Vasculitis in India—the viper or water snake

Abstract

Department of Rheumatology, Madras Medical College, Chennai, India. Correspondence: Dr. S Rajeswari, email: calling_raji@yahoo.com Where do I begin. To tell the story of how great “Vasculitis” can be The Vasculitis story that is older than the sea. The simple truth about the love that Vasculitis brings to me Where do I start ..! I will attempt to answer the questions that have risen in my mind regarding vasculitis and share my thoughts with all. How many are aware as to how to approach a given case of vasculitis. The clinician’s (rheumatologist’s) role cannot be replaced. In India, the expertise of a rheumatologist who makes a good provisional clinical diagnosis with a good history is well begun and half done. Let’s start at the beginning. Is it a vasculitis or a vasculopathy? Especially with regard to antiphospholipid syndrome (APS). Is it a primary or secondary vasculitis? Is it a small, medium or large vessel vasculitis? How much have we progressed as far as aetiology, confirmation, disease activity assessment and treatment of these great masqueraders is concerned? How are we tackling vasculitic emergencies? What steps have we taken to capture “instant strikers” like retinal and CNS (especially primary) vasculitis when they are the initial manifestations? Do we screen routinely for TORCH panels and HIV? More questions than answers! It is ideal to initially rule out a secondary vasculitis (especially small and medium vessel vasculitis with cutaneous involvement), the common causes being lupus, followed by Sjogren, rheumatoid, MCTD, dermatomyositis, etc. Internal organ involvement has to be looked for. A cutaneous vasculitis requires a biopsy ideally within 24 hours of manifestation or at the earliest, with sections including the deep dermis up to the subcutis, including the panniculus. If secondary vasculitis is ruled out (unless the initial clinical picture is striking and fulfills the respective criteria for primary vasculitis) things become easy. Association of both also is not uncommon. Associated infections, drugs, malignancies and co-morbid states should be ruled out. Having attempted to answer these basic questions, let’s self assess ourselves! An easy rule to follow may be is to club the clinical associations, followed by the most immediate appropriate investigations, while instituting timely management (of course aggressive) at the same time. Remember the other rheumatological emergencies—digital gangrene, mononeuritis multiplex, the pulmonary renal syndromes, seizures, CVAs, acute LVF, myocardial infarcts, not forgetting sepsis. A closer look at some of the individual vasculitides may reveal unresolved issues.