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Undifferentiated connective tissue disease

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Synonyms and keywords: Undifferentiated connective tissue disorder, Undifferentiated systemic rheumatic disease, (Early) undifferentiated connective tissue disease, collagen vascular diseases

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

The concept of undifferentiated connective tissue disease(UCTD) came into existence in 1980 when LeRoy et al found out some disease entities which don’t qualify the criteria of definite connective tissue disease but resemble rheumatological diseases. The patients with UCTD presents with signs and symptoms of connective tissue diseases like arthritis, Raynaud’s phenomenon, Dry-mouth, dry eyes.Undifferentiated connective tissue disorder(UCTD) are actually a group of disorders that are unclassified in other connective tissue disorders. UCTD may be classified according to criteria developed by American College of Rheumatology(ACR) and European League Against Rheumatism(EULAR) : early Raynaud’s phenomenon, early inflammatory arthritis that is not classified as rheumatoid arthritis, some manifestations same as that of inflammatory myopathy, systemic lupus erythematosus, Sjögren’s syndrome, vasculitis, serositis, or interstitial lung disease which don’t meet diagnostic criteria for each of them and early scleroderma.The exact pathogenesis of undifferentiated connective tissue disorder(UCTD) is not fully understood. It is understood that UCTD is the result of autoimmune process and occurs in phases: Initial phase: which is asymptomatic and absence of auto-antibodies and second phase with the presence of auto-antibodies. The second phase is usually triggered by the environmental factors, such as infection. Auto-antibodies appear before the symptoms of the disease.The time between the two is variable. Autoantibodies usually seen in UCTD with positive correlation are antibodies against C1q ,Anti heat shock protein(hsp)-65, Anti hsp 60. The clinical picture involves the overlap of symptoms of other rheumatological disease but not completing the criteria of their diagnosis. More studies are needed to find the pathogenesis of UCTD.The cause of undifferentiated connective tissue disease has not been identified.UCTD is differentiated with other causes of arthritis and rash such as SLE, Sjögren’s syndrome, Rheumatoid arthritis. Not enough epidemiological studies have been done but about 25% of the presentation of more than 1 year resembles connective tissue disorder.UCTD is differentiated with other causes of arthritis and rash such as SLE, Sjögren’s syndrome, Rheumatoid arthritis.There are no established risk factors for UCTD.The symptoms of undifferentiated connective tissue disease(UCTD) usually are similar to connective tissue diseases but couldn’t complete the criteria for their definitive diagnosis. If left untreated, 24.3-33% of patients with UCTD may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome. Common complications of UCTD include Interstitial lung disease– Non specific interstitial pneumonia, atherosclerosis. Depending on the extent of the UCTD at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. The presence of interstitial lung disease is associated with a particularly good prognosis among patients with UCTD rather than being idiopathic.Patients with undifferentiated connective tissue disease (UCTD) may have a positive history of Raynaud’s phenomenon, arthritis, photosensitivity, sicca symptoms, pleuritis/pericarditis. The common symptoms of UCTD include arthralgia, dry eyes, rash, photosensitivity and dry mouth. Less common symptoms of UCTD include fever, pleuritic chest pain and seizures. Patients with UCTD usually appear well but can present with low-grade fever, Raynaud’s phenomenon, rash and Erythema nodosum in the skin examination. On examining HEENT,Conjunctivitis, Uveitis, Keratoconjunctivitis sicca, dry-mouth can be seen. In the neck, lymphadenopathy can be seen. Rhonchi, Wheezing, pleural friction rub and Pericardial friction rub can be heard on auscultation.On examining abdomen, tenderness in the right/left upper/lower abdominal quadrant and hepatomegaly are noted. In the back, tenderness over lumbar vertebrae can occur and sacral edema may be seen.Rashes can be seen in the genitourinary exam. The patient can have altered mental statuswith psychosis and peripheral neuropathy and Pitting/non-pitting edema of the lower extremities. Laboratory findings suggestive of undifferentiated connective tissue disease include Leukopenia,increased erythrocyte sedimentation rate, C-reactive protein, Antinuclear antibodies, anti DNA antibodies, anti Ro/SSA antibodies and anti-RNP antibodies.The pharmacologic medical therapy is recommended based on the rheumatic disease pattern manifested by the patient such as methotrexate in those exhibiting arthritis and dermatitis. Supportive therapy includes; avoiding cold exposure in those experiencing Raynaud’s phenomenon, avoiding sun in photosensitivity, non-steroidal anti-inflammatory drugs for pain control, sunscreen used for photosensitivity, and emollients for dry skin, rash.The mainstay of treatment for undifferentiated connective tissue disorder is medical therapy. Surgery is rarely needed for patients for surgical biopsy in case of diagnosis.The feasibility of surgery depends on the prognosis at diagnosis.

Historical Perspective

Undifferentiated connective tissue disease (UCTD) was first explored by LeRoy et al in 1980.

Classification

Undifferentiated connective tissue disorder (UCTD) are actually a group of disorders that are unclassified in other connective tissue disorders. UCTD may be classified according to the criteria developed by American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) into: early Raynaud’s phenomenon, early inflammatory arthritis that is not classified as rheumatoid arthritis, some manifestations same as that of inflammatory myopathy, systemic lupus erythematosus, Sjögren’s syndrome, vasculitis, serositis, or interstitial lung disease which don’t meet diagnostic criteria for each of them and early scleroderma.

Pathophysiology

The exact pathogenesis of undifferentiated connective tissue disorder (UCTD) is not fully understood. It is understood that UCTD is the result of an autoimmune process and occurs in phases: Initial phase: which is asymptomatic and is characterized by absence of auto-antibodies and second phase which is characterized by the presence of auto-antibodies. The second phase is usually triggered by the environmental factors, such as infection. Auto-antibodies appear before the symptoms of the disease.The time between the two is variable. Autoantibodies usually seen in UCTD with positive correlation are antibodies against C1q ,Anti heat shock protein (hsp)-65, Anti hsp 60. The clinical picture involves the overlap of symptoms of other rheumatological disease but not completing the criteria of their diagnosis. More studies are needed to find the pathogenesis of UCTD.

Causes

The cause of undifferentiated connective tissue disease has not been identified.

Differentiating UTCD from Other Diseases

UCTD should be differentiated from other causes of arthritis and rash such as SLE, Sjögren’s syndrome, Rheumatoid arthritis.

Epidemiology and Demographics

There is absence of any epidemiological study regarding UTCD till date but its estimated that UCTD represents 60% of the disease with undifferentiated onset.

Risk Factors

There are no established risk factors for UCTD.

Screening

There is insufficient evidence to recommend routine screening for undifferentiated connective tissue disease.

Natural History, Complications, and Prognosis

The symptoms of undifferentiated connective tissue disease (UCTD) usually are similar to connective tissue diseases but couldn’t complete the criteria for their definitive diagnosis. If left untreated, 24.3-33% of patients with UCTD may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome. Common complications of UCTD include Interstitial lung disease– Non specific interstitial pneumonia, atherosclerosis. Depending on the extent of the UCTD at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. The presence of interstitial lung disease is associated with a particularly good prognosis among patients with UCTD rather than being idiopathic.

Diagnosis

History and Symptoms

Patients with undifferentiated connective tissue disease (UCTD) may have a positive history of Raynaud’s phenomenon, arthritis, photosensitivity, sicca symptoms, pleuritis/pericarditis. The common symptoms of UCTD include arthralgia, dry eyes, rash, photosensitivity and dry mouth. Less common symptoms of UCTD include fever, pleuritic chest pain and seizures.

Physical Examination

Patients with UCTD usually appear well but can present with low-grade fever, Raynaud’s phenomenon, rash and Erythema nodosum. On HEENT examination, Conjunctivitis, Uveitis, Keratoconjunctivitis sicca, dry-mouth can be seen. In the neck, lymphadenopathy can be seen. Rhonchi, Wheezing, pleural friction rub and Pericardial friction rub can be heard on auscultation. On examining abdomen, tenderness in the right/left upper/lower abdominal quadrant and hepatomegaly may be noted. In the back, tenderness over the lumbar vertebrae can occur and sacral edema may be seen. Rash can be seen in the genitourinary exam. The patient can have altered mental status with psychosis and peripheral neuropathy and pitting/non-pitting edema of the lower extremities.

Laboratory Findings

Laboratory findings suggestive of undifferentiated connective tissue disease include Leukopenia,increased erythrocyte sedimentation rate, C-reactive protein, Antinuclear antibodies, anti DNA antibodies, anti Ro/SSA antibodies, and anti-RNP antibodies.

CT scan

There are no CT scan findings associated specifically with undifferentiated connective tissue disease.

MRI

There are no MRI findings associated specifically with undifferentiated connective tissue disease.

Other Imaging Findings

There are no other imaging findings associated specifically with undifferentiated connective tissue disease.

Other Diagnostic Studies

There are no other diagnostic studies associated specifically with undifferentiated connective tissue disease.

Treatment

Medical Therapy

The pharmacologic medical therapy is recommended based on the rheumatic disease pattern manifested by the patient such as methotrexate in those exhibiting arthritis and dermatitis. Supportive therapy includes; avoiding cold exposure in those experiencing Raynaud’s phenomenon, avoiding sun in photosensitivity, non-steroidal anti-inflammatory drugs for pain control, sunscreen used for photosensitivity, and emollients for dry skin and rash.

Prevention

There are no established measures for the prevention of undifferentiated connective tissue disease.

References

Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

Overview

Undifferentiated connective tissue disease (UCTD) was first explored by LeRoy et al in 1980.

Historical Perspective

  • Mixed connective tissue disorder was first coined in 1973 which lead to emergence of a new term in 1980.[1]
  • Undifferentiated connective tissue disease (UCTD) was first explored by LeRoy et al in 1980.[2]

References

  1. ↑ Sharp GC: Mixed connective tissue disease (Chapter 51), Arthritis and Allied Conditions. Edited by DJ McCarty.Ninth edition. Philadelphia, Lea & Febiger, 1979, pp 737-74 1
  2. ↑ LeRoy EC, Maricq HR, Kahaleh MB (March 1980). “Undifferentiated connective tissue syndromes”. Arthritis Rheum. 23 (3): 341–3. PMID 7362686.

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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

Undifferentiated connective tissue disorder (UCTD) are actually a group of disorders that are unclassified in other connective tissue disorders. UCTD may be classified according to the criteria developed by American College of Rheumatology (ACR) and European League Against Rheumatism (EULAR) into: early Raynaud’s phenomenon, early inflammatory arthritis that is not classified as rheumatoid arthritis, some manifestations same as that of inflammatory myopathy, systemic lupus erythematosus, Sjögren’s syndrome, vasculitis, serositis, or interstitial lung disease which don’t meet diagnostic criteria for each of them and early scleroderma.

Classification

References

  1. ↑ Mosca M, Tani C, Vagnani S, Carli L, Bombardieri S (2014). “The diagnosis and classification of undifferentiated connective tissue diseases”. J. Autoimmun. 48-49: 50–2. doi:10.1016/j.jaut.2014.01.019. PMID 24518855.
  2. ↑ Mosca M, Neri R, Bombardieri S (1999). “Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria”. Clin. Exp. Rheumatol. 17 (5): 615–20. PMID 10544849.
  3. ↑ Costenbader KH, Schur PH (May 2015). “We need better classification and terminology for “people at high risk of or in the process of developing lupus““. Arthritis Care Res (Hoboken). 67 (5): 593–6. doi:10.1002/acr.22484. PMC 4391980. PMID 25302656.

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

The exact pathogenesis of undifferentiated connective tissue disorder (UCTD) is not fully understood. It is understood that UCTD is the result of an autoimmune process and occurs in phases: Initial phase: which is asymptomatic and is characterized by absence of auto-antibodies and second phase which is characterized by the presence of auto-antibodies. The second phase is usually triggered by the environmental factors, such as infection. Auto-antibodies appear before the symptoms of the disease.The time between the two is variable. Autoantibodies usually seen in UCTD with positive correlation are antibodies against C1q ,Anti heat shock protein (hsp)-65, Anti hsp 60. The clinical picture involves the overlap of symptoms of other rheumatological disease but not completing the criteria of their diagnosis. More studies are needed to find the pathogenesis of UCTD.

Pathophysiology

Pathogenesis

The overalp of symptoms in UCTD; Source: Pinterest.com[1]
  • The exact pathogenesis of undifferentiated connective tissue disorder (UCTD) is not fully understood.[2]
  • It is understood that UCTD is the result of an autoimmune process and occurs in phases.[3]
    • Initial phase: Asymptomatic and is characterized by the absence of auto-antibodies
    • Second phase: Presence of auto-antibodies
  • The second phase is usually triggered by the environmental factors, such as infection.
  • Auto-antibodies appear before the symptoms of the disease.The time between the two is variable.
  • Autoantibodies usually seen in UCTD with positive correlation are antibodies against:[4]
  • An increase in CD4+ memory and a decrease of naive CD4+ T cells were detected in patients with UCTD in a study that points to an ongoing active immune reaction.[7]
  • One study has shown the basis of impaired endothelial function in UCTD is due to inflammation.[8]
  • The clinical picture involves the overlap of symptoms of other rheumatological disease but not completing the criteria for their diagnosis.
  • Some studies show that about one-third of patients with UCTD can develop other connective tissue diseases.[6][9]
  • More studies are needed to find the pathogenesis of UCTD.

Genetics

  • A genetic basis is suspected for UCTD, as an autoimmune disease but nothing specific has been discovered to date.

Gross Pathology

  • On gross pathology, UCTD shows features of auto-immune disorders.

Microscopic Pathology

  • On microscopic histopathological analysis, UCTD shows features of auto-immune disorders.

References

  1. ↑ https://i.pinimg.com/736x/52/f7/56/52f75625e86e56001103e57adab227fd–invisible-illness-chronic-illness.jpg Image accessed on April 25, 2018
  2. ↑ Mosca M, Neri R, Bombardieri S (1999). “Undifferentiated connective tissue diseases (UCTD): a review of the literature and a proposal for preliminary classification criteria”. Clin. Exp. Rheumatol. 17 (5): 615–20. PMID 10544849.
  3. ↑ Bizzaro N, Tozzoli R, Shoenfeld Y (June 2007). “Are we at a stage to predict autoimmune rheumatic diseases?”. Arthritis Rheum. 56 (6): 1736–44. doi:10.1002/art.22708. PMID 17530702.
  4. ↑ Horváth L, Czirják L, Fekete B, Jakab L, Prohászka Z, Cervenak L, Romics L, Singh M, Daha MR, Füst G (January 2001). “Levels of antibodies against C1q and 60 kDa family of heat shock proteins in the sera of patients with various autoimmune diseases”. Immunol. Lett. 75 (2): 103–9. PMID 11137133.
  5. ↑ Infantino, Maria; Shovman, Ora; Pérez, Dolores; Manfredi, Mariangela; Grossi, Valentina; Benucci, Maurizio; Gobbi, Francesca Li; Bandinelli, Francesca; Damiani, Arianna; Moscato, Paolo; Azoulay, Danielle; Gilburd, Boris; Shoenfeld, Yehuda (2018). “Anti-DFS70 autoantibodies in undifferentiated connective tissue diseases subjects: what’s on the horizon?”. Rheumatology. doi:10.1093/rheumatology/key012. ISSN 1462-0324.
  6. ↑ 6.0 6.1 Cavazzana I, Franceschini F, Belfiore N, Quinzanini M, Caporali R, Calzavara-Pinton P, Bettoni L, Brucato A, Cattaneo R, Montecucco C (2001). “Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients”. Clin. Exp. Rheumatol. 19 (4): 403–9. PMID 11491495.
  7. ↑ Szodoray P, Nakken B, Barath S, Gaal J, Aleksza M, Zeher M, Sipka S, Szilagyi A, Zold E, Szegedi G, Bodolay E (August 2008). “Progressive divergent shifts in natural and induced T-regulatory cells signify the transition from undifferentiated to definitive connective tissue disease”. Int. Immunol. 20 (8): 971–9. doi:10.1093/intimm/dxn056. PMID 18550583.
  8. ↑ Laczik, R.; Soltesz, P.; Szodoray, P.; Szekanecz, Z.; Kerekes, G.; Paragh, G.; Rajnavolgyi, E.; Abel, G.; Szegedi, G.; Bodolay, E. (2014). “Impaired endothelial function in patients with undifferentiated connective tissue disease: a follow-up study”. Rheumatology. 53 (11): 2035–2043. doi:10.1093/rheumatology/keu236. ISSN 1462-0324.
  9. ↑ Bodolay E, Csiki Z, Szekanecz Z, Ben T, Kiss E, Zeher M, Szücs G, Dankó K, Szegedi G (2003). “Five-year follow-up of 665 Hungarian patients with undifferentiated connective tissue disease (UCTD)”. Clin. Exp. Rheumatol. 21 (3): 313–20. PMID 12846049.

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Causes

Causes

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

The cause of undifferentiated connective tissue disease has not been identified. To review risk factors for the development of undifferentiated connective tissue disease, click here.

Causes

  • The cause of undifferentiated connective tissue disease has not been identified. To review risk factors for the development of undifferentiated connective tissue disease, click here.

References

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Differentiating Undifferentiated connective tissue disease from other Diseases

Differentiating Undifferentiated connective tissue disease from other Diseases

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mahshid Mir, M.D. [2] Amandeep Singh M.D.[3]

Overview

UCTD should be differentiated from other causes of arthritis and rash such as SLE, Sjögren’s syndrome, Rheumatoid arthritis

Differential Diagnosis

Differentiating undifferentiated connective tissue disease from other diseases that may cause arthritis and rash

Abbreviations: ANA: Antinuclear antibody, RF: Rheumatoid factor, Anti-CCp: Anti-cyclic citrullinated protein antibody, Anti U1RNP: Anti-U1 ribonucleoprotein antibodies , Anti Sm : Anti-Sm antibodies, Anti Ro: Anti Ro antibody also called anti-Sjögren’s-syndrome-related antigen A antibody, Anti-dsDNA: Anti-double stranded DNA.

Disease Arthritis Auto-antibodies Raynaud phenomenon Rash pattern Distinguishing/specific features
Polyarthritis Tenderness Edema Deformity /Erosion Pattern ANA RF Anti-CCp Anti U1RNP Anti Sm Anti Ro Anti-dsDNA
Undifferentiated connective tissue disease (UCTD)[1] + – – – Lower extremity ↑ ↑ – – ↑ – – + Erythematous macules, patches, or papules with delicate scale Multiple connective tissue diseases with no enough criteria for a single diagnosis
Systemic lupus erythematosus[2] + + + – Small joints ↑ – – – ↑ ↑ – + Malar rash and photosensitivity
Rheumatoid arthritis (RA)[3] + + + + Small and large joints – ↑↑ ↑↑ – – – – + Subcutaneous nodules Erosive arthropathy
Rhupus[4] + + + + Small and large joints ↑ ↑ ↑ ↑ ↑ – ↑ + Malar rash and photosensitivity Erosive arthropathy
Mixed connective tissue disease (MCTD)[5] – – – + Small and large joints – ↑↑ ↑ – – – – + Cutaneous eruptions, gottron’s papules, photodistributed erythema, poikiloderma, and calcinosis cutis Overlapping features of SLE, systemic sclerosis (SSc), and polymyositis (PM) that lead to more than one diagnosis
Systemic sclerosis (SSc)[6] +/- + + +/- Lower extremity ↑↑ – – – ↑ – ↑ + Hyperkeratosis, edema, and erythema Sclerodactyly, Telangiectasias, Calcinosis, Malignant hypertension, acute renal failure
Sjögren’s syndrome[7] +/- +/- – – Lower extremity, axiallary creases ↑ – – – ↑ ↑ – – Xerosis, scaly skin, annular erythema Keratoconjunctivitis sicca
Vasculitis Giant cell[8] – + + – Distal extremity – – – – – – – – Rare Involvement of cranial branches of arteries, visual loss
Takayasu[9] – +/- +/- – Transient extremity – – – – – – – Erythema nodosum, pyoderma gangrenosum Absent or weak peripheral pulse
Poly-arteritis nodosa[10] – +/- – – General and mild – – – – – – – Tender erythematous nodules, purpura, livedo reticularis, bullous or vesicular eruption Testicular pain or tenderness and neuropathies
Behçet’s syndrome[11] +/- +/- +/- – medium and large joints – – – – – – – – Recurrent and usually painful mucocutaneous ulcers, acneiform lesions, papulo-vesiculo-pustular eruptions, superficial thrombophlebitis Male dominancy
Kikuchi’s disease[12] – +/- – – medium and large joints ↑/↓ – – – – – – – Transient skin rashes, malar rash, erythematous macules, patches, papules, or plaques May be associated with SLE
Serum sickness[13] + + +/- – General – – – – – – – – Pruritic rash, urticaria and/or serpiginous macular rash Self-limited
Psoriatic arthritis[14] – – – – Small and large joints – – – – – – – – Psoriasis and onychodystrophy Dactylitis (sausage digits)
Human parvovirus B19 infection[15] + + – – Small joints – – – – – – – – Erythematous rashes Rare in adults, fifth’s disease in children

References

  1. ↑ Alarcón GS, Williams GV, Singer JZ, Steen VD, Clegg DO, Paulus HE, Billingsley LM, Luggen ME, Polisson RP, Willkens RF (1991). “Early undifferentiated connective tissue disease. I. Early clinical manifestation in a large cohort of patients with undifferentiated connective tissue diseases compared with cohorts of well established connective tissue disease”. J. Rheumatol. 18 (9): 1332–9. PMID 1757934.
  2. ↑ Ehmke TA, Cherian JJ, Wu ES, Jauregui JJ, Banerjee S, Mont MA (2014). “Treatment of osteonecrosis in systemic lupus erythematosus: a review”. Curr Rheumatol Rep. 16 (9): 441. doi:10.1007/s11926-014-0441-8. PMID 25074031.
  3. ↑ Lee DM, Weinblatt ME (2001). “Rheumatoid arthritis”. Lancet. 358 (9285): 903–11. doi:10.1016/S0140-6736(01)06075-5. PMID 11567728.
  4. ↑ Panush RS, Edwards NL, Longley S, Webster E (1988). “‘Rhupus’ syndrome”. Arch. Intern. Med. 148 (7): 1633–6. PMID 3382309.
  5. ↑ Cappelli S, Bellando Randone S, Martinović D, Tamas MM, Pasalić K, Allanore Y, Mosca M, Talarico R, Opris D, Kiss CG, Tausche AK, Cardarelli S, Riccieri V, Koneva O, Cuomo G, Becker MO, Sulli A, Guiducci S, Radić M, Bombardieri S, Aringer M, Cozzi F, Valesini G, Ananyeva L, Valentini G, Riemekasten G, Cutolo M, Ionescu R, Czirják L, Damjanov N, Rednic S, Matucci Cerinic M (2012). ““To be or not to be,” ten years after: evidence for mixed connective tissue disease as a distinct entity”. Semin. Arthritis Rheum. 41 (4): 589–98. doi:10.1016/j.semarthrit.2011.07.010. PMID 21959290.
  6. ↑ LeRoy EC, Black C, Fleischmajer R, Jablonska S, Krieg T, Medsger TA, Rowell N, Wollheim F (1988). “Scleroderma (systemic sclerosis): classification, subsets and pathogenesis”. J. Rheumatol. 15 (2): 202–5. PMID 3361530.
  7. ↑ Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P (2004). “Cutaneous manifestations of primary Sjögren’s syndrome are underestimated”. Clin. Exp. Rheumatol. 22 (5): 632–6. PMID 15485020.
  8. ↑ Bablekos GD, Michaelides SA, Karachalios GN, Nicolaou IN, Batistatou AK, Charalabopoulos KA (2006). “Pericardial involvement as an atypical manifestation of giant cell arteritis: report of a clinical case and literature review”. Am. J. Med. Sci. 332 (4): 198–204. PMID 17031245.
  9. ↑ Lupi-Herrera E, Sánchez-Torres G, Marcushamer J, Mispireta J, Horwitz S, Vela JE (1977). “Takayasu’s arteritis. Clinical study of 107 cases”. Am. Heart J. 93 (1): 94–103. PMID 12655.
  10. ↑ Pagnoux C, Seror R, Henegar C, Mahr A, Cohen P, Le Guern V, Bienvenu B, Mouthon L, Guillevin L (2010). “Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database”. Arthritis Rheum. 62 (2): 616–26. doi:10.1002/art.27240. PMID 20112401.
  11. ↑ Tunç R, Uluhan A, Melikoğlu M, Ozyazgan Y, Ozdoğan H, Yazici H (2001). “A reassessment of the International Study Group criteria for the diagnosis (classification) of Behçet’s syndrome”. Clin. Exp. Rheumatol. 19 (5 Suppl 24): S45–7. PMID 11760398.
  12. ↑ Kucukardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2007). “Kikuchi-Fujimoto Disease: analysis of 244 cases”. Clin. Rheumatol. 26 (1): 50–4. doi:10.1007/s10067-006-0230-5. PMID 16538388.
  13. ↑ Kunnamo I, Kallio P, Pelkonen P, Viander M (1986). “Serum-sickness-like disease is a common cause of acute arthritis in children”. Acta Paediatr Scand. 75 (6): 964–9. PMID 3564980.
  14. ↑ Oriente P, Biondi-Oriente C, Scarpa R (1994). “Psoriatic arthritis. Clinical manifestations”. Baillieres Clin Rheumatol. 8 (2): 277–94. PMID 8076388.
  15. ↑ Kaufmann J, Buccola JM, Stead W, Rowley C, Wong M, Bates CK (2007). “Secondary symptomatic parvovirus B19 infection in a healthy adult”. J Gen Intern Med. 22 (6): 877–8. doi:10.1007/s11606-007-0173-9. PMC 2219874. PMID 17384979.

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Epidemiology and Demographics

Epidemiology and Demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

Epidemiology and Demographics

  • There is absence of any epidemiological study till date regarding UTCD but its estimated that UCTD represents 60% of the disease with undifferentiated onset.[1]

Mental disorders may be relevant[2][3].

References

  1. ↑ Mosca M, Tani C, Bombardieri S (December 2007). “Undifferentiated connective tissue diseases (UCTD): a new frontier for rheumatology”. Best Pract Res Clin Rheumatol. 21 (6): 1011–23. doi:10.1016/j.berh.2007.09.004. PMID 18068858.
  2. ↑ Dobkin PL, Filipski M, Looper K, Schieir O, Baron M, McGill Early Arthritis Research Group (2008). “Identifying target areas of treatment for depressed early inflammatory arthritis patients”. Psychother Psychosom. 77 (5): 298–305. doi:10.1159/000142522. PMID 18600035.
  3. ↑ Schieir O, Thombs BD, Hudson M, Taillefer S, Steele R, Berkson L; et al. (2009). “Symptoms of depression predict the trajectory of pain among patients with early inflammatory arthritis: a path analysis approach to assessing change”. J Rheumatol. 36 (2): 231–9. doi:10.3899/jrheum.080147. PMID 19132790.

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Risk Factors

Risk Factors

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

There are no established risk factors for UCTD.

Risk Factors

  • There are no established risk factors for UCTD

References

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screening

screening

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

There is insufficient evidence to recommend routine screening for undifferentiated connective tissue disease.

Screening

  • There is insufficient evidence to recommend routine screening for undifferentiated connective tissue disease.

References

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Natural History, Complications and Prognosis

Natural History, Complications and Prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Overview

The symptoms of undifferentiated connective tissue disease (UCTD) usually are similar to connective tissue diseases but couldn’t complete the criteria for their definitive diagnosis. If left untreated, 24.3-33% of patients with UCTD may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome. Common complications of UCTD include Interstitial lung disease– Non specific interstitial pneumonia, atherosclerosis. Depending on the extent of the UCTD at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good. The presence of interstitial lung disease is associated with a particularly good prognosis among patients with UCTD rather than being idiopathic.

Natural History, Complications, and Prognosis

Natural History

  • The symptoms of undifferentiated connective tissue disease (UCTD) usually are similar to connective tissue diseases but couldn’t complete the criteria for their definitive diagnosis.
  • If left untreated, 24.3-33% of patients with UCTD may progress to develop manifestations of well defined connective tissue disease such as systemic lupus erythematosus, rheumatoid arthritis, Sjögren’s syndrome.[1][2]

Complications

Prognosis

  • Depending on the extent of the UCTD at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as good.
  • The presence of interstitial lung disease is associated with a particularly good prognosis among patients with UCTD rather than being idiopathic.[3]

References

  1. ↑ Danieli MG, Fraticelli P, Salvi A, Gabrielli A, Danieli G (1998). “Undifferentiated connective tissue disease: natural history and evolution into definite CTD assessed in 84 patients initially diagnosed as early UCTD”. Clin. Rheumatol. 17 (3): 195–201. PMID 9694051.
  2. ↑ Cavazzana I, Franceschini F, Belfiore N, Quinzanini M, Caporali R, Calzavara-Pinton P, Bettoni L, Brucato A, Cattaneo R, Montecucco C (2001). “Undifferentiated connective tissue disease with antibodies to Ro/SSa: clinical features and follow-up of 148 patients”. Clin. Exp. Rheumatol. 19 (4): 403–9. PMID 11491495.
  3. ↑ 3.0 3.1 Kinder BW, Shariat C, Collard HR, Koth LL, Wolters PJ, Golden JA, Panos RJ, King TE (April 2010). “Undifferentiated connective tissue disease-associated interstitial lung disease: changes in lung function”. Lung. 188 (2): 143–9. doi:10.1007/s00408-009-9226-7. PMC 2837880. PMID 20069430.
  4. ↑ Laczik, R.; Soltesz, P.; Szodoray, P.; Szekanecz, Z.; Kerekes, G.; Paragh, G.; Rajnavolgyi, E.; Abel, G.; Szegedi, G.; Bodolay, E. (2014). “Impaired endothelial function in patients with undifferentiated connective tissue disease: a follow-up study”. Rheumatology. 53 (11): 2035–2043. doi:10.1093/rheumatology/keu236. ISSN 1462-0324.

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Diagnosis

Diagnosis

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Treatment

Treatment

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Case Studies

Case #1


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