- Email: [email protected]
Lepra reaction presenting with inflammatory polyarthritis
APME-392; No. of Pages 3 apollo medicine xxx (2017) xxx–xxx
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/apme
Case Report
Lepra reaction presenting with inflammatory polyarthritis Abhishek Patil a,*, Sundeep Kumar Upadhyaya b, Nikunjkumar Dadhaniya a, Rohini Handa b a b
DNB Trainee, Department of Rheumatology, Indraprastha Apollo Hospitals, Delhi, India Senior Consultant, Department of Rheumatology, Indraprastha Apollo Hospitals, Delhi, India
article info
abstract
Article history:
Hansen's disease (HD) is still prevalent in many parts of India. It can be complicated by the
Received 30 December 2016
lepra reactions which are immunologically mediated episodes of acute or subacute inflam-
Accepted 5 January 2017
mation. Arthritis and tenosynovitis are under reported and under recognized manifesta-
Available online xxx
tions of these lepra reactions. Here we report a case of long standing HD presenting with polyarthritis and tenosynovitis as the initial manifestation of the lepra reaction.
Keywords:
© 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Hansen's disease Lepra reaction Arthritis Tenosynovitis
1.
Introduction
Hansen's disease (HD) is a chronic granulomatous disease caused by Mycobacterium leprae with predominant involvement of skin and nerves. Although the use of multi-drug therapy (MDT) has caused a significant decrease in the prevalence of leprosy worldwide, India continues to be a high prevalent region. As per NLEP report of 2014–2015, India had a prevalence rate of 0.69 per 10,000 population, with a total of 88,833 reported cases.1 However, the reported cases represent only the tip of the iceberg with most HD cases going unreported. The prevalence of musculoskeletal manifestations in leprosy vary with a range of 1–78% across various studies.2–4 Here, we
report a case of a patient with long standing leprosy presenting with polyarthritis and tenosynovitis due to a lepra reaction.
2.
Case description
Our patient was diagnosed with HD 6 months prior to presentation. He had a history of distal sensory loss with ulcerations in bilateral feet and thickened peripheral nerves (Fig. 1). Previously he had undergone nerve and skin biopsy, based on which he was treated with MDT. Ten days prior to presentation he had acute polyarthritis of hands with extensive tenosynovitis and olecranon bursitis (Fig. 2). He had no history of fever, rash or recent change in drug intake. He
* Corresponding author at: D-35, Seemant Vihar Apartments, Kaushambi, Ghaziabad 201010, UP, India. Tel.: +91 7838693975. E-mail address: [email protected] (A. Patil). http://dx.doi.org/10.1016/j.apme.2017.01.002 0976-0016/© 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Please cite this article in press as: Patil A, et al. Lepra reaction presenting with inflammatory polyarthritis, Apollo Med. (2017), http://dx.doi. org/10.1016/j.apme.2017.01.002
APME-392; No. of Pages 3
2
apollo medicine xxx (2017) xxx–xxx
Fig. 1 – Thickened right greater auricular nerve (red arrow) and ulcerations in both feet. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
Fig. 2 – Bilateral tenosynovitis and right ulnar bursitis.
denied a history of weight loss, urethral discharge, diarrhoea, or oral or genital ulcers. Laboratory investigations revealed Hb 12.2 g/dl, TLC 7500 cells/mm3, platelet count 2.83 lakhs/mm3 and ESR of 42 mm at end of first hour. His rheumatoid factor (RF) and anti-CCP were both negative. Based on the clinical presentation and examination findings he was diagnosed with type 1 (reversal) lepra reaction and was treated with a short course of low dose steroids with 20 mg/day of prednisolone followed by a taper over 2 months with continuation of MDT. He had a prompt response to the treatment with resolution of synovitis over a month.
3.
Discussion
HD is a chronic granulomatous disease, endemic in various parts of India. The awareness of the many possible clinical
scenarios of the disease amongst physicians is vital to prevent delayed diagnosis and treatment. Joint involvement in HD can be mono, oligo or polyarticular.5 The pathogenesis of joint involvement may involve immune-mediated lepra reactions, direct invasion of joint synovium by lepra bacilli or peripheral sensory neuropathy (Charcot joints).6 The immune response in leprosy is dynamic, with either increase or decrease in T-cell reactivity leading to immunological reactions termed lepra reactions. These reactions can present before, during and after the treatment of HD. The type I lepra reaction involves Th1 cell response with an increase of TNF-a, IFN-g, IL-2 and IL-4 cytokines. Alternatively, this reaction may be a downgrading reaction, or a reversal reaction, commonly occurring in patients with borderline or tuberculoid forms of leprosy. It then presents as an increase in pre-existing skin lesions or neuritis with no systemic features.
Please cite this article in press as: Patil A, et al. Lepra reaction presenting with inflammatory polyarthritis, Apollo Med. (2017), http://dx.doi. org/10.1016/j.apme.2017.01.002
APME-392; No. of Pages 3 apollo medicine xxx (2017) xxx–xxx
Type II lepra reaction (erythema nodosum leprosum) occurs in the lepromatous end of HD. This immune complex reaction is characterized by neutrophil infiltration and complement cascade activation. In 90% cases, it follows institution of MDT. Clinically, the patients have new crops of painful erythematous skin nodules along with systemic features such as fever, weight loss and others. Our patient was diagnosed with multibacillary leprosy when he had presented with thickened nerves, skin lesions and chronic non-healing ulcers in both the feet, suggesting sub-optimal response to treatment. After receiving MDT for 6 months, he developed acute polyarthritis and tenosynovitis. Arthritis in HD requires the careful exclusion of other causes before attributing it to HD itself. Male sex, absence of RF, antiCCP, and complete resolution of synovitis with short course steroids distinguish from rheumatoid arthritis in our patient. This case highlights the need for a high index of suspicion required to diagnose arthritis due to leprosy even in a patient with typical neuro-cutaneous manifestations. In the absence of established laboratory or imaging markers of disease, diagnosis of HD arthritis depends on exclusion of other causes. The prompt recognition of the condition would help to initiate appropriate management while avoiding complications.
Conflicts of interest The authors have none to declare.
references
1. NLEP – Progress report for the year 2014–15. (Online accessed on 9 August 2016 at http://nlep.nic.in/pdf/Progress%20report %2031st%20March%202014-15%20-.pdf). 2. Bonvoisin B, Martin JM, Bouvier M, Bocquet B, Boulliat J, Duivon JP. Articular manifestations in leprosy. Sem Hop. 1983;59:302–305. 3. Atkin SL, el-Ghobarey A, Kamel M, Owen JP, Dick WC. Clinical and laboratory studies of arthritis in leprosy. Br Med J. 1989;298:1423–1425. 4. Albert DA, Weisman MH, Kaplan R. The rheumatic manifestations of leprosy [Hansen disease]. Medicine. 1980;59:442–448. 5. Alam F, Emadi S. Case of arthritis secondary to leprosy. Springerplus. 2014;3:734. 6. Chauhan S, Wakhlu A, Agarwal V. Arthritis in leprosy by Sandeep Chauhan. Rheumatology. 2010;49:2237–2242.
Please cite this article in press as: Patil A, et al. Lepra reaction presenting with inflammatory polyarthritis, Apollo Med. (2017), http://dx.doi. org/10.1016/j.apme.2017.01.002
3
Available online at www.sciencedirect.com
ScienceDirect journal homepage: www.elsevier.com/locate/apme
Case Report
Lepra reaction presenting with inflammatory polyarthritis Abhishek Patil a,*, Sundeep Kumar Upadhyaya b, Nikunjkumar Dadhaniya a, Rohini Handa b a b
DNB Trainee, Department of Rheumatology, Indraprastha Apollo Hospitals, Delhi, India Senior Consultant, Department of Rheumatology, Indraprastha Apollo Hospitals, Delhi, India
article info
abstract
Article history:
Hansen's disease (HD) is still prevalent in many parts of India. It can be complicated by the
Received 30 December 2016
lepra reactions which are immunologically mediated episodes of acute or subacute inflam-
Accepted 5 January 2017
mation. Arthritis and tenosynovitis are under reported and under recognized manifesta-
Available online xxx
tions of these lepra reactions. Here we report a case of long standing HD presenting with polyarthritis and tenosynovitis as the initial manifestation of the lepra reaction.
Keywords:
© 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Hansen's disease Lepra reaction Arthritis Tenosynovitis
1.
Introduction
Hansen's disease (HD) is a chronic granulomatous disease caused by Mycobacterium leprae with predominant involvement of skin and nerves. Although the use of multi-drug therapy (MDT) has caused a significant decrease in the prevalence of leprosy worldwide, India continues to be a high prevalent region. As per NLEP report of 2014–2015, India had a prevalence rate of 0.69 per 10,000 population, with a total of 88,833 reported cases.1 However, the reported cases represent only the tip of the iceberg with most HD cases going unreported. The prevalence of musculoskeletal manifestations in leprosy vary with a range of 1–78% across various studies.2–4 Here, we
report a case of a patient with long standing leprosy presenting with polyarthritis and tenosynovitis due to a lepra reaction.
2.
Case description
Our patient was diagnosed with HD 6 months prior to presentation. He had a history of distal sensory loss with ulcerations in bilateral feet and thickened peripheral nerves (Fig. 1). Previously he had undergone nerve and skin biopsy, based on which he was treated with MDT. Ten days prior to presentation he had acute polyarthritis of hands with extensive tenosynovitis and olecranon bursitis (Fig. 2). He had no history of fever, rash or recent change in drug intake. He
* Corresponding author at: D-35, Seemant Vihar Apartments, Kaushambi, Ghaziabad 201010, UP, India. Tel.: +91 7838693975. E-mail address: [email protected] (A. Patil). http://dx.doi.org/10.1016/j.apme.2017.01.002 0976-0016/© 2017 Indraprastha Medical Corporation Ltd. All rights reserved.
Please cite this article in press as: Patil A, et al. Lepra reaction presenting with inflammatory polyarthritis, Apollo Med. (2017), http://dx.doi. org/10.1016/j.apme.2017.01.002
APME-392; No. of Pages 3
2
apollo medicine xxx (2017) xxx–xxx
Fig. 1 – Thickened right greater auricular nerve (red arrow) and ulcerations in both feet. (For interpretation of the references to color in this figure legend, the reader is referred to the web version of this article.)
Fig. 2 – Bilateral tenosynovitis and right ulnar bursitis.
denied a history of weight loss, urethral discharge, diarrhoea, or oral or genital ulcers. Laboratory investigations revealed Hb 12.2 g/dl, TLC 7500 cells/mm3, platelet count 2.83 lakhs/mm3 and ESR of 42 mm at end of first hour. His rheumatoid factor (RF) and anti-CCP were both negative. Based on the clinical presentation and examination findings he was diagnosed with type 1 (reversal) lepra reaction and was treated with a short course of low dose steroids with 20 mg/day of prednisolone followed by a taper over 2 months with continuation of MDT. He had a prompt response to the treatment with resolution of synovitis over a month.
3.
Discussion
HD is a chronic granulomatous disease, endemic in various parts of India. The awareness of the many possible clinical
scenarios of the disease amongst physicians is vital to prevent delayed diagnosis and treatment. Joint involvement in HD can be mono, oligo or polyarticular.5 The pathogenesis of joint involvement may involve immune-mediated lepra reactions, direct invasion of joint synovium by lepra bacilli or peripheral sensory neuropathy (Charcot joints).6 The immune response in leprosy is dynamic, with either increase or decrease in T-cell reactivity leading to immunological reactions termed lepra reactions. These reactions can present before, during and after the treatment of HD. The type I lepra reaction involves Th1 cell response with an increase of TNF-a, IFN-g, IL-2 and IL-4 cytokines. Alternatively, this reaction may be a downgrading reaction, or a reversal reaction, commonly occurring in patients with borderline or tuberculoid forms of leprosy. It then presents as an increase in pre-existing skin lesions or neuritis with no systemic features.
Please cite this article in press as: Patil A, et al. Lepra reaction presenting with inflammatory polyarthritis, Apollo Med. (2017), http://dx.doi. org/10.1016/j.apme.2017.01.002
APME-392; No. of Pages 3 apollo medicine xxx (2017) xxx–xxx
Type II lepra reaction (erythema nodosum leprosum) occurs in the lepromatous end of HD. This immune complex reaction is characterized by neutrophil infiltration and complement cascade activation. In 90% cases, it follows institution of MDT. Clinically, the patients have new crops of painful erythematous skin nodules along with systemic features such as fever, weight loss and others. Our patient was diagnosed with multibacillary leprosy when he had presented with thickened nerves, skin lesions and chronic non-healing ulcers in both the feet, suggesting sub-optimal response to treatment. After receiving MDT for 6 months, he developed acute polyarthritis and tenosynovitis. Arthritis in HD requires the careful exclusion of other causes before attributing it to HD itself. Male sex, absence of RF, antiCCP, and complete resolution of synovitis with short course steroids distinguish from rheumatoid arthritis in our patient. This case highlights the need for a high index of suspicion required to diagnose arthritis due to leprosy even in a patient with typical neuro-cutaneous manifestations. In the absence of established laboratory or imaging markers of disease, diagnosis of HD arthritis depends on exclusion of other causes. The prompt recognition of the condition would help to initiate appropriate management while avoiding complications.
Conflicts of interest The authors have none to declare.
references
1. NLEP – Progress report for the year 2014–15. (Online accessed on 9 August 2016 at http://nlep.nic.in/pdf/Progress%20report %2031st%20March%202014-15%20-.pdf). 2. Bonvoisin B, Martin JM, Bouvier M, Bocquet B, Boulliat J, Duivon JP. Articular manifestations in leprosy. Sem Hop. 1983;59:302–305. 3. Atkin SL, el-Ghobarey A, Kamel M, Owen JP, Dick WC. Clinical and laboratory studies of arthritis in leprosy. Br Med J. 1989;298:1423–1425. 4. Albert DA, Weisman MH, Kaplan R. The rheumatic manifestations of leprosy [Hansen disease]. Medicine. 1980;59:442–448. 5. Alam F, Emadi S. Case of arthritis secondary to leprosy. Springerplus. 2014;3:734. 6. Chauhan S, Wakhlu A, Agarwal V. Arthritis in leprosy by Sandeep Chauhan. Rheumatology. 2010;49:2237–2242.
Please cite this article in press as: Patil A, et al. Lepra reaction presenting with inflammatory polyarthritis, Apollo Med. (2017), http://dx.doi. org/10.1016/j.apme.2017.01.002
3