Lofgren's syndrome is an acute form of sarcoidosis characterized by erythema nodosum, bilateral hilar lymphadenopathy (BHL), and polyarthralgia or polyarthritis. This syndrome is common among Caucasians but rare in the Korean population. A 44-year-old woman was admitted to our hospital complaining of polyarthralgia. A chest radiograph revealed BHL and nodular shadows. Angiotensin-converting enzyme levels were within the normal range. Tissue biopsy from a mediastinum lymph node showed noncaseating granulomas. We diagnosed her with Lofgren's syndrome, an acute form of sarcoidosis.
Sarcoidosis is a multi-systemic granulomatous disease with an unknown etiology characterized by its pathological hallmark [
A 44-year-old woman was admitted to our hospital in June 2011 complaining of arthralgia in both knees, elbows, and ankles. She had been diagnosed with hepatic cysts 10 years ago but was healthy before the appearance of bilateral polyarthritis of the knees 1 month before admission. She went to a nearby orthopedic clinic and reported temporary alleviation of the knee pain upon taking non-steroidal anti-inflammatory drugs (NSAIDs).
A physical examination showed mild swelling and tenderness in both ankles without limitation of range of motion. Laboratory studies showed elevated erythrocyte sedimentation rate (ESR, 39 mm/hr) and C-reactive protein level (CRP, 45.79 mg/L) (
Knee, ankle, hip, and hand X-ray findings showed periarticular osteoporosis (
Chest X-ray findings showed focal mediastinal widening in both the hilum and the right upper trachea (
A mediastinoscopic lymph node biopsy was performed. The obtained tissues of mediastinal lymph nodes showed noncaseating granuloma of epithelioid cells and the presence of giant cells (
Based on the bilateral hilar lymphadenopathy (BHL) with polyarthralgia, the patient was diagnosed with Lofgren's syndrome, although EN. NSAID treatment did not alleviate her symptoms for 1 week. Oral administration of prednisolone (10 mg/day) was initiated. The pain resolved within 1 week, and the levels of ESR and CRP were normalized after 2 weeks (12 mm/hr, 4.96 mg/L). The prednisolone dose was lowered 1 month later (5 mg/day). The pain and joint swelling were improved, but she sometimes felt mild pain in the joints, and BHL was still seen on simple chest radiography after 2 months. For these reasons, prednisolone administration has been continued for 8 months until now. No recurrence has been observed since then.
While sarcoidosis occurs worldwide, higher annual incidence rates have been reported among United States Blacks, the Irish, and Scandinavians [
Acute polyarthritis occurs in 10% to 39% of patients with sarcoidosis, particularly in the early phases of the disease, which could be the presenting feature. BHL is almost invariably present in patients with acute sarcoid arthritis; fever and EN are present in 90% and 60% of the cases, respectively [
In 1953, Lofgren characterized 212 adult patients of BHL who were practically regarded as having sarcoidosis based on the absence of tuberculosis. Lofgren demonstrated that EN was present at the onset of the disease in 113 cases in which articular symptoms were common (101 cases, 89%). There was either pain only in the joints (20%), or pain accompanied by swelling (69%) [
Arthritis in sarcoidosis is usually symmetrical; the ankles are involved in more than 90% of the cases; the knees, small joints of the hands or feet, wrists, and elbows are involved in 15% to 40% [
Diagnosis of Lofgren's syndrome is not complicated but straightforward when the characteristic triad (BHL, polyarthritis, and EN) is present. Nevertheless, the disease may be somewhat difficult to diagnose because variant forms of this syndrome are often observed. In addition, uveitis and elevated levels of ACE, although useful clues for diagnosing sarcoidosis, are often absent in patients with Lofgren's syndrome (eye involvement, 5%; elevated ACE levels, 50%) [
Lofgren's syndrome is described as a symmetric polyarthritis associated with hilar adenopathy with or without EN. Regarding the case described herein, the patient showed polyarthritis, but not EN. Among 49 patients studied by Gran et el. [
Lofgren's syndrome was regarded as a self-limiting disease that is generally resolved within the first year, with the mean duration ranging from 3 weeks to 3.7 months [
Polyarthralgia and EN are often observed in various collagen diseases, including rheumatoid arthritis. Thus, Lofgren's syndrome, an acute form of sarcoidosis, must be differentially diagnosed. It is also noteworthy that, in Korean patients with Lofgren's syndrome, the characteristic triad is not always present. Biopsy from skin rash, lung nodules or lymph nodes should be considered in order to reach an early and precise diagnosis.
No potential conflict of interest relevant to this article was reported.
Simple radiography of (A) bilateral hands, (B) knees, (C) ankles, and (D) hip showed periarticular osteoporosis. There is no swelling or any other abnormal findings.
(A) Simple chest radiography showed mediastinal widening in both hila and in the upper right trachea (arrows). (B) Chest computerized tomography showed multiple enlarged lymph nodes in the mediastinum and in both hilar areas (arrows).
Bone scintigraphy showed mildly increased uptakes in bilateral hands, wrist, shoulders, and small joint areas.
A biopsied mediastinal lymph node treated using hematoxylin and eosin stain showed noncaseating granuloma of epithelioid cells with multinucleated giant cells (A, ×40; B, ×400).
Laboratory findings on admission
C3, third components of complement; C4, fourth components of complement; dsDNA Ab, double-stranded DNA antibody; Anti-CCP Ab, anti-cyclic citrullinated protein antibody.