Rheumatologic Manifestations of Sarcoidosis

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Sarcoidosis is a multi-system disease of unknown etiology characterized by the formation of granulomas in various organs. It affects people of all ethnic backgrounds and occurs at any time of life but is more frequent in African Americans and Scandinavians and in adults between 30 and 50 years of age. Sarcoidosis can affect any organ with a frequency varying according to ethnicity, sex and age. Intrathoracic involvement occurs in 90% of patients with symmetrical bilateral hilar adenopathy and/or diffuse lung micronodules, mainly along the lymphatic structures which are the most affected system. Among extrapulmonary manifestations, skin lesions, uveitis, liver or splenic involvement, peripheral and abdominal lymphadenopathy and peripheral arthritis are the most frequent with a prevalence of 25–50%. Finally, cardiac and neurological manifestations which can be the initial manifestation of sarcoidosis, as can be bilateral parotitis, nasosinusal or laryngeal signs, hypercalcemia and ren...

Caspian Journal of Internal Medicine, 2018

Background: Sarcoidosis is a multisystem granulomatous disease. Coexistence with spondyloarthritis (SA) has been more described as an adverse effect of anti-TNF α therapy than an association. We report herein a case of a typical sarcoidosis confirmed by histological proofs and an advanced SA with a bamboo column. Case Presentation: A 48-years-old woman presented with inflammatory back pain for 5 years and ankle swelling for 1 year. On physical examination, she had an exaggerated dorsal kyphosis and disappearance of lumbar lordosis with limitation in motion of the cervical and lumbar spine. Laboratory tests did not show an inflammatory syndrome or hypercalcemia. Plain radiographies of the spine and pelvic revealed a triple ray appearance with sacroiliitis grade 4. Chest radiography and CT confirmed the presence of bilateral hilar lymph nodes and parenchymal nodes. Bronchoscopy and biopsies were performed showing non-calcified granulomatous reaction without cell necrosis. The diagnosis of SA was performed based on 9 points of Amor criteria associated with pulmonary sarcoidosis. She was treated with 15 mg per week of methotrexate and 1mg/kg/day of prednisone for pulmonary disease with good outcomes. Conclusions: Sarcoidosis may be associated to SA besides paradoxical drug effect. The same physio pathological pathways mediate by TNF α are arguments for association than hazardous coincidence.

Iranian Red Crescent Medical Journal, 2014

Background: Sarcoidosis is a multisystem disease affecting different organs with different frequency rates depending on geographical location. Musculoskeletal abnormalities includes osseous lesions (small and large bone sarcoidosis), sarcoidal arthropathy, and sarcoidal myopathy. Musculoskeletal involvement is reported in a significant number of patients. Objectives: This study aimed to determine the prevalence and clinical picture of musculoskeletal sarcoidosis in Iranian cohort of patients with sarcoidosis. Patients and Methods: We designed a descriptive cross-sectional study including 30 patients with sarcoidosis who had hospitalized in the Rheumatology Department at Ghaem Hospital, Mashhad, Iran. The patients were evaluated for musculoskeletal symptoms using history, physical examination, and paraclinical data. Results: Of the 30 studied patients, 24 were female (80%) and six were male (20%). The mean age at diagnosis was 38 years. Sarcoidal arthropathy (arthritis and periarthritis) was observed in 26 patients (86.6%). Furthermore, the initial presentation was associated with joint symptoms in 19 cases (63.3%); acute arthritis developed in 17 (65%) while bone and muscle involvements each occurred in 2 (6.6%). Conclusions: Sarcoidosis is a common disease in women aged 20 to 40 years. The most common involved joint were consecutively ankles, knees, and wrists, reaching a accumulated frequency of 86.6%; however, bone and muscle involvements were uncommon.

Srpski arhiv za celokupno lekarstvo, 2012

Introduction This is a presentation of a 61-year-old female patient. Since 44 years have passed from the onset of her first symptoms until the final diagnosis of sarcoidosis, this was the reason of our decision to publish the case. Case Outline During the follow-up period of 44 years the patient had ocassional polymorphic complains, such as adynamia, nausea, abdominal pains, myalgia, arthralgia, body weight loss (8-10 kg) etc. The clinical course was predominated by splenomegaly, hepatitis and arthralgia, and later chronic renal failure also developed. Laboratory findings showed elevated markers of acute inflammation and autoantibodies. The patient was hospitalized in different university internal hospitals (gastroenterology, allergology, rheumatology, nephrology and pulmology). Liver biopsy was performed three times, rectum and kidney biopsy once each and finally bronchoscopy and pulmonary biopsy was done. At last, about 40 years from the onset of the first symptoms, in 2006 the diagnosis of lung sarcoidosis was established. Conclusion The final diagnosis of spleen sarcoidosis was confirmed by pathologically verified sarcoidosis of the lungs. This case is particularly interesting because of the presence of familial sarcoidosis (the patient's son also had recurrent pulmonary sarcoidosis).

Journal of Clinical Medicine of Kazakhstan, 2016

Sarcoidosis is a systemic autoimmune disease characterized by noncaseatinggranulomatous inflammation with unknown etiology.Although the lungs and respiratory system are most commonlyinvolved, sarcoidosis may involve virtually any part of the body,including the locomotor system, eyes, skin, lymph nodes. Diagnosis attained via consensus between the clinical presentation and natural history, pattern of major organ involvement, confirmatory biopsy, and response to therapy. Histopathological features remain the gold standard in diagnosis. Radiologic staging in sarcoidosis is based on the chest X-ray. If chest radiographis normal, high-resolution computed tomography (HRCT) can demonstrate pathological changes in a detailed manner. Sarcoidosis generally follows a benign course with occasional spontan remissions. The most important causes of mortality are acute and chronic respiratory failure, pulmonary hypertension and hemoptysis due to aspergillosis. When treatment is indicated, glucocorticoids remain the only recognized effective therapy for active sarcoidosis. Level of evidence for management of sarcoidosis is low, generally reflecting the results of limited number of clinical studies, case series and expert opinion. In selected cases, biological agents including, tumour necrosis factor inhibitors, seem to be promising.

Annals of the Rheumatic Diseases, 2002

Objectives: (a) To describe the clinical characteristics of acute sarcoid arthritis and the diagnostic value of its presenting clinical features; (b) to evaluate whether disease onset is seasonal; and (c) to evaluate whether smoking behaviour or the presence of HLA class II alleles is a risk factor for the disease. Methods: 579 consecutive patients with recent onset arthritis who had been newly referred to a rheumatology outpatient clinic were included in a prospective cohort study. The presenting clinical features, the smoking behaviour, and the results of HLA-DQ and HLA-DR DNA typing of 55 patients with sarcoid arthritis, 524 patients with other arthritides of recent onset, and samples of the normal population were compared. Results: In all cases the disease showed a self limiting arthritis and overall good prognosis. The diagnostic ability of a combination of four clinical features-symmetrical ankle arthritis, symptoms of less than two months, age below 40 years, and erythema nodosum-was exceptionally high. When test positivity is defined as the presence of at least three of four criteria the set rendered a sensitivity of 93%, a specificity of 99%, a positive predictive value of 75%, and a negative predictive value of 99.7%. The disease clustered in the months March-July. The disease was negatively associated with smoking (odds ratio (OR) 0.09; 95% confidence interval (95% CI) 0.02 to 0.37) and positively associated with the presence of the DQ2 (DQB1*0201)-DR3 (DRB1*0301) haplotype (OR 12.33; 95% CI 5.97 to 25.48). Conclusion: The disease entity acute sarcoid arthritis has highly diagnostic clinical features. The seasonal clustering, the protective effect of smoking, and the association with specific HLA class II antigens support the hypothesis that it results from exposure of susceptible hosts to environmental agents through the lungs.

Turkish Journal of Rheumatology, 2013

Bu yazıda, sarkoidoz remisyonunda iken inflamatuvar bel ağrısı ve üveit ile başvuran 49 yaşında bir erkek olgu sunuldu. Diferansiye olmamış spondiloartropati ve sarkoidoz birlikteliği, muhtemel etyolojik ilişki göz önünde bulundurularak, tanı ve tedavideki mevcut zorluklar eşliğinde irdelendi. Anahtar sözcükler: İnflamatuvar bel ağrısı; sarkoidoz; spondiloartropati; üveit. In this article, we present a 49-year-old male case who was admitted with inflammatory back pain and uveitis when he was in remission for sarcoidosis. The concomitance of undifferentiated spondyloarthropathy and sarcoidosis with an emphasis on their possible etiological association was also discussed in the light of current diagnostic and therapeutic challenges.

Journal of Evolution of Medical and Dental Sciences, 2017

BACKGROUND Sarcoidosis is an inflammatory multisystem granulomatous disease of unknown aetiology and predominantly affects the lungs, followed by eye, skin, peripheral lymph node, liver, spleen, heart, central nervous system, musculoskeletal system and salivary glands. Aim-To find out the clinical profile of sarcoidosis patients prospectively admitted to a respiratory unit. MATERIALS AND METHODS The study was conducted on 30 patients of sarcoidosis, who were admitted for various respiratory complaints, in the Department of Pulmonary Medicine, GSVM Medical College, Kanpur & Govt. Medical College, Kannauj. A thorough respiratory evaluation was done including clinical evaluation for signs and symptoms, chest x-ray PA view, HRCT Thorax, Spirometry, sputum for AFB, PPD, serum ACE, serum Calcium, 24-hour urinary calcium, BAL Fluid analysis, CD4 to CD8 of BAL fluid. RESULTS 30 patients of sarcoidosis were evaluated. Of the total patients, male & female were 11 and 19 respectively and ratio was 1:1.7. The mean age of study group was 34.8 years (Range 27-51). Dyspnoea was present in all the patients followed by dry cough (93.3%). Bilateral hilar lymphadenopathy plus pulmonary opacity was the most common radiological presentation of sarcoidosis in 14/30 (46.7%). Pleural effusion was noted in a single case. Extrapulmonary manifestations like subcutaneous nodules were seen in only one case while uveitis also observed in single case only. Sputum for AFB was negative in 2 cases, while others did not raise sputum. PPD testing showed no induration among all, except one where it showed mild induration (6 mm). Serum ACE was elevated in 24/30 patients (86.6%). Serum Calcium was elevated in 10/30 patients (28.6%). 24-hour urinary calcium was elevated in 8/30 patients (26.6%). Spirometry showed a restrictive pattern in 71.4% (i.e. 20/28) and was normal in 17.9% cases while the other three patients did not cooperate. The CD4 to CD8 of BAL fluid levels were raised in all cases of sarcoidosis (range 2.9-4.4). CONCLUSION Bilateral hilar lymphadenopathy plus pulmonary opacity was the most common radiological presentation of sarcoidosis and BAL fluid CD4 to CD8 counts are easy to perform and helpful in supporting diagnosis of sarcoidosis along with clinical and radiological features.

European Journal of Rheumatology, 2017

A 50-year-old female patient with complaints of bilateral ankle joints arthritis, morning stiffness, fatigue, and effor dyspnea was admitted to our rheumatology clinic. Patient medical history indicated that she had hypertension lasting 2 years under control with antihypertensive drugs. On physical examination, bilateral ankle joint arthritis with reduced range of motion was detected. Laboratory tests, including hemogram, fasting blood sugar, urinalysis, and liver and renal function tests were normal. Acute phase reactants were examined: erythrocyte sedimentation rate (ESR): 45 mm/h (normal, <25 mm/h), C-reactive protein (CRP): 4.12 mg/ dL (normal, <0.5 mg/dL). Thyroid function tests, serum tumor markers, and serum amyloid A were normal. Serum angiotensin-converting enzyme (ACE) level was 156 mg/dL (normal, <45 mg/dL); serum calcium and 25-hydroxy vitamin D3 were normal. Serologic tests were performed; complement, rheumatoid factor (RF), antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), and anticyclic cytrullinatedpeptidantibody (anti-CCP) were negative. On abdominal ultrasonography hepatosteatosis and myoma at the uterus was reported. Chest X-ray showed enlarged hilar lymphadenopathies (Figure 1). On thoracic computed tomography (CT), bilateral hilar and mediastinal lymphadenopaties (largest, 38×20mm) and multiple nodules spreading over whole areas but more significantly at the upper and middle zones of both lungs were reported (sarcoidosis grade 2; Figure 2). A chest disease specialist was consulted and endobronchial ultrasonography (EBUS) was performed. On histopathological evaluation noncaseating granuloma formation compatible with sarcoidosis was reported. In terms of extrapulmonary involvement (skin, eyes, neurosarcoidosis), the patient was investigated but no findings were detected. Differential diagnosis was conducted; lymphoma, fungal infection, and tuberculosis were excluded. The treatment was started with corticosteroids 32mg/day and hydroxychloroquine 200 mg/day. Six month later, clinical laboratory and radiologic regression was observed. The patient with fine general condition is in the clinical follow-up program. Case 2 A 27-year-old male patient with complaints of bilateral knee and ankle joints pain, morning stiffness, and fatigue was admitted to our rheumatology clinic. Patient medical history indicated that his mother has had

Reumatología Clínica, 2017

A 35-year-old man presented with a 3-year history of arthralgia and purple coloration of the skin of his fingers and feet. Hand and foot radiography showed cystic bone lesions on phalanges suggestive of sarcoidosis. Lab tests revealed increased liver enzymes. Liver MRI evidenced an enlarged liver and retroperitoneal lymphadenopathy. Histological analysis of the finger skin, lymph nodes and liver demonstrated the presence of granulomas, confirming the diagnosis of sarcoidosis. The patient started prednisolone with rapid improvement of the symptoms. Skin lesions are divided into two groups: specific for sarcoidosis (with granulomas, lupus pernio-like) and nonspecific (without granulomas, erythema nodosum-like). Specific cutaneous lesions usually cause no other symptoms beyond cosmetic changes. Lupus pernio stands out for having distinctive features but, to the best of our knowledge, the simultaneous involvement of both hands and feet has never been reported.