|CASE BASED REVIEW
|Year : 2017 | Volume
| Issue : 3 | Page : 169-174
“Smouldering conditions of the shoulder” Lest we forget!
Anupam Wakhlu1, Archana Wakhlu1, Vini Tandon2, Narendra Krishnani3
1 Department of Rheumatology, KGMU, Lucknow, Uttar Pradesh, India
2 Department of Digdarshika Pathology, SGPGIMS, Lucknow, Uttar Pradesh, India
3 Department of Pathology, SGPGIMS, Lucknow, Uttar Pradesh, India
|Date of Web Publication||19-Jul-2017|
Department of Rheumatology, KGMU, Lucknow - 226 001, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
The shoulder joint may be involved in seronegative spondyloarthropathy, calcium pyrophosphate dihydrate disease and degenerative joint disease. Adhesive capsulitis, calcific tendonitis and rotator cuff degeneration are other diseases that can involve the shoulder. The shoulder joint area may be involved as a consequence of extra-articular disease, and massive shoulder swelling may rarely result. Herein, five uncommon diseases presenting with predominant large shoulder swelling are described. The typical clinical and radiological findings with diagnostic features are highlighted.
Keywords: Amyloid arthropathy, Charcot's joint, Milwaukee shoulder, muscle cysticercosis, syringomyelia, tubercular arthritis
|How to cite this article:|
Wakhlu A, Wakhlu A, Tandon V, Krishnani N. “Smouldering conditions of the shoulder” Lest we forget!. Indian J Rheumatol 2017;12:169-74
| Introduction|| |
It is uncommon to have predominant involvement of the shoulder joint in rheumatic diseases other than the commonly occurring adhesive capsulitis, calcific tendonitis, and rotator cuff degeneration. Shoulder arthropathy can occur in seronegative spondyloarthropathy, calcium pyrophosphate dihydrate disease and degenerative joint disease, among other causes; however, massive shoulder swelling as a presenting manifestation of systemic diseases is distinctly uncommon. It is important to keep in mind that involvement of the shoulder joint area may be a consequence of extra-articular disease. Herein, the clinical features and patho-radiological findings of five uncommon diseases presenting to the rheumatology outpatient department with predominant large shoulder swelling are described, exemplifying the varied pathologies that may affect the shoulder region. The typical clinical and radiological findings with diagnostic features are highlighted along with the need for a high index of suspicion so that these diseases are not overlooked.
| Case Reports|| |
An 84-year-old female patient presented with bilateral massive shoulder swelling for the past 3 years. Despite the size of the swelling, she only complained of mild pain and poor mobility. The swelling would intermittently increase, and “red” fluid had been removed on some occasions, although there was no history of preceding or recurrent trauma. She was not a diabetic, had mild renal derangement not requiring dialysis. Examination revealed bilateral cystic swellings with mild tenderness, restriction of shoulder movements and the shoulder would recurrently dislocate on movement [Figure 1]a. The complete blood counts (CBC), erythrocyte sedimentation rate by Westergren's method (ESR WG) and serum ferritin were normal. Serum uric acid was 8.2 mg%, and bone mineral density revealed osteoporosis. Radiographs of the shoulder revealed marked shoulder swelling, osteopenia, subluxated shoulder, widened subacromial spaces, distended subacromial bursa extending into the deltoid region and extensive damage to the humeral head and shoulder joint [Figure 1]b and [Figure 1]c. A volume of 250 ml of hemorrhagic synovial fluid was aspirated from the left shoulder. The examination of the fluid revealed it to be mildly inflammatory with mononuclear cells, and plenty of red blood cells, Gram-stain, c/s were sterile, acid-fast bacilli (AFB) was negative, and no crystals were seen under polarized microscopy. Malignant cells were negative. However, given the clinical and radiographic picture, fluid was examined under transmission electron microscopy, which revealed needle-like hydroxyapatite crystals (basic calcium phosphate [BCP]) [Figure 1]d. A diagnosis of Milwaukee shoulder was made. The patient was managed conservatively with repeated aspirations of the shoulder, intraarticular steroids, empirical colchicine, and physiotherapy with shoulder support.
|Figure 1: Milwaukee shoulder (a) Large cystic shoulder swelling (b and c) Radiographs showing significant damage to shoulder joint (d) BCP crystals under transmission electron microscopy|
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Milwaukee shoulder is destructive arthritis in elderly persons characterized by rotator cuff defects, changes in cartilage and bone and deposits of BCP crystals in the fluid of affected joints. The clinical picture is exemplified by the case described above. Patients are almost always females over the age of 70 years. They have a long history of mild to moderate pain, swelling and loss of function, most commonly of the shoulder joint. There may be pronounced joint instability, with dislocation of the shoulder. Joint effusion may be massive, and the effusion may extend into the subdeltoid region. Moderate to large amount of hemorrhagic fluid can be aspirated. Knees, elbows, and hips can also be involved. Some joints progress to destruction, while in others it may stabilize after a year or two. Radiographs may show upward subluxation of the humeral head, degenerative, and erosive changes in the humeral head and glenoid of scapula and calcification of the tendinous rotator cuff. Magnetic resonance imaging (MRI) better delineates these changes, including soft tissue damage. BCP crystals are not birefringent and not seen under routine light microscopy. Clumps of BCP crystals can be identified by Alizarin red S stain, which is sensitive but has false positives. The gold standard is the identification of the crystals under electron microscopy. Management is largely supportive and conservative.
Key learning points
- Milwaukee shoulder usually presents in an elderly female, rapid destruction of the joint with aspiration of large amounts of hemorrhagic fluid and characteristic radiographic changes
- BCP crystals are small and nonbirefringent and so cannot be resolved under light or polarized microscopy. The crystals must be visualized under transmission electron microscopy for definitive diagnosis.
A 42-year-old male patient presented with an insidious onset large painless, progressive left shoulder swelling for the last 3–4 years [Figure 2]a. The complaints of the patient were of a general nature that the “shoulder does not look normal.” He complained of some discomfort and some restriction of movements. He had a history of recurrent dislocation of shoulder. He was a smoker but a nondiabetic, and there was no history of preceding trauma. Examination revealed a swelling with mostly cystic and some solid components, no local tenderness or pain on movement and restriction of movements. Radiographs revealed a large shoulder swelling with a disorganized glenohumeral joint. The subacromial bursa was distended with encroachment into the deltoid area. Scattered calcification was present. The humeral head was damaged as if it had been sharply cut [Figure 2]b. Given the characteristic nature of radiographic findings and suggestive history, sensory examination was performed which revealed dissociate sensory loss in cape distribution. A MRI was performed which revealed cervicothoracic syringomyelia [Figure 2]c. A diagnosis of neuropathic (Charcot's arthropathy) was made, caused by syringomyelia with Arnold-Chiari I malformation. The joint was aspirated, physiotherapy and support to the joint was advised and patient was referred to neurosurgery and orthopedics for advice regarding further management.
|Figure 2: Neuropathic (Charcot's) arthropathy cause syringomyelia with Arnold-Chiari I malformation (a) Large painless shoulder swelling (b) Radiograph showing disorganized glenohumeral joint (c) MRI showing cervicothoracic syringomyelia|
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Neuropathic arthropathy (Charcot's arthropathy) is a relatively painless, progressive, destructive arthropathy caused by a neurologic defect. The French neurologist Jean-Martin Charcot gave his name to this condition. Neuropathic arthropathy can result from a variety of sensory neurologic disorders, for example, syphilis, diabetes, leprosy, syringomyelia, multiple sclerosis, amyloidosis, and spinal cord trauma. However, in 30% cases, no neurological disorder is identified. Approximately, 20%–25% patients of syringomyelia develop neuropathic arthropathy. It is usually monoarticular and commonly affects the glenohumeral joint. However, elbows, wrists and fingers may also be involved. Bilaterally symmetrical involvement of shoulder is less common in syringomyelia as compared to tabes dorsalis. Dissociate anesthesia in the cape distribution is pathognomonic of syringomyelia; wasting of the intrinsic muscles of the hand may be seen. Neuropathic arthropathy usually has an acute inflammatory phase for a few months when the radiographs would be normal but MRI would show findings; untreated the disease progresses to chronic neuropathic arthropathy, which shows a disorganized joint characterized by both bone formation and resorption. There may be fractures and free bone fragments. The damaged articular surfaces so resulting have sharp and well-defined margins as opposed to septic arthritis, as was seen in our case. MRI helps in differentiating neuropathic arthritis from infection. PET-CT has 100% sensitivity and 93.8% specificity for this disease. In acute arthropathy, reduction of weight bearing and immobilization are important therapeutic modalities. Bisphosphonates may be used. Chronic arthropathy is managed with supportive care.
Key learning points
- Insidious onset and a relative lack of pain in a chronic, large shoulder swelling with a disorganized joint on radiographs should lead to a suspicion of Charcot's joint
- Sensory examination is mandatory in such a setting but is often missed.
A 64-year-old female patient presented with progressive bilateral large painful shoulder swellings and inflammatory small and large joint polyarthritis for the past 3–4 months. She had incapacitating pain in her shoulders and joints, and nonsteroidal anti-inflammatory drugs (NSAID's) and steroids offered partial relief only. Examination confirmed inflammatory arthritis, and there was an ulcerating shoulder swelling, which appeared to arise from the muscle [Figure 3]a. The bulkiness of both shoulders resembled the “shoulder pad sign.” CBC revealed anemia and thrombocytosis, ESR (WG) was 96 mm for the 1st hr, C-reactive protein (CRP) was 104 mg/dl, serum creatinine - 1.7 mg%, serum urate - 8.9 mg%, serum lactate dehydrogenase was 1750 IU/L, serum calcium and creatinine kinase (CPK) were normal. The urine examination was normal with no proteinuria. Radiograph of the shoulder revealed a swelling with bulky deltoid muscle, diffuse osteopenia with few ill-defined lytic lesions in the humeral head [Figure 3]b. Skeletal survey revealed widespread lytic defects in bone. Computerized tomography (CT) scan and MRI shoulders revealed muscle infiltration and hypertrophy and bone involvement also suggestive of amyloid deposits [Figure 3]c. Bone marrow examination revealed 60% plasma cells suggestive of multiple myeloma (MM) [Figure 3]d. Muscle biopsy revealed eosinophilic material (Congo red) [Figure 3]e with apple-green birefringence under polarized light, suggestive of amyloid deposits [Figure 3]f. Immunofixation revealed Lambda chain myeloma. A diagnosis of muscle and joint amyloid involvement with MM was made. The patient was managed symptomatically and was referred for the management of MM. Arthritis resolved somewhat after initiation of chemotherapy.
|Figure 3: Muscle and joint amyloidosis with multiple myeloma. (a) Ulcerating shoulder swelling (b) Shoulder radiograph with ill-defined lytic lesions (c) CT scan showing muscle hypertrophy (d) Bone marrow with plasmacytosis (e) Muscle biopsy (congo red) (f) Apple green birefringence under polarized light|
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Deposition of immunoglobulin light chains may lead to amyloidosis in diverse organs. The common causes are monoclonal gammopathy (70%), MM (20%) and Waldenstrom's macroglobulinemia (10%). Synovitis can develop when MM-associated light chain amyloid (AL amyloid) deposits in the joint. The presentation of this myeloma-associated amyloid arthropathy may resemble RA. The arthritis is usually nonerosive, inflammatory with stiffness; it is most commonly polyarticular but oligoarticular, and monoarticular involvement has been described. The shoulder joint is involved most frequently, followed by the knee, small joints of hands and wrists. Infiltration of the glenohumeral joint and surrounding tissues may produce the characteristic 'shoulder pad' sign. Extra-articular manifestations related to amyloidosis are carpal tunnel syndrome, macroglossia, soft tissue swelling and nodules. ESR is most consistently elevated in these patients, sometimes markedly so, followed by CRP. Other investigations reflect MM. Radiographs are used for the detection of lytic lesions while bone marrow examination, serum electrophoresis and Immunofixation confirm MM. MRI may have characteristic findings suggesting amyloidosis. Management is mainly directed to the treatment of MM.
Key learning points
- MM/amyloid arthropathy may present as a symmetric, seronegative, nonerosive inflammatory polyarthritis, which may be its presenting manifestation 
- The “shoulder pad sign” is an important clue to the diagnosis but is often missed on clinical examination.
A 28-year-old male presented with a large right shoulder swelling for the past 3 months. The shoulder was painful and he had low-grade fever. Examination of the shoulder revealed the area to be tender with a firm swelling, variable in consistency. There was a pain on the abduction of the shoulder. Other joints and muscles were normal, and the eye and fundus were normal as well. CBC was normal; CRP was mildly elevated, CPK was normal. A high-resolution ultrasonography (USG) of the shoulder region was done which revealed a large cystic area with a hyperechoic dot in the center suggestive of a scolex. There was significant surrounding edema along with pseudohypertrophy of the deltoid muscle [Figure 4]. Anticysticercal antibodies were positive. A diagnosis of deltoid cysticercal cyst of the pseudohypertrophic variety was made. The patient was treated with rest, NSAID's, steroids and albendazole for 14 days. He gradually improved with complete resolution in 3 months.
Cysticercosis is a disease caused by cysticercus cellulosae, the larval form of Taenia solium. Cysticercosis is endemic in India  with a predilection for the central nervous system. Solitary muscular involvement with other sites excluded is rare. Skeletal muscle involvement is usually asymptomatic but may cause symptoms associated with local inflammation such as pain, local tenderness, swelling, and restriction of movement. Muscle pseudohypertrophy may result. Sonographic features are fairly characteristic, revealed a well-defined cystic lesion with an eccentric hyperechoic area within it, representing the scolex. Surrounding inflammation and vascularity may be noted. The pseudohypertrophic variety is distinctly uncommon. MRI is a good modality to confirm the findings and delineate the soft tissues better. Diagnosis is dependent on radiology, ELISA and histopathology. Depending on the extent of involvement and manifestations, treatment may be conservative with NSAID's/steroids and albendazole/praziquantel or surgical with excision of the cyst. Most patients respond favorably and recover completely.
Key learning points
- USG as an imaging modality is fairly diagnostic for muscle cysticercosis with characteristic sonographic findings 
- Cysticercosis is a rare but potentially curable cause of shoulder swelling in rheumatology practice.
A 64-year-old female presented with left shoulder pain, marked swelling, and restriction of movements of 1 year duration. Examination showed markedly tender and swollen left shoulder with a swollen arm and gross restriction of movements. There was no history of trauma, fever, weight loss, diabetes mellitus or injection into the local area. There was no history of tuberculosis (TB) or contact with a patient of TB either in family or friends. Investigations revealed mild anemia, and raised acute phase proteins. Radiograph of the left shoulder showed gross erosion of the head of the humerus with marked soft tissue swelling. MRI of the left shoulder showed gross soft tissue and muscle swelling with collection and osteomyelitis of the head and shaft of humerus [Figure 5]. Fine needle aspiration cytology of the shoulder joint swelling revealed caseous material with plenty of AFB. Culture grew Mycobacterium tuberculosis. Adiagnosis of TB of the left shoulder was made. She received therapy with 4 drug anti-tubercular therapy (ATT) (RHEZ) for 18 months and recovered more than 70% in terms of her physical function. However, partial restriction of movement persisted.
Skeletal TB constitutes 1%–3% of patients of extrapulmonary TB. TB of the shoulder joint constitutes 1%–10.5% of skeletal TB, depending on the series studied. Concomitant pulmonary TB may not be present. It is important to remember that skeletal TB may develop in immunocompetent individuals without any predisposing factor for TB. Joint involvement is either from hematogenous spread or from contiguous bone involvement. TB of the joint may mimic a number of pathologies including malignancy. A high index of suspicion given the frequent lack of systemic features, high inflammatory parameters like ESR and CRP, radiographic and MRI features and histopathology with a demonstration of granulomas and AFB help in making a diagnosis. Radiologically, lesions appear osteoporotic with erosions, reactive sclerosis, reduced joint space, and progressive joint destruction. Periosteal reaction, bone expansion, sinus formation may be seen. MRI may reveal a large collection, bone lysis, sclerosis, periosteitis, and marked synovitis. Histopathology demonstrates the classic granulomas with AFB or examination of the collection will demonstrate M. tuberculosis. Polymerase chain reaction may be used if required. Conventional 4-drug ATT is given for 12–18 months, depending on local recommendations. Surgical debridement is done when necessary.
Key learning points
- Shoulder joint involvement is uncommon in osteoarticular TB
- TB is a great mimic. The absence of systemic features does not preclude a diagnosis of TB. Early detection and treatment, based on a high index of suspicion is important.
| Conclusion|| |
[Table 1] summarises the salient features of the five diseases. Shoulder involvement occurs in a number of rheumatic diseases, but manifestation with shoulder swelling is unusual. One needs to expand one's differential diagnosis when encountered with shoulder involvement with significant swelling. Uncommon causes such as infections and parasites (TB, cysticercosis), crystals (BCP disease), infiltrative systemic disorders (myeloma, amyloid) and neuropathic (syringomyelia) need to be considered. Sometimes, this may be the presenting manifestation. A good clinical examination, imaging and laboratory analysis and a high index of suspicion help in clinching a diagnosis.
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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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]