Czihal, Michael

Jansson, Annette Friederike

Lottspeich, Christian

Oberlack, Ava

English

Universität München: Elektronischen Publikationen

LUCCHESI ET AL 1570       |
 42. Pot C, Jin H, Awasthi A, Liu SM, Lai CY, Madan R, et al. Cutting 
edge: IL- 27 induces the transcription factor c- Maf, cytokine IL- 21, 
and the costimulatory receptor ICOS that coordinately act together 
to promote differentiation of IL- 10- producing Tr1 cells. J Immunol 
2009;183:797–801.
 43. Kimura D, Miyakoda M, Kimura K, Honma K, Hara H, Yoshida H, 
et al. Interleukin- 27- producing CD4+ T cells regulate protective im-
munity during malaria parasite infection. Immunity 2016;44:672–82.
 44. Verstappen GM, Corneth OB, Bootsma H, Kroese FG. Th17 cells in 
primary Sjögren’s syndrome: pathogenicity and plasticity. J Autoim-
mun 2018;87:16–25.
 45. Nezos A, Gravani F, Tassidou A, Kapsogeorgou EK, Voulgarelis M, 
Koutsilieris M, et al. Type I and II interferon signatures in Sjogren’s 
syndrome pathogenesis: contributions in distinct clinical phenotypes 
and Sjogren”s related lymphomagenesis. J Autoimmun 2015;63: 
47–58.
DOI 10.1002/art.41313
Clinical Images: Unilateral Hippocratic fingers and macaroni sign
The patient, a 17- year- old girl, presented to the emergency department with a 2- week history of pain in her left arm and swelling of the 
fingers on her left hand. Her history was otherwise unremarkable. Notably, there were no symptoms of systemic inflammation (night 
sweats, fever, or weight loss). Clinical examination of the left arm revealed absence of pulses. Blood pressure was low (systolic 50 mm Hg) 
when measured on the left arm and elevated (170/100 mm Hg) when measured on the right arm. Physical examination revealed finger 
clubbing (Hippocratic fingers) only on the left hand. The C- reactive protein level was mildly elevated (1.8 mg/dl [normal <0.5]). Color duplex 
sonography showed filiform long- segment stenosis in the left common carotid artery (CCA) with marked concentric intimal thickening 
(arrow). This sonographic feature, known as macaroni sign, confirmed the diagnosis of Takayasu arteritis (TAK). Ultrasound and magnetic 
resonance angiography revealed an occlusion of the left subclavian and axillary artery (asterisk) as an underlying cause of finger clubbing 
on the left hand. Further imaging also revealed significant bilateral renal artery stenosis causing renovascular hypertension. Immunosup-
pressive and antihypertensive treatment was initiated. Subsequently, renal artery stenosis was treated with stent angioplasty. Bilateral club-
bing can be seen in patients with severe cardiopulmonary disease, classically in patients with congenital cyanotic heart disease. Unilateral 
clubbing is a very rare presentation of TAK, resulting from severe chronic upper limb ischemia (1–3).
Christian Lottspeich, MD
Michael Czihal, MD
Medical Clinic and Policlinic IV 
Hospital of the Ludwig-Maximilians-University
Annette Friederike Jansson, MD
Ava Oberlack, MD
Dr. von Hauner Children’s Hospital 
Hospital of the Ludwig-Maximilians-University
Munich, Germany
 1. Kaditis AG, Nelson AM, Driscoll DJ. Takayasu’s arteritis presenting 
with unilateral digital clubbing. J Rheumatol 1995;22:2346–8.
 2. Ishikawa M, Okada J, Kondo H. Takayasu’s arteritis with transient 
clubbed finger. Clin Exp Rheumatol 1999;17:629–30.
 3. Çivilibal M, Selçuk Duru N, Doğdu G, Elevli M, Ayta S. A Takayasu’s 
arteritis case with unilateral digital clubbing. Turk J Rheumatol 2011; 
26:163–6.


Unilateral Hippocratic Fingers and Macaroni Sign

Open Access LMU

LUCCHESI ET AL 1570       | 42. Pot C, Jin H, Awasthi A, Liu SM, Lai CY, Madan R, et al. Cutting edge: IL- 27 induces the transcription factor c- Maf, cytokine IL- 21, and the costimulatory receptor ICOS that coordinately act together to promote differentiation of IL- 10- producing Tr1 cells. J Immunol 2009;183:797–801. 43. Kimura D, Miyakoda M, Kimura K, Honma K, Hara H, Yoshida H, et al. Interleukin- 27- producing CD4+ T cells regulate protective im-munity during malaria parasite infection. Immunity 2016;44:672–82. 44. Verstappen GM, Corneth OB, Bootsma H, Kroese FG. Th17 cells in primary Sjögren’s syndrome: pathogenicity and plasticity. J Autoim-mun 2018;87:16–25. 45. Nezos A, Gravani F, Tassidou A, Kapsogeorgou EK, Voulgarelis M, Koutsilieris M, et al. Type I and II interferon signatures in Sjogren’s syndrome pathogenesis: contributions in distinct clinical phenotypes and Sjogren”s related lymphomagenesis. J Autoimmun 2015;63: 47–58.DOI 10.1002/art.41313Clinical Images: Unilateral Hippocratic fingers and macaroni signThe patient, a 17- year- old girl, presented to the emergency department with a 2- week history of pain in her left arm and swelling of the fingers on her left hand. Her history was otherwise unremarkable. Notably, there were no symptoms of systemic inflammation (night sweats, fever, or weight loss). Clinical examination of the left arm revealed absence of pulses. Blood pressure was low (systolic 50 mm Hg) when measured on the left arm and elevated (170/100 mm Hg) when measured on the right arm. Physical examination revealed finger clubbing (Hippocratic fingers) only on the left hand. The C- reactive protein level was mildly elevated (1.8 mg/dl [normal <0.5]). Color duplex sonography showed filiform long- segment stenosis in the left common carotid artery (CCA) with marked concentric intimal thickening (arrow). This sonographic feature, known as macaroni sign, confirmed the diagnosis of Takayasu arteritis (TAK). Ultrasound and magnetic resonance angiography revealed an occlusion of the left subclavian and axillary artery (asterisk) as an underlying cause of finger clubbing on the left hand. Further imaging also revealed significant bilateral renal artery stenosis causing renovascular hypertension. Immunosup-pressive and antihypertensive treatment was initiated. Subsequently, renal artery stenosis was treated with stent angioplasty. Bilateral club-bing can be seen in patients with severe cardiopulmonary disease, classically in patients with congenital cyanotic heart disease. Unilateral clubbing is a very rare presentation of TAK, resulting from severe chronic upper limb ischemia (1–3).Christian Lottspeich, MDMichael Czihal, MDMedical Clinic and Policlinic IV Hospital of the Ludwig-Maximilians-UniversityAnnette Friederike Jansson, MDAva Oberlack, MDDr. von Hauner Children’s Hospital Hospital of the Ludwig-Maximilians-UniversityMunich, Germany 1. Kaditis AG, Nelson AM, Driscoll DJ. Takayasu’s arteritis presenting with unilateral digital clubbing. J Rheumatol 1995;22:2346–8. 2. Ishikawa M, Okada J, Kondo H. Takayasu’s arteritis with transient clubbed finger. Clin Exp Rheumatol 1999;17:629–30. 3. Çivilibal M, Selçuk Duru N, Doğdu G, Elevli M, Ayta S. A Takayasu’s arteritis case with unilateral digital clubbing. Turk J Rheumatol 2011; 26:163–6.

Kaditis AG

Ishikawa M

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https://epub.ub.uni-muenchen.de/73898/1/art.41313.pdf

Unilateral Hippocratic Fingers and Macaroni Sign

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