RAYNAUD’S PHENOMENON – FIRST SIGN OF MALIGNANCY: CASE REPORT

Raynaudov fenomen (RyF) je česti fenomen u općoj populaciji. Najčešće se javlja u zdravih pojedinaca u kojih se ne nalazi pridruženu bolest ili neki drugi uzrok RyF (primarni ili idiopatski RyF). Sekundarni RyF je čest uz reumatske bolesti (sistemsku sklerozu, sistemski eritemski lupus, primarni Sjogrenov sindrom, miješanu bolest vezivnog tkiva i dr.), okluzivne vaskularne bolesti, hematološke poremećaje, pri korištenju vibrirajućih alata te pri primjeni nekih lijekova, a rijetko uz maligne bolesti. Prikazujemo bolesnicu u koje je bolni RyF u trajanju od tri tjedna bio razlogom traženja pomoći u hitnoj internističkoj ambulanti, a nakon prijma u Kliniku dijagnostičkom je obradom utvrđen adenokarcinom pluća. U bolesnice su, u nižem titru, bila prisutna ANA i anti dsDNA protutijela te antikardiolipinska IgM i IgG protutijela. Poznato je da su u bolesnika s paraneoplastičkim reumatskim sindromima često prisutna antitijela, reumatoidni faktor ili antinuklearna antitijela (ANA) karakteristična za reumatske bolesti, što može navesti na krivi zaključak o eventualnoj sistemskoj bolesti vezivnog tkiva i u konačnici rezultirati kašnjenjem u postavljanju ispravne dijagnoze. Prva pojava RyF kao izoliranog simptoma u osoba starijih od 50 godina uz izrazite bolne znake ishemije tkiva, što je bio slučaj u naše bolesnice, ili kod pojave asimetričnog zahvaćanja prstiju, a posebice u muškaraca, bez obzira na prisutnost RF, ANA, anti dsDNA ili drugih autoantitijela, zahtijeva širu dijagnostičku obradu zbog moguće maligne bolesti.Raynaud’s phenomenon is a common phenomenon in the general population. It most commonly occurs in healthy individuals, in whom there is no associated illness or any other cause of Raynaud’s phenomenon (primary or idiopathic Raynaud’s phenomenon). Secondary Raynaud’s phenomenon is common with rheumatic diseases (systemic sclerosis, systemic lupus erythematosus, primary Sjögren’s syndrome, mixed connective tissue disease, etc.), occlusive vascular diseases, hematologic disorders, use of vibrating tools and use of some medications, and rarely with malignancy. We report on a patient who presented with a three-week history of painful Raynaud’s attacks, which was the reason for seeking assistance of internists in emergency clinic. Upon admission to the hospital and diagnostic work-up, adenocarcinoma of the lung was found. Antinuclear antibodies (ANA), anti-dsDNA antibodies, anticardiolipin IgM and IgG antibodies were present in a lower titer. It is known that rheumatoid factor or ANA characteristic of rheumatic disease are often present in patients with paraneoplastic rheumatic syndromes, which can lead to wrong conclusions about the possible systemic connective tissue diseases and ultimately delay the correct diagnosis. The first appearance of Raynaud’s phenomenon as an isolated symptom in people older than 50, with painful signs of ischemia, as in our patient, or the occurrence of asymmetric grasping fingers, especially in men, regardless of the presence of RF, ANA, anti-dsDNA or other autoantibodies, requires broader diagnostic evaluation for malignancy

Klinička obilježja vaskulitisa povezanih s antineutrofilnim citoplazmatskim protutijelima [Clinical manifestations of antineutrophil cytoplasmic antibodies associated vasculitis]

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare diseases, with the average of 30 new cases per million inhabitants per year. Their main characteristic is systemic involvement with necrosis of the vessel walls (histological changes showing necrosis of the media and inflammation of adventitia and intima). In some forms granulomas may be found surrounding the vessels. ANCA-associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, previously called Churg-Straus). Honorific eponyms are now changing to a disease-descriptive or etiology-based nomenclature. ANCA-associated vasculitides are a distinctive group of vasculitides because they dominantly involve small sized vessels, sometimes even medium sized vessels, are associated with antineutrophil cytoplasmic antibodies with high risk of developing glomerulonephritis and respond well to immunosuppresion with cyclophosphamide

CLINICAL MANIFESTATIONS OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES ASSOCIATED VASCULITIS

ANCA vaskulitisi su rijetke bolesti, koje se u prosjeku javljaju u 30 na milijun stanovnika. Riječ je o sistemskim nekrotizirajućim vaskulitisima (histološki se nalazi nekroza medije uz upalu intime i adventicije), a u pojedinim ANCA vaskulitisima granulomi okružuju krvne žile. U skupinu ANCA vaskulitisa ubrajaju se poliangiitis s granulomatozom (GPA), nekad nazivan Wegenerovom granulomatozom, mikroskopski poliangiitis (MPA) i poliangiitis s eozinofilnim granulomima (EGPA), prije poznat kao Churg-Straussov vaskulitis. Prema novoj nomenklaturi eponimi se zamjenjuju odgovarajućim opisnim nazivima za pojedine entitete. ANCA vaskulitisi čine zasebnu skupinu vaskulitisa s obzirom na to da najčešće zahvaćaju malene, katkad i srednje velike krvne žile, obično su udruženi s ANCA protutijelima i povezani s visokim rizikom od nastanka glomerulonefritisa te dobrim odgovorom na imunosupresivno liječenje ciklofosfamidom.Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare diseases, with the average of 30 new cases per million inhabitants per year. Their main characteristic is systemic involvement with necrosis of the vessel walls (histological changes showing necrosis of the media and inflammation of adventitia and intima). In some forms granulomas may be found surrounding the vessels. ANCA-associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener’s), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, previously called Churg-Straus). Honorific eponyms are now changing to a disease-descriptive or etiology-based nomenclature. ANCA-associated vasculitides are a distinctive group of vasculitides because they dominantly involve small sized vessels, sometimes even medium sized vessels, are associated with antineutrophil cytoplasmic antibodies with high risk of developing glomerulonephritis and respond well to immunosuppresion with cyclophosphamide