fuo und autoimmunerkrankungen · cryptogenic organizing pneumonia cyclic neutropenia drug fever and...
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FUO und Autoimmunerkrankungen
- Ein Streifzug-
Stefan Winkler Klink für Innere Medizin I
Abteilung für Infektionen und Tropenmedizin
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Fieber …
• …ist eine Kardinal-Antwort auf eine Infektion; in der Evolution über 600 Millionen Jahre sowohl in Kalt- als auch Warmblütlern konserviert
• …orchestriert durch physiologische und neuronale Kreisläufe- verbunden mit einem Überlebensvorteil bei Infektionen
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Fieber unbekannter Ursache (FUO)
1961 (Petersdorf und Beeson): Status febrilis ≥ 38,3°C (wiederholt gemessen) von mindestens
3 Wochen Dauer und einer umfassenden stationären
(1-wöchigen) Abklärung
1991 (Durack und Street): wie oben aber
3 Ambulanzbesuche bzw. 3 Tage stationäre Abklärung
(Zeit für BK und TB-Hauttests)
- Klassisches FUO
- Neutropenisches FUO (≤ 500 Granulozyten/µl)
- HIV-assoziiertes FUO
- Nosokomiales (im Spital erworbenes) FUO
Petersdorf, R.G. and P.B. Beeson. Medicine 1961;40:1-30.
Durack DT, Street AC. Curr Clin Top Infect Dis 1991;11:35-51.
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Fieber unbekannter Ursache (FUO)
Petersdorf, R.G. and P.B. Beeson. Medicine 1961;40:1-30. Larson, E.B., H.J. Featherstone, and R.G. Petersdorf. Medicine 1982;61:269-292. Knockaert, D.C., S. Vanderschueren, and D. Blockmans. J Intern Med 2003;253:263-275. de Kleijn, E.M., J.P. Vandenbroucke, and J.W. van der Meer. Medicine 1997;76:392-400. Bleeker-Rovers, C.P., et al. Eur J Nucl Med Mol Imaging 2007;34:694-703.
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Addison’s disease
Adult-onset Still’s disease
Alcoholic hepatitis
Allergic alveolitis
Aortic dissection
Aortitis (e.g. Takayasu`s)
Atrial myxoma
Autoimmune cholangitis
Bartonellosis
Behçet’s syndrome
Brucellosis
Carcinomatous meningitis
Castleman’s disease
Chronic meningitis
Cirrhotic fever
Cryptogenic organizing pneumonia
Cyclic neutropenia
Drug fever and other hypersensitivities
Eosinophilic Granulomatosis with Polyangiitis
(Churg-Strauss Syndrome)
Erythema multiforme
Fabry’s disease
Factitious fever
Familial Hibernian fever
Familial Mediterranean fever
Giant cell arteritis (Horton`s)
Giant coronary aneurysm
Granulomatous hepatitis
Granulomatous peritonitis
Granulomatosis with Polyangiitis (Wegener's)
Hantavirus infection
Haemoglobinopathies
Haemolytic anemias
Histiocytosis X
Human picornavirus infection
Hypereosinophilic syndrome
Hyper-IgD-syndrome
Immunoblastic lymphadenopathy
Haemophagocytic syndrome
Infected urachal cyst
Inflammatory bowel disease
Kikuchi-Fujimoto disease
Lofgren syndrome
Lymphomatoid granulomatosis
Metal fume fever
Myeloproliferative syndromes
Pancreatitis
Parathyroid apoplexy
Paroxysmal nocturnal haemoglobinurias
Pericarditis
Periodic fever syndromes (PFS)
Phaeochromocytoma
Polyarteritis nodosa
Postpericardiotomy syndrome (Dressler syndrome)
Pulmonary emboli
Resorbing haematoma
Retroperitoneal fibrosis
Rosai-Dorfman disease
Sarcoidosis
Schnitzler’s syndrome
Sinusitis
Serum sickness
Sjögren’s syndrome
Subacute necrotizing lymphadenitis
Thrombotic thrombocytopenic purpura
Thyroiditis (e.g. de Quervain) and thyrotoxicosis
TRAPS (tumor necrosis factor receptor–associated periodic fever)
Veno-occlusive disease
Vitamin B12 deficiency
Visceral leishmaniasis
Whipple’s disease
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• Pharmakologieprofessor, 65 a, Russe
• Fieber
• Vorhofflimmern
• viel CRP
• Thorax-Rö, Harnstreiferl unauffällig
Fieber die erste-der Pharmakologieprofessor…
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Antibiotika (empirisch) Clindamycin Amoxi/Clav Ceftriaxon
Therapien im Zuge des ersten stationären Aufenthaltes
Ohne Effekt…
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• TEE, Ganzkörper-CT, PET,…
• Lymphomverdächtige Strukturen in den NNH
–3x BX: nur chron. Entzündung
Fieber die erste-der Pharmakologieprofessor…
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Fieber die erste-der Pharmakologieprofessor…
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• Entlassung mit TBC-Diagnose, INH, Rifampicin und Moxifloxacin nach 10-wöchigem Aufenthalt in einem großen Wiener Spital (weil pos. QuantiFERON und Russe!?)
• Hausarzt bemerkt schlechten AZ und weist weiterhin febrilen Pat. des nächtens ein…
Fieber die erste-der Pharmakologieprofessor…
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Wie ging es weiter?
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Labor I
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Labor II
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PET-CT what else?
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Immunologie
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Diagnose
ANCA assoziierte Vaskulitis der
kleineren Gefäße
nein ja
mikroskopische Polyangiitis
M. Wegener Churg-Strauss-Synd.
Asthma? Massive Eosinophilie?
Nein
M. Wegener
Churg-Strauss-Synd.
Ja
Granulomatose mit
Polyangiitis
Eosinophile Granulomatose mit Polyangiitis früher M. Wegener
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2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides
Arthritis Rheum 2012;65(1):1-11
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PET-CT what else?
Harnzytologie: dysmorphe Erythrozyten
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AFOG stain 200x
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ANCA assoziierte Vasculitis
ANCA = Antikörper gegen Neutrophilen cytoplasmatisches Antigen
Indirekte Immunfluoreszenz
- In fixierten Leukozyten
- Sensitiv Screening
Cytoplasmatisches Muster (c-ANCA)
Perinucleäres Muster (p-ANCA)
ELISA
- Gegen reine Antigene
- Spezifisch
AK gg Myeloperoxidase (MPO-ANCA)
AK gg Proteinase 3 (PR3-ANCA)
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• Clindamycin • Amoxi/Clav • Ceftriaxon • Isoniazid • Rifampizin • Moxifloxacin
Fieber die erste-der Pharmakologieprofessor…
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Ist nicht automatisch eine aktive Tuberkulose!
Fieber die erste-der Pharmakologieprofessor…
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Nasenabstrich – wozu?
• bei 60%
• Herabgesetzte induzierbare epitheliale Sekretion von humanem Beta Defensin
• Peptide von S. aureus zeigen eine Homologie zu PR3: exogener Trigger?
• Häufiger Rückfälle?
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Fieber die zweite-die Lehrerin
• Lehrerin, 58 a
• Fieber
• „Alles tut weh“, kann die Arme nicht heben
• Konstant erhöhtes CRP, seronegativ
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?
Stumme Spondylodiszitis bei Immundefekt
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Fieber die zweite-die Lehrerin
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Therapien im Zuge des ersten stationären Aufenthaltes
Antibiotika (teils empirisch) Amoxi/Clav,
Moxifloxacin, Doxycyclin (wg. Chlamydien)
Ohne Effekt…
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• Kreuzreaktivität mit Umwelt-Stämmen
• Basistiter auf Grund von Kontakt mit Umweltstämmen sehr häufig
• Antikörperproduktion erst 6-8 Wochen nach der akuten Infektion
• Immer direkten Keimnachweis anstreben (Molekularbiologie, Kultur)!
• Keine Behandlung einer pos. Chlamydienserologie OHNE Klinik
Chlamydienserologie macht krank
Chlamydien…
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Fieber die zweite-die Lehrerin
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Labor
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25mg Aprednislon
Schulterschmerz
+ Alter >50
+ viel Entzündungszeichen
+ schnelles Ansprechen auf Kortison
= PMR
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PMR Epidemiologie
• Altershäufung 60 – 75. LJ.
• Geschlecht (f : m) 2 : 1
• Inzidenz /Jahr (bei >50. LJ) 12 / 100.000
• Prävalenz (bei >50. LJ) 600 / 100.000
In Österreich gibt es mindestens 16000 Patienten mit Polymyalgia rheumatica
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Typisches Kortisonschema bei PMR
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• Fieber=Infektion
• Erhöhtes CRP wird als „Infektionsparameter“ gesehen
• Antibiotikagabe: nutzt nix schads nix!?
• Spezialisierung…
• Diagnostische Rundumschläge (z.b. Serologien!) mit
Schwierigkeiten der Test-Interpretation und
direktem Weg in die Sackgasse:
„Legen einer falschen Spur“
Warum wird soviel Zeit bis zur Diagnose verloren?
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Gibt es einen medikamentösen Test um Rheuma/Tumor Fieber von Infektfieber zu unterscheiden?
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Naproxen 2x500mg/die
blau=Temp.
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„Naproxen-Test“
Patienten mit FUO >7 Tage erhielten Naproxen: Patienten mit “Infektions-Fieber” reagierten nicht, bei Tumor-Patienten liess sich das Fieber innerhalb von 24h prompt senken.
Chang JC and HM Gross. Am J Med 1984;76:597-603 Chang JC. Postgrad Med 1988;84:71-72
Zu geringe Sensitivität und Spezifität in der Unterscheidung von “Tumor-assoziiertem” Fieber zu “Infektions-Fieber”
Vanderschueren S. et al. Am J Med 2003;115:572-575
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Gibt es einen Labor-Test um Rheuma/Tumor Fieber von Infektfieber zu unterscheiden?
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Produkt der C Zellen (neuroendokrine Schilddrüsenzellen) als Vorstufe von Calcitonin
Bei bakteriellen Infektionen wird PCT von anderen differenzierten Zelltypen gebildet, die Produktion wird durch IFN-γ abgeschwächt (deshalb? geringe od. fehlende Freisetzung bei Virusinfekten)
Rolle bei der bakteriellen Infektion unklar
Procalcitonin (PCT)
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Fieber die erste-der Pharmakologieprofessor…
mit einem CRP von 20mg/dl
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0,5
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Tumorfieber?
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Schon wieder FUO…
74 Jahre alter Patient…
St.p. Schilddrüsen-CA mit Thyreoidektomie (2004)
St.p. Nierenzell-CA mit Nephrektomie li. Und postoperativer Epididymitis (2013)
Arterielle Hypertonie, Glaukom
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Labor I
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Labor II
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Schon wieder FUO…
Thorax-Rö unauffällig
Harn unauffällig
CT Thorax/Abdomen nicht diagnostisch ausser Leberzysten, deshalb noch MRT:
MRT Abdomen nicht diagnostisch …speziell kein Hinweis auf Rezidivtumore
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PET-MRT
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Diagnose?
M. Kichuchi-Fujimoto
M. Kawasaki
M. Takayasu
M. Hashimoto
M. Kitahara
M. Tako-Tsubo
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M. Takayasu
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Takayasu-Therapie:
Cortison über mind.12 Monate… evtl. MTX usw.
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PET-CT als FUO-Killer?
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Sensitivität 86% , Spezifität 52%, Diagnost. Treffsicherheit 58%
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Schlafkrankheit in Ö?
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70 jähriger Patient
Über die Notfall wegen Fieber und Somnolenz
2 Wochen zuvor komplikationslose Hüft-TEP
CRP 18 mg/dl, Natrium 121 mmol/l
Thoraxröntgen: beginnendes Infiltrat im UL re, Zeichen einer Bronchitis
Schlafkrankheit in Ö.?
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Diagnose: Pneumonie bei St. p. rezenter OP, mit Dehydratation und ausgeprägter Hyponatriämie… Cefuroxim und langsam NaCl…
“nix besonderes“
Infektiologe: wird schon wieder…
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CRP sinkt, Elektrolythaushalt ausgeglichen
Fiebert weiter >39°C Wacht nicht auf Neurologe? will einen CCT
(eh klar) unauffällig Gattin will den OA sprechen!!?
Es wird schlimmer und schlimmer…
Sogar DER wird unruhig…
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Pat. war schon nach der Hüft-OP sehr schläfrig
Seit Jahren ist ein Lupus-Antikoagulans bekannt, pTT > 150, ANA-Titer 1:1280 (Subsets neg), Anticardiolipin und Beta-2 Glykoprotein-AK erhöht
Oje, die Gattin steht im Stützpunkt
???
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…schichten wir halt durch
2 Wochen vorher
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Nebennieren-Infarkt!
Antiphospholipid- Syndrom
M. Addison
Cohen et al. BMJ 2010
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Es gibt aber Evidenz…
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• ACTH: 1400 pg/ml (normal bis 46)
• Cortisol: 4 μg/dl (normal 9,4-23,9)
Hydrocortison
Astonin H
Wiederauferstehung…
mit
bei
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Infektion
Malignome
„Rheuma“
Selbst-induziert
Bleibt ewig unklar…
FUO: Differenzialdiagnose
…und die Hormone
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Ein Klassiker…
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Eigentlich ein Klassiker…
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Immunologie
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SLE…Draft: EULAR/ACR Criteria
Entry ANA ≥1:80 Cut-off ≥10
Ne
ph
riti
s
Class III/IV 10
Skin
ACLE 6
He
mat
ol
a.i. hemolysis 4
Class II/V 8 SCLE/ DLE 4 Thrombocytopenia 4
Protein >0.5 g/d 4 Oral ulcers 2 Leukopenia 3
Ab
s
Anti-Sm 6 Alopecia 2
C3
/4
C3 AND C4 low 4
Anti-dsDNA 6
CN
S Seizures 5 C3 OR C4 low 3
Sero
s
Acute perikarditis
6 Psychosis 3 * Unexplained Fever
2
Effusion 5 Delirium 2 * ACLA/anti-β2gpI 2
*
Arthritis 6
Johnson et al, ACR 2017
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70.000
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Kindergärtnerin, 29 Jahre
> 3 Wochen bis 40°C Fieber (=FUO)
CRP 30mg/dl
Vorstellung an der HNO: kein Fokus
AB-Therapien mit Clarithromycin und Ceftriaxon wirkungslos
Pat. wird mit V.a. beginnende Sepsis (Tachycardie, Hypotonie) zur Übernahme angeboten…
70.000
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Labor bei Aufnahme
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Labor bei Aufnahme
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Hatte Halsweh!
Wechselnde (springende) Arthralgien!
Flüchtiges, lachsfarbenes Exanthem!
Sonst noch…
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EBV? Bystander-Reaktivierung (nicht ursächlich)
= schwer krank…
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70.000?
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• Major criteria: – Fieber >39ºC für >1 Woche
– Arthralgien/Arthritis >2 Wo
– Lachsfarbender Ausschlag
– Leukozytose >10,000/µL
• Minor criteria: – Halsschmerzen
– Lymphadenopathy
– Hepato- oder Splenomegalie
– LFT Erhöhung
• Negativer RA und ANA
≥5 (≥2 major)
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Increased serum vitamin B12 levels are associated with adult-onset Still's disease with reactive macrophage activation syndrome. (Kalyoncu U et al. Joint Bone Spine 2010;77:131-4)
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Hypertriglyzeridämie ohne Hypercholesterinämie durch Hemmung der Lipoproteinlipase
Erhöhung der Plasmakonzentration des Interleukin-2-Rezeptors (sCD25), Erhöhung der CD163-Serumkonzentration
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Hämophagozytose Score für MAS (Makrophagenaktivierungssyndrom)
Bekannte Immunsuppression (nein [0 Punkte]; ja [18 Punkte])
Körpertemperatur (<38,4° C [0 Punkte], 38,4–39,4° C [33 Punkte], >39,4° C [49 Punkte])
Organomegalie (nein [0 Punkte], Hepatomegalie oder Splenomegalie [23 Punkte], Hepatosplenomegalie [38 Punkte])
Zytopenien (Mono- [0 Punkte], Bi- [24 Punkte], Panzytopenie [34 Punkte])
Ferritin (<2000 ng/ml [0 Punkte], 2000–6000 ng/ml [35 Punkte], >6000 ng/ml [50 Punkte])
Triglyzeride (<1,5 mmol/l [0 Punkte], 1,5–4 mmol/l [44 Punkte], >4 mmol/l [64 Punkte])
Fibrinogen (>2,5 g/l [0 Punkte], <2,5 g/l [30 Punkte])
ASAT (<30 IE/l [0 Punkte], >30 IE/l [19 Punkte])
Hämophagozytose laut Hämatomyelogramm (nein [0 Punkte], ja [35 Punkte])
Die Wahrscheinlichkeit eines MAS variiert zwischen <1% (bei
einem HScore von <90) und >99% (bei einem HScore von >250) Maximaler HScore: 337 Punkte
Fardet L et al. Arthritis & Rheumatology
2014 ;66:2613–20.
Unsere Pat.: 254!
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Dg.: Morbus Still mit Makrophagenaktivierungssyndrom
(MAS)
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Ihre Morbus Still/MAS-Therapie:
Aprednislon 250mg iv
Tocilizumab (RoActemra®) mit 8mg/kg
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FUO AM KITZSTEINHORN
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46 jähriger Patient
Seit 24 Tagen Fieber
Antibiotika as usual…
Psoriasis mit Bedarfs-Humira (Adalimumab = anti-TNF-a)
FUO am Kitzsteinhorn…
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Labor I
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Labor II
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= 24 Tage Fieber und völlig normales Labor!
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Labor III
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Mediastinoskopie
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Gefrierschnitt: granulomatöse Entzündung…
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Granulomatöse Entzündung
M. Kichuchi-Fujimoto
M. Horton/M. Takayasu
Thyreoiditis de Quervain
Granulomatöse Polyangiitis (früher M. Wegener)
Granulomatöse Polyangiitis mit Eosinophilie (früher Churg-Strauss)
Sarkoidose
M. Crohn
Rheuma-Granulom bei RA
Mykobakteriosen (Tb/Lepra/Buruli, atypische usw.)
Listerien/Leishmanien/Histoplasmose/Bilharziose
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Nicht verkäsende Granulome mit Epitheloidzellen
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versus…
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verkäsendes Granulom
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Sarkoidose
Welches Syndrom?
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Löfgren Syndrom
Fieber
Bihiläre Lymphadenopathie
Sprunggelenkarthritis
Erythema nodosum
keine Biopsie notwendig Wahrscheinlichkeit der Sarkoidose:
…95%
Exzellente Prognose (wenige % chronisch)
Original: Lofgren, S, Lundback, H. The bilateral hilar lymphoma syndrome; a study of the relation to tuberculosis and sarcoidosis in 212 cases. Acta Med Scand 1952;142:265-273.
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Gibt`s sowas?
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Patientin, 38 Jahre, Schriftstellerin
Kommt mit Ehemann (gutsituierter Unternehmer) zur Abklärung
Seit 2 Jahren Entzündung der Nasenscheidewand…M. Behçet? (Pat. ist türkischstämmig)
Als Therapie in Kärnten Vitamininfusionen
Mit Cortison besser, therapiert sich selber
Gibt`s sowas?
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Granulomatose mit Polyangiitis (Wegener) Sarkoidose M. Behçet Polychondritis Tuberkulose/Lepra Leishmaniose Syphilis Klebsiella ozaena („Stinknase“) oder andere
chron. bakterielle Infekte Lymphome Sonstige Tumore
DD
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TB?
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gepoolte Sensitivität von 0,65
→ obwohl die diagnostischen Sensitivitäten beider IGRAs höher waren
als beim TST (gepoolte Sensitivität und Spezifität: 65% und 75%),
waren sie nicht hoch genug um eine TB auszuschließen…
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S. aureus in 60%
Peptide von S. aureus zeigen eine Homologie zu PR3: exogenerTrigger?
Häufiger Rezidive?
Nasenabstrich bei M. Wegener?
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M. Behçet?
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J Eur Acad Dermatol Venereol. 2014 Mar;28:338-47
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Immuno I
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Immuno II
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X-ANCA?
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Therapie?
Cortison!
Besser!
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Relapsing Polychondritis?
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und…
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!
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Neu auf der Mariahilferstrasse!
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Danke!
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Die Nasennebenhöhlen…
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37 jährige Patientin
Muskelschmerzen (Beginn in der
Kaumuskulatur) und Parästhesien
Seit Monaten Rhinitis und Asthma-
Symptome
FESS (functional endoscopic sinus
surgery) April 2016
Einmalig Fieber vor Aufnahme
Gewichtsverlust von 5kg in 2 Wochen
Die Nasennebenhöhlen…
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Labor bei Aufnahme
6730 Eos/µl
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Immunologie
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Eosinophile Granulomatose mit Polyangiitis (früher Churg-Strauss)
Diagnose-Kriterien
Lanham-Kriterien:
- Asthma
- Eosinophilie > 1500/µl
- Vaskulitis mind. zweier Organe
Chapel Hill Consensus Conference 1994
- Eosinophilen-reiche, granulomatöse Entzündung
des Respirationstraktes
- Betroffen sind kleine bis mittlere Gefäße
- Asthma und Eosinophilie
Lanham JG, et al. Medicine 1984;63:65-81 Jennette JC, et al. Arthritis Rheumatism 1994;37:187-192
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Lunge?
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(CIDP: Chronisch inflammatorische demyelinisierende Polyneuropathie)
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Periphere Neuropathie – häufig bei AAV?
Periphere Neuropathie 56 von 128 Pat (43,8%)
Charakteristika Anzahl (%) der PatientInnen
Symmetr. sensomotorische PNP 31 (55,4)
Mononeuritis multiplex 25 (44,6)
Peronäus 23
Tibialis 6
Suralis 2
Ulnaris 6
Medianus 3
Radialis 2
Axonale Neuropathie 41 (73,2)
Demyelinisierende Neuropathie 2 (3,6)
Akuter Beginn 25 (44,6)
Chronischer Verlauf 28 (50)
De Groot et al. Arch Neurol 2001;58:1215-1221
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Therapie? Cortison!
Noch was?
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Atemnot, Fieber und Schwäche
?
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Dyspnoe: Ist es das Herz?
Herzultraschall
⊥
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Dyspnoe: Ist es das Blutbild?
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Asthma bronchiale?
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Labor - Chemie
!
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Labor
Alpha 2 Gamma
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Thoraxröntgen
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Thorax-CT
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Thorax-CT
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Radiologie - CT
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Labor und Histologie
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Labor
!
!
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Werner Klotz, Universitätsklinik f. Innere Medizin I, Innsbruck, Rheumalabor
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Anti-Jo? Antikörper gegen Histidyl-tRNA-Synthetase bei etwa
35 % der Erwachsenen mit Polymyositis oder Dermatomyositis, vor allem (> 70 %) bei Patienten mit gleichzeitiger Lungensymptomatik, Arthritis und Raynaud-Phänomen=anti-Synthetase Syndrom
Klinik: Myositis (90 - 100 %), interstitielle Lungenfibrose (80 - 90 %), Arthritis (50 - 90 %), mit sog. Mechaniker-Händen (Fissuren bei Hyperkeratosen), Raynaud-Phänomen, Sklerodaktylie, Calcinosis cutis und Sicca-Symptomen.
Die Antikörper sind nicht selten auch mit anti-SS-A/Ro (anti-Ro 52 kDa) assoziiert.
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PET/CT – what else?
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Therapie
• Cortison
• IVIG mit Mycophenolate mofetil
• Rituximab
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IVIG mit Mycophenolate mofetil
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Rituximab
Nature Clinical Practice Rheumatology (2007) 3, 86-95
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Late
Early
Muskel-Symptomscore
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Spätfrühling 2013
Mit Rituximab!