, giant cell arteritis, Behçet disease) and primary antineutrophil cytoplasmic autoantibody-associated small-vessel vasculitides (Wegener granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome). Primary pulmonary vasculitides are rare, and their signs and symptoms are nonspecific, overlapping with those of infections, connective tissue diseases, and malignancies. The radiologic findings in primary pulmonary vasculitis vary widely and can include vessel wall thickening, nodular or cavitary lesions, ground-glass opacities, and consolidations, among others. Diffuse alveolar hemorrhage usually results from primary small-vessel vasculitis in the lungs. To diagnose vasculitis, medical teams must recognize characteristic combinations of clinical, radiologic, laboratory, and histopathologic features.

Concepts: Inflammation, Blood, Blood vessel, Rheumatology, Vasculitis, Wegener's granulomatosis, Giant cell arteritis, Vascular-related cutaneous conditions


Systemic vasculitides are great masqueraders and at times their presenting manifestations can be very different from the usual recognized patterns. Such uncommon presentations of granulomatosis with polyangiitis (Wegener’s granulomatosis), classical polyarteritis nodosa and unclassifiable vasculitides are described here with the relevant review of literature.

Concepts: Rheumatology, Vasculitis, Wegener's granulomatosis, Arthritis, Polyarteritis nodosa, Vascular-related cutaneous conditions, Microscopic polyangiitis, Diseases involving the fasciae


Cutaneous polyarteritis nodosa, a special form of polyarteritis nodosa (PAN) without systemic involvement, is classified as one of the ANCA-negative vasculitides of small and medium-sized vessels. It is a very rare disease with unknown etiology and occurs more commonly in women over the age of 40. Typical skin lesions are subcutaneous nodules, livedo racemosa, and ulcerations. We report the case of a 46-year-old woman presenting to our outpatient department who reported having very painful ulcerations of the lower legs with unknown origin for 6 months.

Concepts: Rheumatology, Vasculitis, Polyarteritis nodosa, Vascular-related cutaneous conditions, Livedo reticularis


This article provides an update on the diagnosis and management of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides, granulomatosis with polyangiitis (formerly Wegener), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss). Focus is on new schemes of classification and the importance of ANCAs in the diagnosis and prognosis of these systemic vasculitides. Current therapeutic strategies consisting of glucocorticoids in conjunction with conventional or biologic agents for both induction of remission and remission maintenance are outlined. Future research directions include investigation of the optimal duration and frequency of maintenance therapy and development of targeted therapeutic agents.

Concepts: Rheumatoid arthritis, Rheumatology, Vasculitis, Wegener's granulomatosis, Arthritis, Anti-neutrophil cytoplasmic antibody, Vascular-related cutaneous conditions


Postural tachycardia syndrome (POTS) is a syndrome of orthostatic intolerance in the setting of excessive tachycardia with orthostatic challenge, and these symptoms are relieved when recumbent. Apart from symptoms of orthostatic intolerance, there are many other comorbid conditions such as chronic headache, fibromyalgia, gastrointestinal disorders, and sleep disturbances. Dermatological manifestations of POTS are also common and range widely from livedo reticularis to Raynaud’s phenomenon.

Concepts: Cardiology, Rheumatology, Syndromes, Orthostatic hypotension, Postural orthostatic tachycardia syndrome, Vascular-related cutaneous conditions, Orthostatic intolerance, Livedo reticularis


Buerger’s disease or thombo-angiitis obliterans disease is a small vessel’s vasculitis, frequently observed in young and smoker’s males. Diagnosis is based on both clinical and radiological arguments. There is no specific treatment designed for this disease. We report the case of 43 years old patient presenting with an acute kidney injury associated with Buerger’s disease. We reviewed the different case of kidney disease in this rare disease.

Concepts: Epidemiology, Death, Medical terms, Acute kidney injury, Vascular-related cutaneous conditions, Buerger's disease


Adenosine deaminase-2 (ADA2) deficiency (DADA2) is associated with early onset polyarteritis nodosa and vasculopathy. Classic presentation includes livedo reticularis, vasculitis, and stroke. However, the phenotype and disease severity are variable. We present a 5-year-old female who presented with features that mimicked autoimmune lymphoproliferative syndrome (ALPS) in the absence of classic features of DADA2. Exome sequencing identified a novel homozygous splicing variant in ADA2 c.882-2A > G. Patient responded to anti- tumor necrosis factor medication and is in complete remission. Hematologists should be aware of various hematological presentations of DADA2, including ALPS-like disorder, that might lack vasculitis and livedo reticularis to prevent delay in initiating optimal therapy.

Concepts: Rheumatology, Vasculitis, Polyarteritis nodosa, Vascular-related cutaneous conditions, Livedo reticularis


To report a case of retinal vasculitis associated with cutaneous leukocytoclastic vasculitis.

Concepts: Vasculitis, Vascular-related cutaneous conditions, Cutaneous small-vessel vasculitis


Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are the two main large vessel vasculitides. They share some similarities regarding their clinical, radiological and histological presentations but some pathogenic processes in GCA and TAK are activated differently, thus explaining their different sensitivity to biological therapies. The treatment of GCA and TAK essentially relies on glucocorticoids. However, thanks to major progress in our understanding of their pathogenesis, the role of biological therapies in the treatment of these two vasculitides is expanding, especially in relapsing or refractory diseases. In this review, the efficacy, the safety and the limits of the main biological therapies ever tested in GCA and TAK are discussed. Briefly, anti TNF-α agents appear to be effective in treating TAK but not GCA. Recent randomized placebo-controlled trials have reported on the efficacy and safety of abatacept and mostly tocilizumab in inducing and maintaining remission of GCA. Abatacept was not effective in TAK and robust data are still lacking to draw any conclusions concerning the use of tocilizumab in TAK. Furthermore, ustekinumab appears promising in relapsing/refractory GCA whereas rituximab has been reported to be effective in only a few cases of refractory TAK patients. If a biological therapy is indicated, and in light of the data discussed in this review, the first choice would be tocilizumab in GCA and anti-TNF-α agents (mainly infliximab) in TAK.

Concepts: Medicine, Effectiveness, Rheumatoid arthritis, Vasculitis, Giant cell arteritis, Takayasu's arteritis, Arteritis, Vascular-related cutaneous conditions