|Year : 2019 | Volume
| Issue : 2 | Page : 176-180
A commonly missed diagnosis: Poncet's disease
Vishal Mehrotra, Kriti Garg, Parvathi Devi, Shiv Chauhan
Oral Medicine and Radiology, Rama Dental College, Kanpur, Uttar Pradesh, India
|Date of Submission||15-Jan-2019|
|Date of Acceptance||19-Apr-2019|
|Date of Web Publication||24-Jun-2019|
Dr. Kriti Garg
117/K-68 Sarvodaya Nagar, Kanpur, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Tuberculosis (TB) is on the rise particularly in developing countries like India. The lung happens to be the most common site for the occurrence of the same, but extra pulmonary manifestation is not that uncommon. Joint involvement in TB is usually monoarticular, rarely may present as polyarthritis. Reactive arthritis in TB is known as Poncet's disease, a rare aseptic form of arthritis characterized by polyarticular impairment observed in patients with active TB, with no evidence of direct bacillary invasion of the joints. The condition is different from tuberculous arthritis which is usually monoarticular and is caused by direct tubercular involvement of the joint. A 40 year female patient reported with gradually enlarging swelling of left preauricular region and painful joints based on clinical history and investigations she was diagnosed with Poncet's disease.
Keywords: Monoarticular, polyarthritis, Poncet's disease, tuberculosis
|How to cite this article:|
Mehrotra V, Garg K, Devi P, Chauhan S. A commonly missed diagnosis: Poncet's disease. J Indian Acad Oral Med Radiol 2019;31:176-80
|How to cite this URL:|
Mehrotra V, Garg K, Devi P, Chauhan S. A commonly missed diagnosis: Poncet's disease. J Indian Acad Oral Med Radiol [serial online] 2019 [cited 2019 Jul 21];31:176-80. Available from: http://www.jiaomr.in/text.asp?2019/31/2/176/261080
| Introduction|| |
The incidence of tuberculosis (TB) has increased exponentially and has remained a major source of morbidity and mortality worldwide. Approximately 10-19% of extrapulmonary TB involves joints and bones. Almost half of these cases are spinal TB, followed by TB arthritis, TB osteomyelitis, and reactive arthritis; the latter, reactivearthritis, is also known as Poncet's Disease.
Poncet's disease (PD) is a rare syndrome first introduced in 1897 by the Frenchman Antonin Poncet, when he described a polyarthritis in an acute stage of TB, which resolved without joint damage. Continuous reports on patients with similar characteristics led authors to improve the definition, and in 1978, Bloxham and Addy defined PD as a para infective arthritis, but its existence has been questioned. However, many cases have been reported over the years. Poncet's disease is characterized by articular affection in patients diagnosed with TB, not related to direct invasion by the micro-organism, but to an immune reaction to the tuberculous protein, constituting a reactive arthritis.,
The exact mechanism underlying polyarthritis is not known, however the induction of cell-mediated immunity and/or autoimmunity are possible postulation. Almost all patients treated with antituberculous drugs have reported resolution of symptoms on therapy. This is an unusual and uncommon condition. The diagnosis needs strong clinical suspicion.,
This case is reported because of its rarity, and in a country such as India, one should keep this possibility in mind in patients with polyarthritis, as early recognition of this complication is of major importance to avoid delayed initiation of appropriate treatment.
| Case History|| |
A 40-year-old female patient reported to the department with a gradually enlarging asymptomatic swelling of left pre-auricular lymph node [Figure 1] along with painful knees, wrists and shoulder joints since the past 6 months. She also reported gradual deterioration over preceding months, resulting in slight difficulty in walking. The joint symptoms were present throughout the day with no diurnal variation. The intensity of pain in the larger joints including her upper lower limbs, elbow and knee joints has increased over period of time and got aggravated by joint movement and got relieved by taking rest and NSAIDs. Patient also gave a history of morning stiffness which usually lasted for half an hour. They also reported about the low-grade evening rise of temperature, loss of appetite and weight and productive cough with sputum during the same period. She also reported about the development of diffuse hyper-pigmented areas in form of small blackish macular spots all over her chest [Figure 2] and feet [Figure 3] since the past 6months, and they were gradually spreading with no other associated signs and symptoms. There was no history of trauma, conjunctivitis, bowel or bladder symptoms, or similar episodes in the past. There was neither family history of pulmonary tuberculosis rheumatic or autoimmune diseases.
|Figure 1: Frontal profile of patient reveals an extra oral swelling in the left pre-auricular region|
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Clinical examination revealed good general nutritional status and all the vital parameters were within normal limits. On extraoral examination there was an enlarged, firm, mobile, 3 × 3 cm lymph node mass in the pre-auricular area. No other significant lymphadenopathy was noticed. Liver and spleen were not palpable.
Based on the history and clinical presentation a provisional diagnosis of pulmonary tuberculosis with tuberculous lymphadenopathy and arthritis was taken into consideration.
Routine blood haemogram revealed hemoglobin 9.00 g/dl, WBC: 11 × 109/L with 80% granulocytes, 24% lymphocytes and 2% monocytes, ESR was 102 mm in the 1st hour. Radiograph of the chest [Figure 4] and the involved joints did not reveal any radiographic changes, there were no fractures or dislocations and bone and joints under vison appears to be normal [Figure 5]. The ultrasound of head and neck revealed [Figure 6] a multi-lobulated mass measuring 31×26×20mm seen in pre-auricular region. Area of necrosis/cystic lesion attenuation was seen in this lesion. Multiple lymph nodes measuring – 8 to 10mm in short axis diameter with loss in hilar pattern seen in left submandibular, bilateral upper jugular and bilateral mid jugular region (left > right). Central necrosis was also seen in few of these lymph nodes. Based on the ultrasound investigation reportwhich showed bilateral left upper and mid jugular necrotic lymphadenopathy with left preauricular lymph node mass, a possibility of tubercular/infective etiology most commonly was considered.
|Figure 6: Ultrasound shows area of necrosis and multiple lymphnodes of size 8 × 10mm in short with loss of hilar pattern|
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Later, CT examination of the head and neck [Figure 7] was performed, which also revealed involvement of preauricular lymph nodes in relation to left side. Sputum Ziehl-Neelsen (Z-N) staining was positive for AFB. Mantoux test was strongly positive (15 × 12 mm).
Serum uric acid was 5.6 mg%. Joint fluid aspiration revealed leukocyte count of 10 × 109/L and there were no crystals and the cultures were sterile.
Rheumatoid factor, anti-CCP antibodies, c-ANCA and p-ANCA, anti-nuclear antibody (ANF) and Anti-Streptolysin O (ASLO) titre were negative. Alanine Aminotransferase (ALT) 50 IU/L Aspartate Aminotransferase (AST) 46 IU/L and serum alkaline phosphatase 470 IU/L values were obtained.
Based on the clinical findings and investigations we arrived at a final diagnosis of Poncet disease with differential diagnosis of Inflammatory joint disease, erythema nodosum, viral arthritis, arthropod-borne arthritis, bacterial arthritis, reactive polyarthritis, gout and Lofgrens syndrome.
The lymph node in the left pre-auricular region was excised under general anesthesia [Figure 8] and the excised specimen was sent for histopathological examination [Figure 9]. Histopathological examination revealed a casseating granulomatous lesion suggestive of active tuberculosis [Figure 10].
Thus, the patient was confirmed with Poncet's disease and was referred to a physician for treatment. The patient was started on Streptomycin 0.75 mg, Isoniazid 300 mg, Rifampicin 450 mg and Pyrazinamide 1.5 g daily (2 SHRZ/7 HR). After a period of 1 month, the patient showed good improvement in her articular and systemic complaints including her bone pain, morning stiffness, and pigmentation over her chest, with evening rise in temperature and loss of appetite. There was also healing in the preauricular area where the sutures where placed at the time of surgical excision of the enlarged preauricular lymph nodes in relation to left side of her face [Figure 11].
|Figure 11: Post surgical healing after 1 month in left preauricular region|
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| Discussion|| |
Poncet's disease often affects young people and is slightly more common in women. Clinical findings include fever, malaise, and polyarthritis of large joints. Diagnosis of PD is generally difficult because it starts insidiously, progresses slowly, and has no extra-articular features. The most frequent clinical sign is the change in the range of motions with pain or painless. The fact that TB septic monoarthritis, in which Mycobacterium tuberculosis can be isolated from the joint, may complicate TB infection is widely known. On the other hand, active TB may also be complicated by a sterile reactive arthritis called PD, which is less recognized and therefore frequently missed.
In contrast to the usual tuberculous arthritis which is monoarticular, infectious and destructive, tuberculous rheumatism (Poncet's disease) is a non-destructive para- infective symmetric polyarthritis occurring in patients with active visceral or disseminated tuberculosis, in which there is neither evidence of bacteriological involvement of joint themselves nor any other known cause of polyarthritis detected. This finding was consistent with the clinical presentation in our case in which the patient reported to us symptoms of painful knee, upper/lower limbs, elbows and shoulder joints. The symptoms resolve completely with anti-tuberculosis therapy. In the present case as well after the initiation of anti-tubercular regimen, the patient reported marked reduction in bone pain, no morning stiffness of joints, increase in appetite, no evening rise in temperature and pigmentation over her chest started to reduce.
Considering the incidence of tuberculosis in our country the number of reported cases of Poncet's disease are few thereby confirming that the diagnosis needs strong clinical surmise. Poncet's initial concept of tubercular rheumatism was based on association of polyarthritis with:
- Active or inactive visceral tuberculosis, or
- Family history of tuberculosis or
- Presence of a true tubercular joint in any patient before, coincident with, or following a polyarthritis of any type.
The above criterion was met with many controversies, as it was very broad based and led to diagnostic inaccuracies. The very existence of such an entity was questioned by some. The pathogenesis of Poncet's disease is still uncertain but is considered to be immune cells mediated, since there is no direct bacteriological involvement of the joint itself. In the last decade, there has been accumulating experimental evidence implicating mycobacteria in the pathogenesis of arthritis. However, both host factors as well as mycobacterial antigens have been implicated. Poncet's disease has been considered to represent a hypersensitive immune response to tuberculoprotein, which may produce a reaction in the joint spaces, similar to that produced in skin in erythema nodosum occurring in active tuberculosis.
Since Poncet's disease apparently occurs only in a small proportion of patients with active tuberculosis, a genetic predisposition might be involved. In one patient with Poncet's disease, the HLA haplotype was DR4. Genes coding for this allele are associated with rheumatoid arthritis and may also influence the immune response to mycobacterial antigens. DR4+ patients are hyper-responsive to mycobacterial antigens. Poncet's disease might thus result from a genetically determined (HLA linked) hyper responsiveness to mycobacterial antigens disseminating into joint spaces., While host factors may be important in the pathogenesis of Poncet's Disease, the role of mycobacteria and their antigens also needs to be considered. Molecular mimicry between mycobacterial antigens and host tissues and the resulting immunological cross reactivity may also play a role. Antigenic similarity between a fraction of M. tuberculosis and human cartilage has been shown. A T cell mediated cross-reactive autoimmune response might also be operative in the pathogenesis of Poncet's disease.
The prognosis in Poncet's disease is, however, uniformly good, if the prime cause is treated promptly. In view of the high prevalence of tuberculosis in our country, it is possible that the condition is more common than is reported in the literature.,
| Conclusion|| |
In conclusion, the diagnosis of Poncet's disease remains clinical and is established on excluding other potential causes of arthritis in a patient with active tuberculosis. The complete resolution of arthritis of Poncet's disease on anti-tuberculosis therapy also furnishes further proof of the diagnosis. Correct identification of this rare complication of TB may avoid delayed initiation of appropriate treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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