. 2018; 5(10): 000942.
Published online 2018 Oct 24. doi: 10.12890/2018_000942
PMCID: PMC6346892
PMID: 30755978

Systemic Sarcoidosis Associated with Exposure to Borrelia burgdorferi in a 21-Year-Old Man


A 21-year-old male forestry worker with a history of recent retrobulbar headaches and a droopy left eyelid was seen at the emergency department because of progressive bilateral swelling and redness of his legs. Previous analysis of his headaches by a neurologist showed no abnormalities and the complaints were interpreted as non-specific, cluster-like headaches.

Over the previous 3 weeks, the patient had experienced progressive swelling, redness and skin peeling of both legs. In addition, his symptoms included a stinging pain behind his sternum, fatigue, weight loss, loss of appetite, thirst and nycturia, although the headaches had stopped. Due to his profession, he frequently encountered tick bites. He used no medication, had stopped smoking 2 years earlier and reported no alcohol or drug use.

Examination was unremarkable except for bilateral pitting oedema of the lower legs and diffuse, confluent, flat, red and scaling lesions. The patient further presented with a frequent dry cough.


The combination of symptoms and bilateral hilar adenopathy on chest X-ray (Fig. 1) were indicative of sarcoidosis. Additional laboratory tests, imaging studies and an endoscopic ultrasound (EUS) with lymph node biopsies were performed (Table 1). Most notably, the laboratory results showed an elevated level of creatinine and ionized calcium, in keeping with the systemic effects of sarcoidosis. Other haematological, endocrine, auto-immune and infectious causes (tuberculosis, HIV, lues) were excluded (data not shown). A definite diagnosis of systemic sarcoidosis with involvement of the lungs, skin and kidneys was made when non-caseating granulomas were described in the lymph node and skin biopsies. While waiting for the results, the patient received hyperhydration therapy and infusion with bisphosphonates for his hypercalcaemia, with minimal effect. However, after he started high-dose prednisolone, the hypercalcaemia as well as renal function normalised within only 2 days, further supporting the diagnosis of sarcoidosis.

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The patient’s previously unexplained neurological complaints in combination with his job as a forestry worker in an area with a high tick density led to the decision to further pursue diagnostics for Lyme disease. A lumbar puncture was performed 2 days after hospitalization. We found a solitary, immunoblot-confirmed Borrelia burgdorferi IgG response in the serum, and a positive IgG cerebrospinal fluid (CSF)/serum ratio. Together with signs of inflammation in the CSF, these results are indicative of recent exposure to or infection with B. burgdorferi and local antibody production in the CSF.
The patient was treated with intravenous ceftriaxone for 2 weeks followed by 30 days doxycycline per os in addition to the immunosuppressive therapy for sarcoidosis, with the aim of preventing a flare up of a possible dormant/lingering B. burgdorferi infection. The patient recovered quickly within a few days of starting a high-dose prednisolone regime, and was discharged free of symptoms and in good health.


Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology, most commonly affecting young adults who generally present with bilateral hilar lymphadenopathy, pulmonary infiltrations, and skin or eye lesions[]. It has been hypothesised before that an infection with B. burgdorferi, the causal agent of Lyme disease, could act as a trigger for sarcoidosis[]. A Japanese study showed an association with sarcoidosis and B. burgdorferi infection in a region where Lyme borreliosis is endemic[]. In 2000, Danish physicians described the case of a man with neuroborreliosis who developed Löfgren’s syndrome and reacted well to treatment with doxycycline[]. In patients with cutaneous sarcoidosis, Borrelia-like organisms have been detected in skin tissue by focus-floating microscopy[].

This case is a rare presentation of sarcoidosis in a healthy young man with neuroborreliosis as a putative trigger. Unfortunately, the presence of B. burgdorferi DNA could not be confirmed by PCR, as the sensitivity of these tests is notoriously low (around 40%). In retrospect, the cluster-like headaches reported by the patient a few months before his visit to the emergency department could be explained by a recent episode of neuroborreliosis. This unusual case suggests we should maintain a high index of suspicion for underlying infectious processes like neuroborreliosis in patients with new-onset sarcoidosis before starting immunosuppressive regimens.


1. Iannuzzi MC, Rybicki BA, Teirstein AS. Sarcoidosis. N Engl J Med. 2007;357:2153–2165. [PubMed]
2. Jacob F. Could Borrelia burgdorferi be a causal agent of sarcoidosis? Med Hypotheses. 1989;30:241–243. [PubMed]
3. Ishihara M, Ohno S, Ono H, et al. Seroprevalence of anti-Borrelia antibodies among patients with confirmed sarcoidosis in a region of Japan where Lyme borreliosis is endemic. Graefes Arch Clin Exp Ophthalmol. 1998;236:280–284. [PubMed]
4. Derler AM, Eisendle K, Baltaci M, et al. High prevalence of ‘Borrelia-like’ organisms in skin biopsies of sarcoidosis patients from Western Austria. J Cutan Pathol. 2009;36:1262–1268. [PubMed]
5. Klint H, Siboni AH. Borreliosis associated with Lofgren’s syndrome. Ugeskr Laeger. 2000;162:4154–4155. [PubMed]

I omitted tables 1 & 2 (diagnostic findings) which may be viewed in the link at the top of the page.
To understand sarcoidosis, please see:  Excerpt:
Sarcoidosis (pronounced SAR-COY-DOE-SIS) is an inflammatory disease characterized by the formation of granulomas—tiny clumps of inflammatory cells—in one or more organs of the body. When the immune system goes into overdrive and too many of these clumps form, they can interfere with an organ’s structure and function. When left unchecked, chronic inflammation can lead to fibrosis, which is the permanent scarring of organ tissue.This disorder affects the lungs in approximately 90% of cases, but it can affect almost any organ in the body. Despite increasing advances in research, sarcoidosis remains difficult to diagnose with limited treatment options and no known cure.
When patients are first diagnosed, they often present with a classic set of signs described as Lofgren’s Syndrome:
  • Fever
  • Enlarged lymph nodes
  • Swollen and painful joints, arthritis
  • Erythema nodosum, raised, red, and tender bumps to form on the skin, usually on the front of the legs. Nearby joints are often swollen and painful.

Common Signs:

Because sarcoidosis so often affects the lungs, other common symptoms of sarcoidosis include shortness of breath, wheezing, and chronic cough. Some patients will also experience chest pain and others will have no signs at all, even when inflammation is present.

Other Symptoms:

Because sarcoidosis can affect any organ in the body, a wide variety of symptoms can be seen, including:

  • Fatigue
  • Unexplained weight loss
  • Night sweats
  • Overall feeling of sickness
  • Irregular heart beat
  • Swollen legs
  • Headaches
  • Visual problems
  • Weakness or numbness of an arm, leg, or part of the face
  • Discoloration of the nose, cheeks, lips, and ears
  • Scaly-appearing skin rash
  • Joint pain
  • Muscle swelling and soreness
  • Arthritis
  • Burning, itching, tearing, or pain in the eyes
  • Red eyes
  • Sensitivity to light
  • Blurred vision

Note that this is not an all-encompassing list. There are many more signs and symptoms that can be seen in patients depending on the organs involved.

Please remember that just because the authors state this is a rare case, nobody’s keeping score.  Since testing misses so many cases, people are not getting diagnosed.  Again, authors would be much more accurate by stating that this is the first recorded case.  There may be many, many more, that didn’t make it into the literature.  This is true for every aspect of Lyme/MSIDS.