Acute Form of Sarcoidosis: Löfgren’s Syndrome

 

 Permissions and Reprints

Abstract

Löfgren’s syndrome, an acute form of sarcoidosis, is the combination of bilateral hilar lymphadenopathy (BHL), erythema nodosum (EN) and polyarthralgia or arthritis. Unlike sarcoidosis, Löfgren syndrome has good prognosis and a lower relapse rate. A 58-year-old woman was admitted to our department complaining of arthralgia, rashes on the pretibial region and other nonspecific symptoms such as fever, cough and weakness. Based on clinical symptoms, radiological and histological findings we diagnosed Löfgren’s syndrome. The patient was treated with Prednisolone 0,5 mg/kg/day for six months and acute arthralgia as well as erythema nodosum disappeared.

Zusammenfassung

Eine spezifische akute Form der Sarkoidose – Löfgren-Syndrom – manifestiert als Kombination aus bilateraler Hiluslymphadenopathie (BHL), Erythema nodosum und Polyarthralgie oder Arthritis. Im Gegensatz zur Sarkoidose hat das Löfgren-Syndrom eine gute Prognose. Eine 58-jährige Frau wurde in unsere Klinik wegen Arthralgie, akutem prätibialem Erythema nodosum, Fieber, Husten und allgemeiner Schwäche aufgenommen. Basierend auf den klinischen, radiologischen und histologischen Befunden wurde ein Löfgren-Syndrom diagnostiziert. Die Patientin wurde mit Prednisolon 0,5 mg/kg pro Tag sechs Monate lang behandelt. Alle Krankheitssymptome zeigten eine Remission.

• References

• 1 Petrilla JC. Lofgren’s syndrome: A Clinical Variant of Sarcoidosis. Hospital Physician 2002; 40-43
  PubMedGoogle Scholar
• 2 Tejera Segura B, Holgado S, Mateo L et al. Löfgren syndrome: a study of 80 cases. Med Clin (Barc) 2014; 143: 166-169
  CrossrefPubMedGoogle Scholar
• 3 Mañá J, Gómez-Vaquero C, Montero A et al. Löfgren’s syndrome revisited: a study of 186 patients. Am J Med 1999; 107: 240-245
  CrossrefPubMedGoogle Scholar
• 4 Grunewald J, Eklund A et al. Sex-specific manifestations of Löfgren’s syndrome. Am J Respir Crit Care Med 2007; 175: 40-44
  CrossrefPubMedGoogle Scholar
• 5 Byun CW, Yang SN et al. Lofgren’s Syndrome – Acute Onset Sarcoidosis and Polyarthralgia: A Case Report. Ann Rehabil Med 2013; 37: 295-299
  CrossrefPubMedGoogle Scholar
• 6 Zisman DA, Shorr AF, Lynch 3rd JP et al. Sarcoidosis involving the musculoskeletal system. Semin Respir Crit Care Med 2002; 23: 555-570
  Article in Thieme ConnectPubMedGoogle Scholar
• 7 Tambouret R, Geisinger KR, Powers CN et al. The clinical application and cost analysis of fine – needle aspiration biopsy in the diagnosis of mass lesions in sarcoidosis. Chest 2000; 117: 1004-1011
  CrossrefPubMedGoogle Scholar
• 8 Reich JM, Brouns MC, O’Connor EA et al. Mediastinoscopy in patients with presumptive stage I sarcoidosis: a risk/benefit, cost/benefit analysis. Chest 1998; 113: 147-153
  CrossrefPubMedGoogle Scholar
• 9 Kumar G, Kumar N et al. All red is not always bacterial cellulitis: a case of Löfgren’s syndrome. WMJ 2010; 109: 31-33
  PubMedGoogle Scholar
• 10 Dr. Chloe et al. Löfgren’s syndrome. http://www.patient.co.uk/doctor/lofgrens-syndrome
  PubMedGoogle Scholar
• 11 Dubrey S, Shah S, Hardman T et al. Sarcoidosis: the links between epidemiology and aetiology. Postgrad Med J 2014; 90: 582-589
  CrossrefPubMedGoogle Scholar
• 12 Hosoda Y, Sasagawa S, Yasuda N et al. Epidemiology of sarcoidosis: new frontiers to explore. Curr Opin Pulm Med 2002; 8: 424-428
  CrossrefPubMedGoogle Scholar
• 13 Rybicki BA, Iannuzzi MC, Frederick MM et al. Familial aggregation of sarcoidosis. A case-control etiologic study of sarcoidosis (ACCESS). Am J Respir Crit Care Med 2001; 164: 2085-2091
  CrossrefPubMedGoogle Scholar
• 14 Fité E, Alsina JM, Mañá J et al. Epidemiology of sarcoidosis in Catalonia: 1979 – 1989. Sarcoidosis Vasc Diffuse Lung Dis 1996; 13: 153-158
  PubMedGoogle Scholar
• 15 Sipahi Demirkok S, Basaranoglu M, Dervis E et al. Analysis of 87 patients with Löfgren’s syndrome and the pattern of seasonality of subacute sarcoidosis. Respirology 2006 11: 456-461
  PubMedGoogle Scholar
• 16 Newman LS, Rose CS, Bresnitz EA et al. A case control etiologic study of Sarcoidosis: environmental and occupational risk factors. Am J Respir Crit Care Med 2004; 170: 1324-1330
  CrossrefPubMedGoogle Scholar
• 17 Terčelj M, Stopinšek S, Ihan A et al. In vitro and in vivo reactivity to fungal cell wall agents in sarcoidosis. Clin Exp Immunol 2011; 166: 87-93
  CrossrefPubMedGoogle Scholar
• 18 Darlington P, Gabrielsen A, Sörensson P et al. HLA-alleles associated with increased risk for extra-pulmonary involvement in sarcoidosis. Tissue Antigens 2014; 83: 267-272
  CrossrefPubMedGoogle Scholar
• 19 Spagnolo P, Renzoni EA, Wells AU et al. C-C chemokine receptor 2 and sarcoidosis: association with Lofgren’s syndrome. Am J Respir Crit Care Med 2003; 168: 1162-1166
  CrossrefPubMedGoogle Scholar