[The importance of an early diagnosis: about a case of Wegener's granulomatosis].

Serviço de Nefrologia, Hospital de Curry Cabral, Lisboa.
Acta medica portuguesa (Impact Factor: 0.28). 17(5):399-404.
Source: PubMed

ABSTRACT We report a case of a young man with Wegener's granulomatosis. Initial clinical features were constitutional symptoms and nasal sinus involvement. Those manifestations were not correctly understood during four years, based on the lack of pathological findings. It was only when nephrotic syndrome was present, apparently after a three to four years renal involvement that diagnosis was found, supported upon renal biopsy, the presence of serum antineutrophil cytoplasm antibodies (ANCA) and revision of initial pathological specimen from sinus surgery. For many reasons it was not possible to make a correct diagnosis on time to prevent permanent renal failure and facial destroying lesions. The natural history of this clinical case emphasizes the need of an early and correct diagnosis, to prevent serious consequences of a severe illness.

Download full-text


Available from: Fernando Teixeira e Costa, Jul 06, 2015
  • Source
    Nephrology Dialysis Transplantation 07/1999; 14(6):1366-75. DOI:10.1093/ndt/14.6.1366 · 3.49 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: The epidemiology of the systemic vasculitides is poorly documented. Many studies have been conducted from tertiary referral centers, with resulting problems of referral bias and uncertainty of denominator population, or have involved small populations. We have estimated the incidence of the major forms of systemic vasculitis in a stable, ethnically homogeneous population of 414,000 adults from 1988 to 1994. The overall annual incidence of systemic vasculitis (excluding giant cell arteritis) is 39/million (95% confidence intervals; ranging from 31 to 47). The annual incidence of Wegener's granulomatosis is 8.5/million (range, 5.2 to 12.9), Churg-Strauss syndrome 2.4/million (0.9 to 5.3), microscopic polyangiitis 2.4/million (0.9 to 5.3), adult Henoch-Schonlein purpura 1.2/million (0.3 to 3.5), and systemic rheumatoid vasculitis 12.5/million (8.5 to 17.7). These data suggest that the overall incidence of systemic vasculitis is greater than previously thought (10/million) with Wegener's granulomatosis and systemic rheumatoid vasculitis being the most common. Whether this represents a genuine increase in incidence or increased physician awareness is uncertain.
    Seminars in Arthritis and Rheumatism 08/1995; 25(1):28-34. DOI:10.1016/S0049-0172(95)80015-8 · 3.63 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Immunopathologic studies over the past two decades have demonstrated that rapidly progressive glomerulonephritis (RPGN) can result from glomerular deposition of anti-GBM antibody, immune complexes, or from some as yet undefined mechanism that does not involve glomerular antibody deposition. The latter process may be cell mediated and resembles a small vessel vasculitis. Most cases of idiopathic RPGN are not accompanied by pathogenic glomerular immunoglobulin deposition. Recent experimental studies of immune mechanisms of glomerular injury have identified several new processes that can induce damage to the capillary wall sufficient to result in crescentic glomerulonephritis (GN). These include direct effects of anti-GBM antibody alone and of the complement C5b-9 (membrane attack) complex, nephritogenic effects of inflammatory effector cells that involve reactive oxygen species and glomerular halogenation, and injury mediated by sensitized lymphocytes independently of antibody deposition. Macrophages have been shown to participate in both intracapillary and extracapillary fibrin deposition and crescent formation as well as to mediate capillary wall damage. The role of resident glomerular cells and cell-cell interactions in glomerulonephritis is still under active investigation. Despite these several advances in understanding immune injury to the glomerulus, therapy for RPGN remains largely empiric. Although the prognosis in RPGN has clearly improved over time, no form of disease-specific therapy has been clearly shown yet to be beneficial in a controlled study. Interpretation of the existing literature on therapy is complicated by the availability of only historical rather than concurrent controls, lack of attention to several variables known to affect disease outcome, and uncertainty regarding bias in favor of reporting positive results. Available data suggests that optimal outcomes may be achieved in anti-GBM nephritis by treatment with steroids, immunosuppression and plasma exchange, particularly when therapy is directed at patients with mild but rapidly progressive disease before oliguria or severe azotemia develop. Pulse steroids are probably the most cost-effective therapy for the idiopathic form of RPGN, but treatment with cytotoxic agents should be considered if clinical or histologic evidence of vasculitis is present.
    American Journal of Kidney Diseases 07/1988; 11(6):449-64. DOI:10.1016/S0272-6386(88)80079-9 · 5.76 Impact Factor