|Ahead of print publication
Myositis as initial presentation of sarcoidosis: A rarity
Mayank Gupta, Lalit Duggal, Neeraj Jain, Bhavya Chintala
Department of Rheumatology and Clinical Immunology, Sir Gangaram Hospital, New Delhi, India
90, Vinay Nagar, Sector 2, Janak Colony, Gwalior - 474 012, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
Sarcoidosis is a multisystem chronic inflammation characterized by noncaseating granulomas with internal organ involvement. Lung and lymph nodes are most commonly affected with muscles are involved in up to 3% of patients. Here, we discuss a 39-year-old male patient consulted in rheumatology OPD with chief complaints of localized swelling over the left calf since 6 months. Routine blood investigations were normal. Magnetic resonance imaging of the left thigh muscles was done which was suggestive of features of inflammatory myositis. Muscle biopsy was suggestive of epithelioid granulomas, more in favor of sarcoidosis. Contrast-enhanced computed tomography chest revealed symmetrical nodules in upper lobes of both lungs. We made a diagnosis of granulomatous myositis secondary to sarcoidosis. Steroids and azathioprine were started. The patient responded well to treatment and muscle swelling subsided.
Keywords: Biopsy, granulomatous myositis, sarcoidosis
| Introduction|| |
Sarcoidosis is a multisystem chronic inflammation characterized by noncaseating granulomas. It can involve any organs. Lung and lymph node are involved in 90% and contributes to major morbidity and mortality with the disease., Asymptomatic muscle involvement is common (20%–80%), symptomatic involvement in <3% of cases., A random muscle biopsy in a patient with sarcoidosis without myopathic symptoms will show granulomas in up to 80% of cases and can be used for tissue diagnosis., Here, we discuss a case of sarcoidosis in which muscle involvement was the initial and only manifestation with lung involvement being picked up during routine screening.
| Case|| |
A 39-year-old male patient consulted in rheumatology OPD with chief complaints of localized swelling over the left calf since 6 months [Figure 1]. The patient presented with complaints of the stiffness of calf muscle, which was more on medial side, associated with fullness, with localized swelling, there was no localized redness or raised temperature. No history of fever, muscle weakness, cough, chest pain, tingling, numbness, rashes, and joints pain. There was no significant past, personal, and family history. On examination, blood pressure was 124/82 mm Hg, pulse rate 86/min, and respiratory rate 12/min. There was no pallor, icterus, cyanosis, lymphadenopathy, and pedal edema. Cardiovascular system (CVS), central nervous system, abdomen examination were normal. In respiratory system examination, crepts were present in bilateral lungs in lower zone. In musculoskeletal examination, there was fullness of left calf muscle with the induration of the posterior aspect of the left thigh. There was no joint tenderness and synovitis. Hemogram, creatine phosphate kinase (95 IU/L), lactate dehydrogenase (138 IU/L) liver, kidney function tests, and serum calcium levels were normal [Table 1]. Myositis specific antibodies were negative. Chest X-ray showed reticular opacities in right middle and upper zone. Serum angiotensin-converting enzyme level was normal; Mantoux and Quantiferon gold test were negative. Magnetic resonance imaging of left thigh and calf muscle revealed T1-weighted hyperintense signals in multiple muscles predominantly in medial head of gastrocnemius with thin layer of fluid around muscles suggesting Inflammatory Myositis [Figure 2]. Muscle biopsy was done from calf muscle which showed intrafascicular and perifascicular epithelioid granulomas without caseous necrosis favoring the diagnosis of sarcoidosis [Figure 3], acid-fast bacilli and fungal staining were negative. Contrast-enhanced computed tomography (CT) chest revealed symmetrical nodules in upper lobes of both lungs with multiple enlarged lymph nodes in hilar, paratracheal region, suggestive of sarcoidosis [Figure 4]. On the basis of clinical history and investigations, the diagnosis of granulomatous myositis due to sarcoidosis was made. Prednisolone 40 mg daily was started with gradual tapering of dose along with azathioprine 50 mg twice daily. The patient responded well to treatment with reduction in the size of swelling and improvement of symptoms.
|Figure 1: A 39-year-old male with swelling of the left calf muscle, more on medial side with induration|
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|Figure 2: Magnetic resonance imaging of left thigh and calf muscles showing hyperintense signals in multiple muscles predominantly in medial head of gastrocnemius (white arrow) with thin layer of fluid around muscles with the possibility of inflammatory myositis|
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|Figure 3: Biopsy from calf muscle taken which showed intrafascicular and perifascicular epithelioid granulomas (white arrow) accompanied by Langerhans giant cells without caseous necrosis|
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|Figure 4: Contrast-enhanced computed tomography chest showing multiple symmetrical nodules in both lungs (white arrow)|
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| Discussion|| |
Sarcoidosis is a multisystem autoimmune disease characterized by the presence of noncaseating epithelioid granulomas in various organs and tissues with lungs and lymph nodes most commonly affected. Muscles are involved in less percentage of patients with <3% have initial musculoskeletal involvement.
Three types of muscle involvement in sarcoidosis are described – (1) chronic myopathy, (2) acute granulomatous myositis, and (3) muscle nodules.
In chronic myopathy, there is neurogenic atrophy caused by granulomatous infiltration of nerves, electromyography shows myopathic changes, but muscle enzyme levels are frequently normal or only minimally elevated., Muscle biopsy shows granulomas and a diffuse infiltrate with macrophages and CD4+ T cells. Acute granulomatous myositis and muscle nodules are the other two types but are unusual manifestations. Acute sarcoid myositis is rare, occurring mostly in young black girls and women. Signs and symptoms similar to those of polymyositis with myalgias, proximal muscle weakness muscle tenderness, high levels of muscle enzymes, and muscle necrosis or granulomatous inflammation on biopsy. Palpable granulomatous nodules and mass or tumor-like lesions may cause pain, stiffness, and cramping of the involved muscle. The response of acute granulomatous myositis to treatment with corticosteroids is good, and the course is usually benign. However, in chronic myopathy and nodular disease, the response to therapy is unpredictable, with variable remissions and exacerbations requiring continued immunosuppression. In some studies, sarcoid myositis was frequently associated with severe disability and rarely improved after corticosteroid treatment, whereas most patients with isolated granulomatous myositis improved.
In our case, muscle involvement was the initial and only manifestation of sarcoidosis. Chest CT was done due to clinical findings in the chest and to rule out some underlying etiology which was suggestive of symmetrical nodules in upper lobes and hilar and lymphadenopathy. The diagnosis was confirmed with muscle biopsy. The patient responded well to immunosuppressive medication.
| Conclusion|| |
Myositis can be focal or generalized and can be due to a variety of causes. It may be due to infection, inflammatory disorder, or some malignancy., We must consider rare and atypical causes of focal myositis. Sarcoidosis is one of the rare cause in which myositis can be initial presentation. Tissue biopsy is must for making a diagnosis. Rare presentations of rare diseases should also be kept in mind.
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]