Romanian Journal of Rhinology, Vol. 4, No. 14, April-June 2014 Corresponding author: Codrut Sarafoleanu, MD, PhD, Professor of Otorhinolaryngology email: csarafoleanu@gmail.com LITERATURE REVIEW Codrut Sarafoleanu, Claudiu Manea ENT&HNS Department, “Sfanta Maria” Hospital, Bucharest, Romania “Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania Nasal manifestations of systemic diseases – a literature review ABSTRACT Multiple systemic diseases may have an expression in the sinonasal area, developing various, slow and atypical clinical manifes- tations, difficult to diagnose. An increased index of suspicion on behalf of the otolaryngologist and also a good multidisci- plinary collaboration is required in order to establish a correct diagnosis and to treat this kind of pathology. This article is a review of the most common clinical entities with sinonasal manifestation, emphasizing on the diagnosis and therapeutic algorithm used for each pathological entity. KEYWORDS: granulomatous diseases, Wegener’s granulomatosis, sarcoidosis, Churg-Strauss syndrome, mucociliary diseases INTRODUCTION Although nowadays the incidence of systemic dis- eases affecting the upper airway appears to be increas- ing, the diagnosis is being made earlier because of the heightened degree of suspicion on the part of otolar- yngologists, as they play an important role in the early and differential diagnosis of these diseases. A thor- ough clinical examination and paraclinical evaluation as well as a multidisciplinary approach are essential for the diagnosis and treatment of these illnesses 1,2 . Multiple systemic diseases may have an expression in the sinonasal area developing various, slow and atypical clinical manifestations, difficult to diagnose. Evaluation of such patients must consider the multisys- temic effects of these pathologies and combine the exhaustive clinical examination and the thorough an- amnesis with a multidisciplinary approach. There are two ways of dealing with such disorders: understand- ing the pathophysiology of the systemic diseases and using the specific diagnostic tools necessary to confirm or rule out different diagnoses 2 . Systemic diseases with rhinosinusal symptoms can be divided in inflammatory, infectious, malignant and genetic. The nasal signs that may lead us to search for a systemic disease, even if they are not pathognomonic or specific, are the presence of crusting, septal perfo- ration and a granular mucosa. Also, we should expect for a head and neck manifestation in the patients with primary antibody deficiencies, uncontrolled diabetes mellitus, leukaemia, AIDS, bone marrow or organ transplantation 3 . In this literature review, we will try to present the most common systemic diseases with sinonasal mani- festation, emphasizing on the diagnosis and therapeu- tic algorithm used for each pathological entity. GRANULOMATOUS DISEASES There are several granulomatous diseases that may cause head and neck manifestations, characterized by a local inflammatory response mainly in the upper nasal passages. These include Wegener’s granuloma- tosis (WG), the most common of them, Churg-Strauss syndrome (CSS) and sarcoidosis 3 . The key element of granulomatous condition is, of course, the presence of a granuloma containing mac- rophages, epithelioid cells and multinucleated giant cells. Unfortunately, this histologic configuration is