|Year : 2020 | Volume
| Issue : 4 | Page : 23-27
Poncet's disease (tubercular rheumatism): a narrative review
Ganesh Singh Dharmshaktu
Department of Orthopaedics, Government Medical College, Haldwani, Uttarakhand, India
|Date of Web Publication||29-Dec-2020|
Ganesh Singh Dharmshaktu
Department of Orthopaedics, Government Medical College, Haldwani, Uttarakhand
Source of Support: None, Conflict of Interest: None
Poncet's disease or tubercular rheumatism is uncommon, and there are not many related studies. No consensus has been achieved with respect to its etiopathogenesis. The low prevalence of this Poncet's disease even in endemic regions suggests hidden mechanisms that require further research to fully comprehend this enigmatic disorder. Careful identification of primary focus of tubercular infection, clinical suspicion and knowledge of this disorder is crucial for prompt identification and treatment. This article aims to provide an overview of the recently published articles about Poncet's disease. The article attempts to highlight key features of the disease for better understanding of this peculiar disorder.
Keywords: mycobacterium tuberculosis; Poncet′s disease; review; synovitis; tubercular rheumatism; tuberculous arthritis
|How to cite this article:|
Dharmshaktu GS. Poncet's disease (tubercular rheumatism): a narrative review
. Clin Trials Orthop Disord 2020;5:23-7
| Introduction|| |
Poncet's disease (PD) or tubercular rheumatism is an uncommon presentation in patients of primary visceral or disseminated tuberculosis.  The entity, first described by Antonin Poncet as polyarthritis associated with tuberculosis, currently is used to describe only cases with reactive arthritis associated with tuberculosis without direct bacteriological confirmation. , It usually presents as nondestructive arthritis where evidences of bacterial presence are negative following exclusion of other differentials. In contrast to tubercular arthritis directly affecting the joint, PD is usually polyarticular and is found to respond well to anti-tubercular treatment (ATT). Few other important features that may help differentiate PD from rheumatoid arthritis and other rheumatic conditions (as rheumatoid factor [RA] factor and anti-cyclic citrullinated protein [CCP] can be positive in both cases) and go in favor of PD are reactive arthritis at younger age, arthritic symptoms at initial phase of infection (not weeks after or after infection subsides), dramatic response to ATT (improvements noted in weeks or few months).  Very few cases of PD have been reported in the medical literature and many cases, experts believe, might be underreported even from endemic countries. Severe form of PD may result in protracted course with varying morbidity and deformities. These cases require high index of suspicion for diagnosis and careful exclusion of other probable conditions.
A search in the PubMed central (www.ncbi.nlm.nih.gov/pmc) and Google Scholar database for full text articles with search query "Poncet's disease" the year from 2005 to 2020 was done. Only the articles concerning PD, after going through full-texts, were included. The objective of the review was to provide readers a quick overview of recent updates on PD for better acknowledgement and identification of this uncommon condition.
| Pathogenesis|| |
As the exact pathogenesis is not clear, various theories try to explain the probable etiopathogenesis of PD. Genetic theory is suggested by some due to its selective and rare affliction in an otherwise common disease of high prevalence. Human leukocyte antigen (HLA-DR3 and 4 haplotype) links have been associated with its causation.  Case control study of cases of PD against normal subject also highlights linkage of HLA (HLA-B27, DQB*0301) with PD and further research is advocated for more comprehensive information. ,
Immunological theory suggests hypersensitivity response to tubercular proteins as another mechanism.  There seems to exist cross reactivity and immune response due to antigenic similarity between human cartilage and mycobacteria.  Besides, intra-articular injection of heat dessicated tuberculosis (TB) bacilli in animals is shown to promote synovitis response substantiating previous theories.  It has been observed that in cases of bladder carcinoma receiving attenuated Bacillus Calmette-Guerin (BCG) intra-vesical instillation, 0.55% of these develop reactive polyarthritis.  This fact also supports link between mycobacterial antigen response and PD and should be investigated further. Reactive arthritis has been associated with HLA-B27 and association between HLA-B27 and DQB* 0301 alleles was also noted in PD.  Cell mediated immune response to tubercular antigen and probable molecular mimicry between mycobacteria heat shock protein 65 and human heat shock protein has been implicated as another theory.  Mycobacterial degradation products and overt mycobacterial DNA are found to be present in affected joint synovium.  Experts also believe the presence of mycobacterial sequences in normal and inflamed joint relates to trafficking of antigens from BCG vaccination.  The linking of PD with rheumatoid arthritis has also been substantiated. 
| Clinical Presentations|| |
The presentation of PD is reactive, aseptic, non-destrcutive arthritis of large joints. Other common presentation may be pauci-articular arthritis, whereas axial joints are usually spared.  Lymphadenopathy, fever, and erythema nodosum are other notable associations. , In an extensive review of literature, Kroot et al.  noted most cases were male and most common locations were knee, ankle and wrist. Septic arthritis was ruled out by culture in very few cases and only one case had septic tubercular arthritis along with the joint PD. A large study including 23 cases of PD described most cases presenting with oligoarthritis followed by polyarthritis.  Ankle joint was commonest affected site and systemic symptoms were absent in 48% cases, while Mantoux test was positive in 81% cases. A recent systematic review of the cases in medical literature included 199 patients with PD including author's case. Mean age was 33.7 years (range 2-78 years) and most cases (42.2%) were males. Most number of cases (35%, n=70) were reported from India followed by Brazil. In 96.5% cases, specific site of tuberculosis was mentioned, 56.8% had extrapulmonary TB and lymph node involvement was found in 42% cases. Ankle joint was common involvement followed by knee and wrist and lung was common associated involvement. Usually first line ATT works in most cases, but second line can be used in multi-drug resistant TB as was used in pulmonary multi-drug resistant case with small joint involvement.  The cases with other disorders closely associated with TB such as human immunodeficiency virus have also presented with painful inflammatory arthritis, finally diagnosed as PD.  Localized immune phenomena without organism isolation is common in two tubercular forms- tuberculids (cutaneous immune reactions in high cell mediated immunity patients) and PD. A delayed type hypersensitivity reaction in from of papulonecrotic tuberculid and PD was reported in a recent case.  The other epiphenomena associated with tuberculosis such as erythema induratum of Bazin or livedo reticularis in conjunction with an extraskeletal tubercular focus and polyarthritis may suggest PD and require ATT trial.  The diagnosis of PD, when presented with history of watery diarrhea and symmetrical multiple joint (including small joints of hand) arthritis, may be difficult and requires careful exclusion of reactive and rheumatoid arthritis.  Paediatric cases are another underrepresented groups with few reports. In a large case pool of 462 cases of pediatric rheumatologic disorders from South Africa, only four cases of PD were described.  Some of the described and notable cases have also been included in tabulated form in the article [Table 1].
|Table 1: A few notable cases described with key details from year 2007 onwards|
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| Recent Updates In Diagnosis|| |
No accepted diagnostic criteria exist for PD that invariably presents as reactive arthritis in the presence of extra-articular tuberculosis. Recent studies have attempted to form a diagnostic criteria following study of all known factors contributing to diagnosis of PD. The criteria by Sharma et al. had two essential, two major and two minor criteria and are simple in practical application. ,, Probable cases are called definitive only following positive response to ATT. The previous criteria given by Novaes et al. are also there but little used nowadays.  Diagnostic criteria were suggested to improve and strengthen another criteria used to diagnose PD. Utility of criteria by Sharma was used in a subsequent series of five cases and concluded that if the case does not fit into "definite" category, the case should be kept in close observation with regular reconsideration by using the criteria.  One case of atypical seropositive RA was found and was termed "pseudo Poncet's disease" by authors. 
High titers of rheumatoid factor and anti-CCP have been found in handful of cases that might be confusing as they mimic RA. Describing their case with high rheumatoid factor and anti-CCP in the setting of positive sample for TB monoarthritis have been found to respond to ATT. Occasional reports of positive anti-CCP in cases of tuberculosis are found and this fact should be remembered in these contexts.  The differentiation of PD from another uncommon disorder called Löfgren's syndrome (acute presentation of sarcoidosis) is challenging and an algorithm was advised by Garg et al.  in this regard. They distinguished 10 cases as Löfgren syndrome and 8 cases as PD out of 18 cases of acute ankle inflammatory arthritis. This is important as the treatment is entirely different for two conditions - glucocorticoid, methotrexate, hydroxychloroquin in Löfgren's syndrome and ATT in PD. Equally important is careful exclusion of differential diagnosis such as other causes of arthritis of viral, bacterial, arthropod-borne etiology, reactive arthritis, gout and chronic arthritis.  In absence of conclusive diagnosis, presence of surrogate findings such as reactive immunologic reactions, arthritis and erythema nodosum, may be suspected for tubercular infection as respiratory symptoms may be absent at presentation or may follow later.  In a case, presence of erythema nodosum and polyarthritis with mediastinal widening due to lymphadenopathy on CT was later diagnosed as PD.  Lymph node biopsy, in presence of lymphadenopathy is important and has been found to reveal epitheloid granuloma or acid fast bacillus in many cases.  Synovial biopsy, either surgical (open or arthroscopic) or ultrasound based, is a critical step to rule out many joint pathologies and should be more widely available and hurdles regarding this should be remedied. 
| Treatment|| |
The fortunate fact is that PD is a non-destructive, transient and migratory mono or polyarthritis without organism isolation that resolves without any structural articular damage. Anti-tubercular treatment is the mainstay of treatment. Resolution of symptoms was usually noted with 1 week to 4 months of initiation of ATT and no case developes irreversible arthritis. ,, In presence of chronic inflammation with no relief on prolonged course of treatment, the diagnosis of PD requires reevaluation. Defaulter to ATT treatment may also develop PD and restart of ATT may be done in such cases. Cases once treated as PD, may rarely be diagnosed as RA in future and for which disease modifying drugs might be used then.  In another case of PD treated with ATT for relief in polyarthralgia and low back pain, adalimumab was administered for remaining enthesitis and tendinitis.  Development of PD after BCG instillation for urogenital malignancy is also reported in the literature with final diagnosis of PD and subsequent improvement on BCG discontinuation and ATT. 
PD cases are less in number despite high tuberculosis incidence in developing countries may signify either underreporting, as the total reported cases are in hundreds only, or the unclear pathogenesis that makes it a selective disorder. Most cases, however, are managed well conservatively without long-term morbidity or arthritis.  Occasionally, ATT defaulters can also present with polyarthalgia like symptoms and careful history and previous investigations should be checked and new ones advised to rule out tuberculosis and in worst case scenario a drug-resistant one.  In one case on tumor necrosis factor alpha therapy for ulcerative colitis and later presenting with diffuse joint pain along with fever, erythema nodosum and lymphadenopathy, the biopsy of which was positive for TB. Adalimumab probably reactivated the tubercular lymphadenitis in that case.  The polyarthralgia may mimic rheumatoid arthritis and may complicate the things as tubercular cases may also show positivity to rheumatoid factor (8-60%) and anti-CCP (7-39%).  The other benefit of early diagnosis of PD is prevention of further complication such as pulmonary TB or pleural effusion in late stages as described in a previous report.  Occult abdominal TB with hyper-ferritenmia mimicking adult onset Still's disease underlines importance of looking beyond joints and careful exclusion of PD.  Death has been rare complication in healthy patient soon after the diagnosis of disseminated tuberculosis and bone marrow examination showing haemophagcytic lymphohistiocytosis in the setting of PD. 
| Conclusion|| |
Acknowledgement of this condition and anticipation in known cases of tuberculosis in the endemic region shall be important for early diagnosis. Cases with arthritis and negative culture may also be looked for systemic tuberculosis and PD should be kept as an uncommon differential diagnosis.
GSD contributed to the conception, design, manuscript preparation and manuscript review and approved the final version of the manuscript.
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