Sarcoidosis is an inflammatory disorder characterized by noncaseating granulomas infiltrating affected organs. Any organ system can be involved, and more than 90 % of patients have a primary pulmonary manifestation. The incidence of radiographically evident bone involvement has been reported over a wide range: from 3 to 39 % depending on the population studied and imaging modalities used. Any bone may be affected in osseous sarcoidosis. Bilateral involvement of the phalanges of the hand and feet is most common. However, reports of long bone, skull, rib and vertebral involvement have appeared. To date, there are no specific tools for the assessment of skeletal disease activity in sarcoidosis. Plain radiograph picks lesions in the small bones of hands and feet greater than does in other bones. Bone scan is useful for defining the extent of the skeletal disease and bone marrow involvement. Magnetic resonance imaging usually demonstrates multifocal lesions within the vertebrae that are hypointense on T1-weighted images and hyperintense on T2-weighted images. In cases of multifocal bone lesions and an established diagnosis of sarcoidosis, a diagnosis of bone sarcoidosis should be considered in the differential diagnosis. Optimal treatment of osseous sarcoidosis remains controversial. We present five cases of multisystem sarcoidosis with skeletal involvement including long bones and vertebrae and a description of immunosuppressive therapies used in our patients. A literature review highlighting the diagnostic approach using radiographic imaging, as well as treatment strategies, is provided.
Subcutaneous oedema is a rare presenting clinical manifestation of dermatomyositis. In this case, we report a 44-year-old man presenting with bilateral upper extremity predominant swelling and weakness. The proximal muscle weakness, dysphagia and presence of Gottron's papules as well poikiloderma like skin changes led to the clinical diagnosis of dermatomyositis. He received aggressive treatment with high-dose glucocorticoids and required intravenous immunoglobulin with improvement in his symptoms.
Background The mixed cryoglobulinemia (MC) syndrome is a systemic inflammatory syndrome that causes small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes most commonly caused by chronic hepatitis C virus (HCV), and rarely by chronic hepatitis B virus (HBV). Its clinical presentation is significantly varied, with manifestations ranging from purpura, arthralgia, and myalgia to more severe neurologic and renal involvement. Pulmonary involvement as organizing pneumonia, alveolar hemorrhage, and pulmonary vasculitis have been reported, but appear to be quite rare. Case presentation We report an uncommon case of a patient who presented with primary pulmonary syndrome without renal involvement in the setting of MC, due to untreated chronic hepatitis B infection. Early diagnosis and consequent institution of glucocorticoids, B-cell-depleting monoclonal antibody and antiviral therapy led to a favorable outcome and prevented any fatal sequelae. Conclusion Pulmonary compromise in MC syndrome is very uncommon and carries a high rate of mortality. Therefore, in patients with HBV presenting with hemoptysis, physicians must carry a high clinical suspicion for alveolar hemorrhage secondary to cryoglobulinemic vasculitis.
The clinical presentation of Systemic Lupus Erythematosus (SLE) is diverse and vasculitis can be a potential manifestation. Cutaneous lesions involving small vessels are the most frequent presentation. However, medium and large vessel vasculitis may present with life-threatening visceral manifestations. We present a unique case of pelvic vasculitis mimicking a pelvic mass as an initial presentation of SLE. There are case reports of systemic vasculitis involving the female genital tract with giant cell arteritis (GCA), polyarteritis nodosa (PAN), and granulomatous with polyangiitis and microscopic polyangiitis (GPA/MPA), among others, but only a few cases attributed to SLE. Awareness of this condition and a prompt diagnosis are warranted as this is a severe and potentially life-threatening condition.
Patient: Male, 58-year-old Final Diagnosis: Cardiomyopathy Symptoms: Arthritis • dynpnea • rash Medication: — Clinical Procedure: — Specialty: Cardiology • Dermatology • Rheumatology Objective: Rare co-existance of disease or pathology Background: Psoriasis is a chronic inflammatory skin disease associated with multiple comorbidities including psoriatic arthritis (PsA), atherosclerotic disease, metabolic syndrome, diabetes, hypertension, obesity, and depression. Interestingly, nonischemic cardiomyopathy, especially dilated cardiomyopathy (DCM), has been associated with psoriasis and reported in only in a few cases in the literature. Case Report: We report the rare case of a 58-year-old man with a medical history of untreated severe psoriasis and PsA who presented with a sudden onset of shortness of breath. Laboratory and radiographic studies showed an elevated level of B-type natriuretic peptide and acute bilateral pulmonary edema. The patient had normal coronary arteries on cardiac catheterization and echocardiography showed newly diagnosed DCM with systolic and diastolic dysfunction. Cardiac magnetic resonance imaging was consistent with nonischemic DCM (NIDCM) with no evidence of hypertrophy, infiltrative process, or edema. The patient was diagnosed with acute congestive heart failure secondary to NIDCM in the setting of long-standing untreated psoriasis. He responded well to diuretics, was placed on guideline-directed medical therapy, and was discharged with a LifeVest personal cardiac defibrillator. As an outpatient, the patient was started on secukinumab, a monoclonal antibody against inter-leukin-17A. At his last follow-up appointment, the patient reported improvement in his cardiac symptoms and resolution of his psoriatic skin lesions; repeat echocardiography showed improvement in his ejection fraction. Conclusions: Although studies have shown a higher prevalence of cardiovascular disease in patients with psoriasis, an association with NIDCM has not been studied sufficiently. We recommend further studies of the prevalence, pathogenesis, screening, and management of NIDCM in patients with psoriasis.
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