Abstract

---

Sarcoidosis is a multisystem disorder which rarely affects the musculoskeletal system. The presentation as chronic arthritis is even rarer and leads to significant disability in patients with large joint involvement, who already have a compromised respiratory reserve. We report a case of bilateral hip sarcoid arthritis treated by total hip replacement which led to a good functional outcome. Total hip replacement leads to significant lifestyle improvement by reducing the total energy expenditure involved in activities of daily living.

Introduction

---

Sarcoidosis is a disease of unknown etiology involving multiple organs usually lungs, lymph nodes, skin, eye and rarely musculoskeletal system. Osteoarticular manifestations of sarcoidosis may be specific or nonspecific. They may be the presenting feature or may occur late after onset, either isolated or combined with other clinical manifestations. Clinical features of joint involvement are found in 14% of cases at presentation, and up to 38% during follow-up [1]. The frequency of muscle and bone involvement at presentation is low (<1%), and is found in 5–13% of patients during follow-up [1]. Locomotors involvement is often subclinical or not clinically recognized because of mild or unspecific symptoms in spite of a high prevalence on biopsy, as shown for muscle involvement [2]. There are earlier several reports of large joint arthritis in this condition, but we believe this is the first report of a bilateral hip sarcoid arthritis treated by total hip replacement.

Case Report(s)

---

In 2001, a 40 year old house wife presented with complaints of backache, intermittent low grade fever, malaise, weakness and pain in joints predominantly involving hip joints since 4 years. She was being treated as a case of seronegative arthritis with only NSAIDS. She even had difficulty in performing daily activities. On general examination, she was found to have multiple erythematous maculopapular lesions on extensor surfaces (Fig 1A & B), which was being treated as psoriasis.

On local examination of the hip, she had a waddling gait with bilateral, fixed flexion deformity of 10^oand fixed adduction deformity of 10^o. There were no rotational movements possible. Radiographs showed bilateral concentric reduction of joint space with subchondral sclerosis without any osteophytes. The shape of the acetabulum and the femoral head were maintained(Fig 2).

A bilateral total hip replacement was planned for which a pre-anesthetic check up was undertaken. Chest radiographs (Fig 3) showed bilateral hilar adenopathy with fibrotic lung lesion, which was suspicious of sarcoidosis. To further evaluate a CT of thorax (Fig 4) was done which revealed mediastinal lymphadenopathy with interstitial tissue involvement.

The dermatology consultation was soughted for the skin lesions. The biopsy of the erythematous skin lesion showed non-caseating epitheloid granuloma. Kveim test was positive. These faetures were consistent with sarcoidosis. Laboratory investigations further revealed hemoglobin of 14.4 gm%, total leucocyte count of 11000/cc, Neutrophil-56, Lymphocytes-32, Eosinophil-8, Basophil-2, Monocyte-2. The blood film showed normocytic normochromic red blood cells. However the platelet count was raised. ESR was 22mm in first hour with random blood sugar value of 99mg/dl. Serum creatinine was 1.1 mg/dl. ASO titre and C-reactive protein levels were with in normal limits. Rhematoid factor and Anti Nuclear Antibody were also negative. Anti-double-stranded DNA antibody was 3.6-IU/ml. The patient was diagnosed as a case of sarcoidosis affecting bilateral hips. Considering the morbidity of the patient, bilateral total hip replacement was planned. No other medical treatment was given preoperatively. Patient underwent bilateral, staged uncemented total hip replacement (Fig 5), three months apart. After 6 months of surgery she was allowed to walk without any aids. At the latest follow up (after 8 years), the patient had a satisfactory functional outcome with normal daily activities.

Discussion

---

Sarcoidosis is a disease of unknown etiology involving multiple organs usually lungs, lymph nodes, skin, eye and less frequently the musculoskeletal system. The disease commonly affects between the ages of 20 to 40 years. It has a wide spread geographic distributions, but is relatively rare in the Southeast Asians. Bone lesions are observed in only 5% of sarcoidosis patients and joint manifestations, including arthritis and periarthritis, occur in 14–38% of patients [1, 3–6]. Arthralgias are even more common (70%) [1]. Sarcoid rheumatic involvement is generally divided into acute and chronic types [1, 3–6]. The most common form of joint involvement is an acute polyarthritis/periarthritis. The arthritis may be migratory, intermittent or additive in time, and can precede other manifestations of sarcoidosis by several months. These clinical presentations can, therefore, resemble reactive arthritis [7], rheumatoid arthritis or even spondylarthropathies [5]. More commonly, however, it is non-migratory and accompanied by other signs of sarcoidosis [1], known as the Lofgren’s syndrome [7–9]. Chronic sarcoid articular involvement is rare and appears to affect only 0.2% of sarcoid patients [10, 11]. The chronic sarcoid joints usually present with other complications of sarcoidosis like, chronic cutaneous sarcoidosis [5] and are common in black patients [4, 5]. Medium-sized and large joints are often affected symmetrically and a simultaneous tenosynovitis may occur. Some cases have been described with severe destructive arthropathy requiring total arthroplasty [12]. Synovial biopsy shows non-caseating granulomas. For a typical case, the diagnosis of sarcoidosis is made by a combination of clinical, radiological and histological findings. In a young adult with constitutional complaints, respiratory symptoms, erythema nodosum, blurred vision and bilateral hilar adenopathy the diagnosis is almost always sarcoidosis [13, 14]. The ESR is raised and the kveim test may be positive. In cases of acute sarcoid arthritis, many times the diseased joint responds to NSAID with cold packs and training physical rehabilitation programme. [4]. The outcome of articular involvement is generally favourable as recovery is uneventful, generally within 1–6 months [4]. According to open label studies, sarcoid arthritis has also been treated efficaciously by systemic glucocorticoids and colchicine (oral or intravenous) [10]. Periarthritis is often responsive to rest, cold application and NSAIDs, whereas glucocorticoids are generally not needed nor recommended. A chronic destructive synovitis may need treatment with glucocorticoids intraarticularly or systemically, and, in the latter case, is probably best combined with methotrexate or azathioprine, though firm evidence from randomised controlled trials is lacking [1, 3–6]. Possibly, there is an indication for an anti-tumour necrosis factor (TNF)-a strategy in selective cases with chronic destructive arthritis in the near future. But the mechanical problems of severe destructive arthritis of major joints of lower limb like hip joint challenge the usual activities of daily living by restricting the mobility of the individual. The energy expenditure involved in performing routine activities dramatically increases in such patients, who already have a poor respiratory reserve due to the interstitial lung disease.  The quality of life improves dramatically in most of the arthritic patients after the joint replacement surgery [15, 16, 17]. The present case is doing well even after eight years of total hip arthroplasty.

Conclusion

---

This case highlights the fact that there should be a high index of suspicion in any young female patient presenting with non specific arthritis, towards the possibility of sarcoidosis. Sarcoidosis can be treated effectively if diagnosed early in the course of the disease. A planned artroplasty at appropriate time can significantly improve the lifestyle of patients suffering from chronic destructive arthritis.

Abbreviations(s)

---

NSAID: Non-Steroidal Anti-Inflammatory Drugs
CT: Computed Tomography
ESR: Erythrocyte Sedimentation Rate
ASO: Anti-streptococcal 'O' Antibody titer

Acknowledgement(s)

---

None

Authors contribution(s)

---

BKSS and RS managed the patient and performed the surgery.SD collected the data of the patient. NT reviewed the litarature. SD,SKT and NT  prepared the manuscript. All authors have read the article and aprooved. 

References

---

1. West SG, Kotzin BL. Sarcoidosis. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology, 3rd Edn. Edinburgh, Mosby Elsevier, 2003; pp. 1735–1752.
2. Silverstein A, Siltzbach LE. Muscle involvement in sarcoidosis. Arch Neurol 1969; 21: 235–241.
3. Barnard J, Newman LS. Sarcoidosis: immunology, rheumatic involvement, and therapeutics. Curr Opin Rheumatol 2001: 84–91.
4. Awada H, Abi-Karam G, Fayad F. Musculoskeletal and other extrapulmonary disorders in sarcoidosis. Best Pract Res Clin Rheumatol 2003: 971–987.
5. Pettersson T. Rheumatic features of sarcoidosis. Curr Opin Rheumatol 1998: 73–78.
6. Wilcox A, Bharadwaj P, Sharma OP. Bone sarcoidosis. Curr Opin Rheumatol 2000: 321–330.
7. Glennas A, Kvien TK, Melby K, et al. Acute sarcoid arthritis: occurrence, seasonal onset, clinical features and outcome. Br J Rheumatol 1995; 34: 45–50.
8. Lofgren S, Lundback H. The bilateral hilar lymphoma syndrome. A study of the relation to age and sex in 212 cases. Acta Med Scand 1952; 142: 259–264.
9. Mana´J, Gomez-Vaquero C, Montero A, et al. Lofgren’s syndrome revisited: a study of 186 patients. Am J Med 1999: 107–245.
10. Torralba KD, Quismorio FP. Sarcoid arthritis: a review of clinical features, pathology and therapy. Sarcoidosis Vasc Diffuse Lung Dis 2003; 20: 95–103.
11. JamesDG, Neville E, Carstairs LS. Bone and joint sarcoidosis. Semin Arthritis Rheum1976; 6: 53–81.
12. Sokoloff L, Bunim JJ. Clinical and pathological studies of joint involvement in sarcoidosis. N Engl J Med 1959; 260: 841–847.
13. John CJ, Michele TM: the clinical management of sarcoidosis. A 50 years experience at the Johns Hopkins Hospital, Medicine 78: 65,1999.
14. Judson MA et al : Defining organ involvement in sarcoidosis : The ACCESS proposed instrument sarcoidosis vasc diffuse : Lung Dis 16:75,1999.
15. Callaghan JJ, Dysart SH, Savory CG: The uncemented porous coated anatomic total hip prosthesis : Two years results of a prospective consecutive series, J Bone Joint  Surg 70 A: 337, 1998.
16. Charnley J, cupic Z: the nine and ten years result of the low friction arthroplasty of the hip. Clin orthop 95:9,1973.
17. Schmalzried TP, Wessinger SJ, Hill GE, Harries WH: The screw Fixation: five years radiographic analysis of primary cases. J Arthroplasty 9:235,1999.

Source(s) of Funding

---

No funds from any agencies or institutional bodies were received in support of this study.

Competing Interests

---

Nil

Disclaimer

---

This article has been downloaded from WebmedCentral. With our unique author driven post publication peer review, contents posted on this web portal do not undergo any prepublication peer or editorial review. It is completely the responsibility of the authors to ensure not only scientific and ethical standards of the manuscript but also its grammatical accuracy. Authors must ensure that they obtain all the necessary permissions before submitting any information that requires obtaining a consent or approval from a third party. Authors should also ensure not to submit any information which they do not have the copyright of or of which they have transferred the copyrights to a third party.
Contents on WebmedCentral are purely for biomedical researchers and scientists. They are not meant to cater to the needs of an individual patient. The web portal or any content(s) therein is neither designed to support, nor replace, the relationship that exists between a patient/site visitor and his/her physician. Your use of the WebmedCentral site and its contents is entirely at your own risk. We do not take any responsibility for any harm that you may suffer or inflict on a third person by following the contents of this website.