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Pediatric Lymphadenopathy: Causes, Evaluation and Management
- 1. Dr. Sabona Lemessa (Assistant professor in pediatrics and child health, JUMC) 8/12/2022 1
- 2. Outline Introduction Definition Classification Etiologies Diagnosis Approach Treatment Reference 8/12/2022 2
- 3. Introduction The lymphoid tissues can be primary and secondary lymphoid organs Primary lymphoid tissues are sites where lymphocytes develop from progenitor cells into Functional and mature lymphocytes Marrow-the site where all lymphocyte progenitor cells reside and initially differentiate Thymus- differentiate into mature thymus-derived (T) cells 8/12/2022 3
- 4. Cont… Secondary lymphoid tissues are- sites where lymphocytes interact with each other and nonlymphoid cells to generate immune responses to antigens. Spleen, lymph nodes, and mucosa-associated lymphoid tissues (MALT). 8/12/2022 4
- 5. Lymph Nodes Are the primary sites of immune response to tissue antigens. Are round or kidney-shaped clusters of mononuclear cells. normally are less than 1 cm in diameter typically present at the branches of the lymphatic vessels and form part of the extensive network of lymphatic channels that extends throughout the body The site where different types of lymphocytes, macrophages and dendritic cells can interact with one another to generate an immune response to antigens carried within the lymph 8/12/2022 5
- 7. 8/12/2022 7
- 8. Lymphadenopathy Palpable lymph nodes are common in pediatrics because their immune systems are being activated by environmental Ag and the common organisms to which they are exposed LAP defined as any node greater than 1 cm in dimension Localized if it involves a single node or single nodal area and Generalized if it involves more than 2 noncontiguous nodal groups and may include HSM Chronic adenopathy is variably defined as enlargement that persists for longer than 3 weeks. 8/12/2022 8
- 9. Cont… Regional adenopathy is most frequently the result of infection in the involved node and/or its drainage area Generalized adenopathy caused by systemic disease and is often accompanied by abnormal physical findings in other systems 8/12/2022 9
- 10. Cont… Usually caused by either proliferation of normal lymphoid elements in response to antigenic stimuli by infiltration with malignant or phagocytic cells Deposition of foreign material within histiocytic cells of the node Vascular engorgement and edema secondary to local cytokine release Suppuration secondary to tissue necrosis 8/12/2022 10
- 11. Etiologies of Generalized LAP Infant Child Adolescent Common causes Syphilis Toxoplasmosis CMV HIV Viral infection EBV CMV HIV Toxoplasmosis Viral infection EBV CMV HIV Toxoplasmosis Syphilis Rare causes Chagas disease Leukemia Tuberculosis Reticuloendotheliosis Lymphoproliferative disease Metabolic storage disease Histiocytic disorders Serum sickness, SLE, JIA Leukemia/lymphoma, TB Measles, Sarcoidosis Fungal infection, Plague LCH,Chronic granulomatous ds Sinus histiocytosis Drug reaction Serum sickness, SLE, JIA Leukemia/lymphoma Hodgkin disease, TB Lymphoproliferative disease Histoplasmosis, Sarcoidosis Fungal infection, Plague Drug reaction Castleman disease 8/12/2022 11
- 12. Sites of Local Lymphadenopathy and Associated Diseases Cervical Ant. auricular Supraclavicular Epitrochlear Oropharyngeal infection (viral or GAS, Scalp infection/infestation (head lice) Mycobacterial lymphadenitis (tuberculosis and nontuberculous mycobacteria) Viral infection (EBV, CMV, HHV-6), Cat-scratch disease Toxoplasmosis, Kawasaki disease, Thyroid disease Autoimmune lymphoproliferative disease Periodic fever, aphthous stomatitis, pharyngitis Conjunctivitis Other eye infection Oculoglandular tularemia Facial cellulitis Otitis media Viral infection (especially rubella, parvovirus) Malignancy or infection in the mediastinum (right) Metastatic malignancy from the abdomen (left) Lymphoma Tuberculosis Hand infection, arm infection Lymphoma Sarcoid Syphilis 8/12/2022 12
- 13. Cont… Inguinal Hilar Axillary Abdominal Urinary tract infection Venereal disease (especially syphilis or lymphogranuloma venereum) Other perineal infections Lower extremity suppurative infection Plague Tuberculosis Histoplasmosis Blastomycosis Coccidioidomycosi s Leukemia/lympho ma Hodgkin disease Metastatic malignancy Sarcoidosis Castleman disease Cat-scratch disease Arm or chest wall infection Malignancy of chest wall Leukemia/lymphoma Brucellosis Malignancies Mesenteric adenitis (measles, tuberculosis, Yersinia , group A streptococcus) 8/12/2022 13
- 14. Cont… Mimics of lymphadenopathy include- Infection or stones in any of the salivary glands Congenital anomalies: branchial cleft cyst, cystic hygroma, thyroglossal duct cyst Thyroid nodule Soft-tissue swelling from trauma or an insect bite or sting Hematoma Inguinal hernia Hemangioma, lymphangioma Lipoma , Dermoid Rheumatoid nodules 8/12/2022 14
- 15. Approach History Location, and laterality of adenopathy Local symptoms suggestive of infection Associated constitutional symptoms (fever, weight loss, night sweats, arthralgias, skin rash) Contacts (viral respiratory infections, CMV, EBV,) GAS, TB Ingestion of unpasteurized animal milk (brucellosis, M. bovis ) or undercooked meats (toxoplasmosis, tularemia) Animal exposures (cat scratch disease, toxoplasmosis [cats], brucellosis [especially goats] Medications eg, phenytoin , carbamazepine Tick bites, flea bites, exposure to biting flies or mosquitoes (Lyme disease, bubonic plague, tularemia) 8/12/2022 15
- 16. Cont… Physical examination Anthropometry All system should be examined to have clue Lymph nodes - Location ,Size,Consistency , Fixation ,Tenderness 8/12/2022 16
- 17. General Characteristics of Enlarged Lymph Nodes According to Cause 8/12/2022 17
- 18. Investigations CBC, ESR, Serology, TST Gram stain and culture Fine needle aspiration (FNA) for cytology Biopsy Imaging CXR, Ultrasonography, CT scan 8/12/2022 18
- 19. Cont… Lymph node biopsy- early biopsy indicated for Systemic symptoms (fever >1 week, night sweats, weight loss (>10%)) Supraclavicular and lower cervical nodes Multiple sites of enlarged lymph nodes Fixed, non tender nodes in the absence of other symptoms Abnormal CXR or CBC Lack of infectious symptoms in the ear, nose, and throat regions Lymph nodes of >1 cm with onset in the neonatal period Lymph nodes >2 cm in diameter that have increased in size from baseline in 2wks or no decrease in size in 4-6 wk, no regression to “normal” in 8-12 wk 8/12/2022 19
- 20. Worrisome features Systemic symptoms fever >1 week night sweats weight loss >10% of body weight Supraclavicular (lower cervical) nodes Generalized lymphadenopathy Fixed, nontender nodes in the absence of other symptoms Lymph nodes of >1 cm with onset in the neonatal period (<1 month of age) 8/12/2022 20
- 21. Cont… Lymph nodes >2cm in diameter that have increased in size from baseline or have not responded to two weeks of antibiotic therapy Abnormal chest radiograph, particularly mediastinal mass or hilar adenopathy Abnormal CBC and differential (eg, lymphoblasts, cytopenias in more than one cell line) Lack of infectious symptoms in the ear, nose, and throat regions Persistently elevated ESR/CRP or rising ESR/CRP despite antibiotic therapy 8/12/2022 21
- 22. Diagnostic approach to Generalized LAP Occurs present in two or more noncontiguous regions usually is a manifestation of systemic disease When due to an infection, generalized lymphadenopathy is more commonly caused by viruses CMV, EBV or Mycobacterium tuberculosis than by S. aureus or group A Streptococcus (GAS) Indications for early biopsy Supraclavicular nodes Massively enlarged lymph nodes (ie, >4 cm) A group of nodes with a total diameter >3 cm 8/12/2022 22
- 23. Cont… Indications for biopsy within four weeks if Any lymph nodes increase in size There is a lymph node ≥2 cm in diameter and either of the following: The diagnosis remains uncertain after four weeks There is no response to therapy as indicated by the findings of initial 8/12/2022 23
- 24. Diagnostic approach to Localized adenopathy Supraclavicular lymphadenopathy- Associated with malignancy up to 75% in children Including leukemia, lymphoma, histiocytosis, neuroblastoma and GCT Right supraclavicular adenopathy is associated with cancer of the mediastinal lymph nodes. Left supraclavicular adenopathy ("Virchow's node") suggests intra-abdominal malignancy, most often lymphoma 8/12/2022 24
- 25. Axillary adenopathy Caused by Infections, including cat scratch disease and pyogenic infections (ie, S. aureus, GAS) of the upper arm For an obvious cause provide initial treatment as indicated. No obvious cause depends upon the size of the lymph nodes 8/12/2022 25
- 26. Step wise approach 8/12/2022 26
- 27. 8/12/2022 27
- 28. Inguinal lymphadenopathy In children usually is not associated with a specific cause unless the nodes are very large (>3 cm) Common causes in children and adolescents include sexually transmitted infections herpes simplex virus, syphilis, gonorrhea and lymphoma 8/12/2022 28
- 29. Step wise Approach 8/12/2022 29
- 30. 8/12/2022 30
- 31. Cervical lymphadenopathy Only one-quarter of patients have another serious disease, which most often is mycobacterial Upper posterior cervical lymphadenopathy rarely is associated with significant diseases in children the neck (scrofula). M. avium complex and M. scrofulaceum account for most cases in children Cervical lymphadenopathy and an obvious cause GAS pharyngitis provide initial treatment as indicated incision and drainage may be indicated 8/12/2022 31
- 32. 8/12/2022 32
- 33. TREATMENT Guided by the probable etiologic factor, as determined from the history and physical examination. If bacterial infection is suspected, antibiotic treatment covering at least streptococci and staphylococci is indicated Failure to decrease in size within 10-14 days also suggests the need for further evaluation Treating depending on final diagnosis Chemotherapy AntiTB 8/12/2022 33
- 34. Trial of antibiotic therapy Trial of antibiotic therapy is both a diagnostic and therapeutic intervention in children with localized adenopathy, uncertain diagnosis and no worrisome features. May be undertaken even In the absence of symptoms and signs of infection Because localized (nonsupraclavicular) adenopathy is so frequently caused by S. aureus, GAS, atypical mycobacteria, or B. henselae 8/12/2022 34
- 35. Cont… The antibiotic choice is influenced by the prevalence of community- associated methicillin-resistant S. aureus (CA-MRSA): High CA-MRSA prevalence – Clindamycin Low CA-MRSA prevalence – First-generation cephalosporin (eg, cephalexin or amoxicillin-clavulanic) For patients with exposure to cats or kittens coverage for B. henselae (eg, Azithromycin) in the initial regimen 8/12/2022 35
- 36. Cont… Broadened coverage If the patient's systemic symptoms (eg, fever) do not improve within 72 hours or the lymph node increases in size (at any point during treatment). 8/12/2022 36
- 37. Reference Richard L. Tower and Bruce M. Camitta, Lymphadenopathy in children, Nelson 21st edition. P. JOAN CHESNEY, Lymphatic System and Generalized Lymphadenopathy, Principles and Practice of Pediatric Infectious Diseases, 2nd edition. Sarah Alexander and Adolfo A. Ferrando, Approach to the Patient with LAP, Nathan and Oski chapter 53, 2015. Kenneth L Mcclain, MD, PHD, Peripheral lymphadenopathy in children, Uptodate 21.6. Leung AKC, Robson WLM. Childhood cervical lymphadenopathy. J Pediatr Health Care 2004;18:3–7 Philip Lanzkowsky, M.B., Ch.B., M.D., Sc.D. (honoris causa), F.R.C.P., D.C.H., F.A.A.P. manual pediatric hematology and oncology 4th edition. 8/12/2022 37 ed
Editor's Notes
- Location – Localized lymphadenopathy (present in only one region) suggests local causes and should prompt a search for pathology in the area of node drainage ( table 2 ), although some systemic diseases, such as plague ( Y. pestis ), tularemia, and aggressive lymphomas, can present with local adenopathy. Unilateral localized lymphadenopathy occurs in Hodgkin lymphoma or Rosai-Dorfman disease, whereas bilateral localized lymphadenopathy occurs in non-Hodgkin lymphoma and autoimmune lymphoproliferative disease (ALPS) ( table 3A-B ). Small occipital and postauricular nodes are common in infants, but not in older children [ 7,10 ]. However, multiple pea-sized occipital nodes are often found in children with acute lymphoblastic leukemia. In contrast, cervical and inguinal nodes are more common after two years of age than in the first six months of life [ 7,10 ]. Epitrochlear and supraclavicular adenopathy are uncommon at any age. Generalized adenopathy (present in two or more noncontiguous regions) usually is a manifestation of systemic disease ( table 3A-B ). Palpation of inguinal, cervical, and axillary nodes, in addition to the liver and spleen, can determine whether lymphadenopathy is localized or generalized. Size – The size of a lymph node that is considered to be normal varies depending upon the lymph node region and age of the child. Lymph nodes in most regions are less than 1 cm in their longest diameter; lymph nodes in the epitrochlear region are usually not palpable. Abnormal nodes generally are greater than 2 cm in diameter, but the probability of malignancy varies depending upon the age of the child, size of the node, and number of nodal groups involved (see 'Overview' above). Although malignancy can be found in smaller nodes in 10 to 20 percent of cases, it is more likely to be found in nodes larger than 2 cm [ 1 ]. In one series of 75 biopsies performed in children 8 months to 17 years, 15 percent of patients had nodes >3 cm in diameter [ 11 ]. Among these, six patients had reactive hyperplasia, seven granulomatous disease, and two lymphomas. In patients with Hodgkin lymphoma being evaluated by CT scans, the threshold for abnormal nodes is 1 to 1.5 cm in transverse diameter (Cotswolds convention) [ 12 ]. Consistency – Hard nodes are found in cancers that induce fibrosis (scirrhous changes) and when previous inflammation has left fibrosis. Firm, rubbery nodes are found in lymphomas and chronic leukemia; nodes in acute leukemia tend to be softer. (See "Overview of the presentation and classification of acute lymphoblastic leukemia in children" .) Spontaneous drainage of the lymph node and/or formation of a fistulous tract, if it develops over weeks to months, is suggestive of mycobacterial infection. Rapidly developing suppuration and drainage suggests pyogenic infection, usually a staphylococcal or streptococcal infection. Fixation – Normal lymph nodes are freely movable in the subcutaneous space. Abnormal nodes can become fixed to adjacent tissues (eg, deep fascia) by invading cancers or inflammation in tissue surrounding the nodes. They also can become fixed to each other ("matted") by the same processes. Tenderness – Tenderness suggests that recent, rapid enlargement has caused tension in the pain receptors in the capsule. Tenderness typically occurs with inflammatory processes, but it also can occur because of hemorrhage into a node, immunologic stimulation, and malignancy [ 1 ]. Thus, tenderness is not particularly helpful in discriminating between infectious and noninfectious causes of lymphadenopathy.
- GENERALIZED LYMPHADENOPATHY — Generalized lymphadenopathy is present in two or more noncontiguous regions. It may be a feature of numerous systemic diseases, many of which are recognized by other clinical findings (table 1A-B). Several of these diseases are discussed below. Systemic infection — Systemic bacterial or viral illnesses are the most common causes of generalized adenopathy [12]. Common viral causes of generalized lymphadenopathy include Epstein-Barr virus or cytomegalovirus mononucleosis, rubella, and measles (in parts of the world where rubella and measles are endemic). Mononucleosis — Classic infectious mononucleosis is characterized by the triad of moderate to high fever, pharyngitis, and lymphadenopathy. A characteristic distribution of lymph node involvement occurs; typically it is symmetric and involves the posterior cervical more than the anterior cervical chain. The posterior cervical nodes are deep to the sternocleidomastoid muscles (figure 1). Lymphadenopathy also may be present in the axillary and inguinal areas, which helps to distinguish infectious mononucleosis from other causes of pharyngitis. The lymph nodes are kidney-shaped and may be large. Lymphadenopathy peaks in the first week and then gradually subsides over two to three weeks. (See "Infectious mononucleosis in adults and adolescents".) HIV — Lymphadenopathy is common in primary human immunodeficiency virus (HIV) infection. Nontender adenopathy primarily involving the axillary, cervical, and occipital nodes develops in the majority of individuals during the second week of acute symptomatic HIV infection, concomitant with the emergence of a specific immune response to HIV [12]. The nodes decrease in size after the acute presentation, but a modest degree of adenopathy tends to persist. (See "Pediatric HIV infection: Classification, clinical manifestations, and outcome", section on 'Clinical manifestations'.) Miliary tuberculosis — Miliary tuberculosis is an important consideration in patients with generalized lymphadenopathy. Miliary tuberculosis can be mistaken for malignancy. (See "Clinical manifestations, diagnosis, and treatment of miliary tuberculosis" and "Epidemiology and pathology of miliary and extrapulmonary tuberculosis".) Systemic lupus erythematosus — Enlargement of lymph nodes occurs in approximately 50 percent of patients with systemic lupus erythematosus (SLE). The nodes typically are soft; nontender; discrete; varying in size from 0.5 to several centimeters; and usually detected in the cervical, axillary, and inguinal areas. Lymphadenopathy is noted more frequently at the onset of disease or in association with an exacerbation. Lymph node enlargement also can be caused by infection or a lymphoproliferative disease in SLE; when infections are present, the enlarged nodes are more likely to be tender. (See "Systemic lupus erythematosus (SLE) in children: Clinical manifestations and diagnosis", section on 'Hematologic abnormalities'.) Medications — Numerous medications may cause serum sickness that is characterized by fever, arthralgias, rash, and generalized lymphadenopathy (table 2). Phenytoin can cause generalized lymphadenopathy in the absence of a serum sickness reaction. (See "Serum sickness and serum sickness-like reactions" and "Antiseizure drugs: Mechanism of action, pharmacology, and adverse effects", section on 'Phenytoin' and "Drug eruptions", section on 'Drug reaction with eosinophilia and systemic symptoms (DRESS)'.) UNCOMMON BUT IMPORTANT CAUSES — Lymphadenopathy is a central feature of numerous less common systemic diseases that are important to consider because they are life-threatening or require specific treatment (table 3). Malignancy — Neoplastic causes of lymphadenopathy include Hodgkin disease, non-Hodgkin lymphoma, neuroblastoma, acute lymphocytic leukemia, acute myeloid leukemia, and rhabdomyosarcoma. The prevalence of malignancy among patients seen in the primary care setting is relatively low [4,5]. In contrast, the prevalence of malignancy in lymph node biopsies performed in pediatric referral centers ranges from 13 to 27 percent [3,6,7]. (See 'Epidemiology' above.) As discriminant features of malignancy versus other etiologies, fever, duration of adenopathy, and tenderness are not particularly helpful. However, a higher incidence of cancer is expected in patients with certain clinical characteristics (table 5). We recommend immediate biopsy for patients who have these findings. In addition to the other reasons for biopsy (weight loss, abnormal complete blood count, etc), lymph node biopsy also may be indicated in children with persistently elevated erythrocyte sedimentation rate (ESR) or rising ESR despite antibiotic therapy. (See "Peripheral lymphadenopathy in children: Evaluation and diagnostic approach", section on 'Lymph node biopsy'.) Kawasaki disease — Kawasaki disease is the most frequent cause of childhood vasculitis. This syndrome is associated with fever, cervical lymphadenopathy, and a variety of other manifestations, including conjunctivitis, mucositis, rash, and coronary artery aneurysms (table 6). (See "Kawasaki disease: Clinical features and diagnosis".) Tularemia — Patients with tularemia typically present with fever and a single erythematous papuloulcerative lesion with a central eschar (picture 3) that is accompanied by tender regional lymphadenopathy (picture 4). (See "Clinical manifestations, diagnosis, and treatment of tularemia", section on 'Clinical manifestations'.) The majority of cases in the United States occur in the central United States (eg, Arkansas, Missouri, Kansas, Nebraska, and Oklahoma). (See "Epidemiology, microbiology, and pathogenesis of tularemia", section on 'Distribution'.) Sources of infections in humans include vectors (eg, ticks, biting flies, and mosquitoes), handling of infected animals (rodents and rabbits), ingestion of inadequately cooked meat, drinking contaminated water, cat scratches or bites, or splashing infected material into the eye or rubbing eyes with contaminated fingers. (See "Epidemiology, microbiology, and pathogenesis of tularemia", section on 'Sources of human infection'.) Langerhans cell histiocytosis — Langerhans cell histiocytosis (LCH) may present with unilateral or bilateral cervical lymph nodes. Additional clinical manifestations of LCH may include lytic bone lesions, skin lesions, and hepatosplenomegaly. (See "Clinical manifestations, pathologic features, and diagnosis of Langerhans cell histiocytosis".) Sometimes there is massive enlargement similar to those seen in Rosai-Dorfman disease. (See 'Rosai-Dorfman disease' below.) Hemophagocytic lymphohistiocytosis — Hemophagocytic lymphohistiocytosis (HLH) patients have diffuse adenopathy in one-third of cases. Additional clinical manifestations of HLH may include fever, hepatosplenomegaly, neurologic symptoms, and skin lesions. (See "Clinical features and diagnosis of hemophagocytic lymphohistiocytosis", section on 'Clinical features'.) Chronic granulomatous disease — Chronic granulomatous disease encompasses a heterogeneous group of disorders characterized by genetic defects in the ability of phagocytes to generate reactive oxygen intermediates from molecular oxygen. These defects manifest primarily as an immunodeficiency resulting in frequent, severe infections, including pneumonia, abscesses, suppurative adenitis, osteomyelitis, bacteremia/fungemia, and superficial skin infections (cellulitis/impetigo). (See "Primary disorders of phagocytic function: An overview".) Castleman's disease — Castleman's disease is an uncommon lymphoproliferative disorder characterized by massive lymphadenopathy and systemic features such as fever, hepatomegaly, splenomegaly, and polyclonal hypergammaglobulinemia. Anemia is a common associated finding. (See "Unicentric Castleman's disease" and "Multicentric Castleman's disease".) Autoimmune lymphoproliferative disease — Patients with autoimmune lymphoproliferative disease (ALPS), also known as the Canale-Smith syndrome or autoimmunity/lymphoproliferation syndrome, usually present in the first year of life with massive cervical adenopathy that may obscure the angle of the jaw [13]. Splenomegaly also can occur. Frequent associations with autoimmune diseases, including hemolytic anemia, neutropenia, immune thrombocytopenia, glomerulonephritis, and Guillain-Barré syndrome, have been reported. The syndrome appears to be caused by a defect in lymphocyte apoptosis (programmed cell death). (See "Autoimmune lymphoproliferative syndrome (ALPS): Epidemiology and pathogenesis" and "Autoimmune lymphoproliferative syndrome (ALPS): Clinical features and diagnosis" and "Autoimmune lymphoproliferative syndrome (ALPS): Management and prognosis".) Rosai-Dorfman disease — Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) often presents in children with markedly enlarged, nontender cervical adenopathy of massive proportions, similar to patients with ALPS [14-18]. However, other nodal sites, including the mediastinum, retroperitoneal, axillary, and inguinal sites, also may be involved. Other manifestations include involvement of the nasal cavity, salivary gland tissue and other regions of the head and neck, lytic bone lesions, pulmonary nodules, or rash [19]. Patients often are febrile when massive lymphadenopathy is present. Laboratory evaluations show leukocytosis, polyclonal hypergammaglobulinemia, a hypochromic or normocytic anemia, and elevated ESR. Treatment is variable depending upon involvement of other nodal areas. Clofarabine has been especially effective for patients with bone and orbital involvement [20]. Spontaneous resolution may be observed but can take many months if not years [21]. Kikuchi disease — Kikuchi disease is a rare, benign condition of unknown cause usually characterized by cervical lymphadenopathy (although it may be more generalized) and fever. It is discussed in detail separately. (See "Kikuchi disease".) Kimura disease — Kimura disease, another rare cause of cervical lymphadenopathy, is characterized by a triad of painless subcutaneous masses in the head and neck with lymphadenopathy, blood and tissue eosinophilia, and markedly increased serum immunoglobulin E [22]. Inflammatory pseudotumor — Inflammatory pseudotumor of lymph nodes is a syndrome of lymphadenopathy in one or more node groups, often with systemic symptoms. Nodes show a fibrosing and inflammatory process [23]. Sarcoidosis — Children with sarcoid may present with impressive cervical adenopathy, similar to ALPS or Rosai-Dorfman disease patients. Lymphadenopathy is present in 50 percent of patients with sarcoidosis, who often have weight loss, cough, fatigue, lethargy, bone and joint pain, anorexia, and headache [24]. African-Americans represent two-thirds to three-fourths of patients. (See "Clinical manifestations and diagnosis of pulmonary sarcoidosis".)