Epidemiology of Digital Clubbing and Hypertrophic Osteoarthropathy: A Systematic Review and Meta-analysis.

ABSTRACT: Digital clubbing and hypertrophic osteoarthropathy (HOA) are long-standing clinical entities, but their prevalence have not been synthesized. We aimed to estimate the prevalence of digital clubbing and HOA in people with existing medical conditions.We comprehensively searched PubMed, Embase, and Web of Science to select studies addressing HOA or digital clubbing and published through March 23, 2021. Summary estimates of the prevalence were derived through random-effects meta-analysis and narrative synthesis. The review protocol has been registered with PROSPERO, CRD42021243934.Of 3973 records, we included 142 studies. In adults, the pooled prevalence of digital clubbing was 33.4% (95% confidence interval [CI], 16.6-52.8), 31.3% (95% CI, 22.4-41.1), 27% (95% CI, 9.4-49.5), and 22.8% (95% CI, 10.8-37.6) in subjects with intestinal diseases, interstitial lung diseases, infective endocarditis, and hepatic diseases, respectively. In children and adolescents, the pooled prevalence of digital clubbing was 29.1% (95% CI, 19.4-39.9), 23% (95% CI, 9.0-41.1), 19.5% (95% CI, 4.1-42.4), and 17.1% (95% CI, 9.5-26.5) in subjects with human immunodeficiency virus infection, hemoglobinopathies, cystic fibrosis, and tuberculosis. The pooled prevalence of HOA was 10.1% (95% CI, 2.0-23.1) in adults with cancers, and 5% (95% CI, 2.5-8.2) in children and adolescents with cystic fibrosis.In conclusion, the prevalence of digital clubbing varied across disease groups in both adults and children. Full-spectrum HOA was mostly reported in adults with liver disease and cancers, and in children and adolescents with cystic fibrosis.

Atypical and incomplete pulmonary hypertrophic osteoarthropathy in the left distal femur: a case report.

BACKGROUND: Pulmonary hypertrophic osteoarthropathy (PHO) is a rare paraneoplastic syndrome that mainly occurs in patients with lung cancer. Most symptoms occur symmetrically, and unilateral symptoms without clubbing are infrequent. This report presents the case of a rare atypical symptom that was highly suspected of being PHO. CASE PRESENTATION: A 77-year-old woman with swelling and severe pain in the left femur for 2 months was referred to our hospital. Radiography revealed a remarkably osteogenic thickening and sclerotic lesion in her distal femur. Preliminary diagnoses of malignant bone tumor and hematological tumor were made based on laboratory test results, radiological examination, and clinical manifestation. A needle biopsy was performed, which ruled out the diagnosis of malignant bone tumors. A low-grade bone tumor was still suspected. After that, en bloc resection was performed, followed by replacement of the femur with a mega-prosthesis. Pathological analysis revealed no malignant tumor cells, and immunohistochemical staining for CDK4 and MDM2 yielded negative results. Meanwhile, pulmonary large cell carcinoma was identified on biopsy. Based on published studies, a diagnosis of atypical PHO was made according to clinical and imaging manifestations. CONCLUSIONS: This is an infrequent case of PHO with unilateral symptoms in the left femur. When clinical manifestations and radiological findings are inconsistent with the pathological results, the possibility of benign lesions with malignant clinical manifestations or imaging features should be carefully considered.

Hypertrophic osteoarthropathy.

Hypertrophic osteoarthropathy (HOA) is an orphan syndrome characterized by abnormal proliferation of the skin and osseous tissues at the distal parts of the extremities. The main clinical features are: a peculiar bulbous deformity of the tips of the digits conventionally described as "clubbing," periosteal proliferation of the tubular bones, and synovial effusions. In most instances, HOA develops a reaction to a severe internal illness, such as lung cancer, cyanotic heart disease, or liver cirrhosis. There is a subgroup of patients who do not have underlying pathology. Such cases are classified as having primary HOA. Digital clubbing is easy to recognize. Any patient with newly developed digital clubbing should undergo careful search for an underlying illness with special attention to intra-thoracic pathologies. Painful HOA is treated with non-steroidal anti-inflammatory medications. Vascular endothelial growth factor and prostaglandin E2 have been proposed as key bone proliferating mediators.

Hipocratismo digital: paciente con osteoartropatía hipertrófica y presencia de acro-osteólisis / Digital clubbing: a patient with hypertrophic osteoarthropathy and the presence of acro-osteolysis

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Finger clubbing and cirrhosis in a sarcoidosis patient.

Sarcoidosis is a multisystem granulomatous inflammatory disorder frequently affecting the lungs, but also the liver, along with cirrhosis and portal hypertension occurring in less than 1% of the patients. A 56-year-old female presented with dyspnea, abdominal and leg swelling. Physical examination revealed finger clubbing, ascites and pretibial edema. Chest CT revealed diffuse micronodular opacities in both lungs without any enlarged thoracic lymph nodes. PFTs and DLCO/VA were moderately decreased. Transbronchial biopsy revealed non-caseified granulomas compatible with sarcoidosis. Serologic markers for infectious and autoimmune hepatitis were negative. Liver biopsy showed non-caseating granulomas, severe hepatitis and fibrosis. Stool, urinary analysis and antibodies for Schistosoma infection were negative. Final diagnosis was cirrhosis associated with stage III sarcoidosis. We report a case of sarcoidosis complicated by cirrhosis and portal hypertension with finger clubbing. Clinicians should bear in mind that cirrhosis, portal hypertension and clubbing may arise as the initial manifestations of sarcoidosis.

Nociceptive pain unmasking a serious pathology - paraneoplastic hypertrophic osteoarthropathy: A case report.

RATIONALE: Hypertrophic osteoarthropathy, also named Pierre Marie-Bamberger syndrome, represents a rare medical condition that may be considered either a primary or a secondary disease, and lung malignancies are responsible for more than two-thirds of the cases with secondary forms of the disease. PATIENT CONCERNS: We present the case of a 41-year-old man referred to our Neurology Department for pain that was considered secondary to cervical disc protrusions. The neurologic examination was normal. However, the general examination showed digital clubbing, right lateral cervical adenopathy, and pachydermia. The radiographic examinations of the upper and lower limbs depicted osseous abnormalities typical for periostosis, and the computed tomography of the thorax showed the presence of a mass lesion in the right upper pulmonary lobe. High values of vascular endothelial growth factor were also found. The patient was admitted to the Pneumology Clinic, where biopsy was performed from the lateral cervical adenopathy. DIAGNOSES: The anatomopathological examination revealed multiple neoplastic infiltrates suggestive of adenocarcinoma metastasis. Based on the clinical examination and radiological and histologic findings, the diagnosis of pulmonary adenocarcinoma with lymph nodes metastases and paraneoplastic hypertrophic osteoarthropathy was established. INTERVENTIONS: The patient received treatment with nonsteroidal antiinflammatory drugs and opiate analgesics that relieved the pain. OUTCOMES: The patient was referred to the Oncology Department for further treatment of the primary pathology. He received different types of chemotherapeutics, immunotherapy, and radiotherapy. However, despite all therapeutic measures, the disease rapidly progressed and the patient died 9 months later. LESSONS: This is an interesting case of a patient with an overlooked pathology, which was refereed to our clinic for further investigations of a pain that was considered neuropathic, secondary to small cervical protrusions. Conversely, the pain proved to be nociceptive and Pierre Marie-Bamberger syndrome was the positive diagnosis in our patient, as it can be associated with numerous diseases, especially of neoplastic origin.

Co-morbidity with hypertrophic osteoarthropathy: A possible Iron Age Sarmatian case from the Volga steppe of Russia.

PURPOSE: Hypertrophic osteoarthropathy (HOA) is a condition that can be inherited or acquired. It causes diffuse periosteal new bone formation on the long bones, with a predilection for the appendicular skeleton. When acquired, it is a nonspecific indicator of systemic disease that arises following a primary condition. This paper reviews the palaeopathological literature associated with this rare condition. It also describes the first possible case of co-morbidity associated with hypertrophic osteoarthropathy in an adult skeleton (cal. BC 170 - 1 cal. AD) from the mobile pastoralist Sarmatian culture of the Volga steppes of Russia. METHODS: Macroscopic and radiological examination provide differential diagnoses of the lesions, while clinical and bioarchaeological analyses offer insights into the possible experience of disease and social implications of care among the nomadic populations of Iron Age Russia. RESULTS: The analysis of Sk. 6524.102 displays lesions that may be due to both hypertrophic osteoarthropathy and osteomalacia. The man was physically impaired and his participation in physically challenging activities would have been limited. CONCLUSIONS: The study stresses that co-morbidity is a key parameter when interpreting disease in past populations, particularly when the diagnosis involves hypertrophic osteoarthropathy. SIGNIFICANCE: This is the first case of hypertrophic osteoarthropathy identified in Eurasian prehistoric populations. The research emphasises the significance of co-morbidity in the past. LIMITATIONS: The diagnosis of co-morbid diseases in human remains is extremely complex and the conditions were identified as most probable by a process of elimination. SUGGESTIONS FOR FURTHER RESEARCH: Further studies should be dedicated to understanding co-morbidity in the past.

[Paraneoplastic syndromes in rheumatology]. / Paraneoplastische Syndrome in der Rheumatologie.

Malignancies can present as inflammatory rheumatic diseases. These rheumatic paraneoplastic syndromes are rare, but characteristic in their pattern. This article focuses on epidemiology, clinical and diagnostic features as well as treatment of paraneoplasic rheumatic diseases such as paraneoplastic arthritides, vasculitides, myositis and hypertrophic osteoarthropathy. The knowledge of their clinical patterns is of utmost importance for early diagnosis and prognosis of yet undiagnosed malignancies.

Pierre-Marie-Bamberger syndrome and solitary fibrous tumor: a rare association.

A solitary fibrous tumor originates in the pleura with variable degrees of invasion. Hypertrophic osteoarthropathy, known as Pierre-Marie-Bamberger syndrome, is characterized by clubbing of the fingers due to bone surface and soft tissue calcification, historically known as a bronchogenic carcinoma paraneoplastic syndrome; however, a few cases have been associated with solitary fibrous tumors. We describe the case of a 38-year-old woman who presented with clubbing of the fingers. Studies revealed an intrathoracic fibrous tumor that was successfully treated with improvement in symptoms.

Touraine-Solente-Gole syndrome.

Touraine-Solente-Gole syndrome, also known as Pachydermoperiostosis (PDP) or Primary Hypertrophic Osteoarthropathy, is a rare hereditary disorder, which affects both bones and skin. It is characterized by a combination of dermatologic changes (pachydermia or thickening of the skin) and rheumatologic manifestations (periostosis and finger clubbing). Eyelid ptosis which is caused by thickened eyelids (blepharoptosis) is a less common symptom. We report the case of a patient with a complete form of Touraine-Solente-Gole syndrome with bilateral blepharoptosis as presenting feature.

Polyarthritis is a Rare Manifestation of Pachydermoperiostosis: A Case Report.

Pachydermoperiostosis or primary hypertrophic osteoarthropathy, also known as Touraine-Solente-Gole syndrome, is a rare process, frequently inherited. In its complete form it is characterized by pachydermia (thickening of the skin), skeletal changes (periostosis) and acropachia (digital clubbing). Diagnosis can be made considering the typical clinical features and the histological feature. We report a patient of 25-year old man presented with joint pain involving in multiple joints for last 7 years and progressive enlargement of his hands and feet with profuse sweating of palms and soles for last 4 years. Physical examination revealed thickened skin with excessive furrowing of his forehead, dropping of both eyelids, clubbing of all fingers, toes and enlargement of his hands and feet with pamoplantar hyperhidrosis. Laboratory investigation shows raised CRP, X-ray feet lateral view showed normal heel pad thickness, new bone formation and periosteal elevation in lower end of tibia and fibula and skin biopsy showed dense fibrocollagenous tissue in dermis and subcutis and mild acanthosis. With this scenario he was diagnosed as primary hypertrophic osteoarthropathy (Pachydermoperiostosis). After diagnosis he was treated with cholchicine (0.6mg) twice daily, naproxen (500mg) twice daily, and risedronate (150mg) monthly. After one year his skin texture became less thickened, joint pain improved, there was no further enlargement of acral part of fore arm. In this report we review the characteristic features of this syndrome. We highlight the importance of ruling out secondary forms of hypertrophic osteoarthropathy and of a close follow-up of these patients because of complications that might develop on long-term. Although no treatment was satisfactory, we wanted to emphasize that NSAIDs, Cholchicine and risedronate could be an effective treatment option.